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Pediatric acute appendicitis: Is it time for nonoperative treatment (NOT)?
Clinical question
What are the differences in rates of treatment failure, duration of hospitalization, and cost between nonoperative treatment (NOT) for acute uncomplicated appendicitis versus urgent appendectomy?
Background
Acute appendicitis is found in around 5% of children presenting for urgent or emergent evaluation of abdominal pain. It is the most common illness prompting emergency abdominal surgery in children.
Several trials in adults have shown that urgent surgery may not be necessary, and NOT of uncomplicated appendicitis may be both effective and safe. NOT involves a course of IV antibiotics and careful clinical monitoring while hospitalized, then a course of oral antibiotics after discharge. Regimens vary but include coverage for aerobic and anaerobic gut flora, such as piperacillin-tazobactam followed by amoxicillin. Little is known about the safety and efficacy of NOT in children.
Study design
Meta-analysis.
Search strategy
PubMed, MEDLINE, EMBASE, and Cochrane Library were searched for relevant studies. This search identified 527 potential articles, of which the authors examined the full text of 68 and ultimately identified 5 single-center trials for analysis (4 prospective cohort trials and 1 randomized, controlled trial).
Synopsis
A total of 404 patients with uncomplicated appendicitis were seen in all trials: 168 received NOT and 236 received standard surgical care (urgent appendectomy). In the single randomized, controlled trial, patients were assigned NOT or surgical care randomly. In the other trials parental preference directed therapy.
The heterogeneity of the design, populations, definitions of illness, duration of follow-up, and NOT treatment regimens made the meta-analysis challenging. Antibiotic options for NOT varied by center but included a course of IV antibiotics followed by 7-10 days of oral antibiotics. NOT success was defined as no need for surgery within 48 hours and no recurrence of appendicitis within 1 month. Of the 236 patients who received standard surgical care, all had appendicitis and 1 had a complication requiring repeat operation. Of the NOT group, 16 (9.5%) had treatment failures, including 3 with perforated appendicitis, and 45 (27%) went on to have an appendectomy within the following year, yielding a risk ratio of failure versus standard treatment of 8.9 (95% confidence interval, 2.7-29.8). A subgroup analysis of patients with appendicoliths who received NOT found that these patients experienced a substantially increased risk of treatment failures and recurrent appendicitis with the risk ratio versus NOT without appendicolith of 10.4 (95% CI, 1.5-74). Of the 30 patients who experienced treatment failure with NOT, 15 had appendicoliths. NOT lengthened hospital stays by 14.3 hours (95% CI, 7.5-21.1) but led to lower total costs by $1,310 (95% CI, $920-$1,690).
Bottom line
NOT may be a reasonable alternative to standard surgical management for acute uncomplicated appendicitis without appendicolith in children, with a success rate of greater than 90%. Further larger, randomized prospective studies are required to establish its safety and efficacy.
Citation
Huang L et al. Comparison of antibiotic therapy and appendectomy for acute uncomplicated appendicitis in children: A meta-analysis. JAMA Pediatr. 2017;171(5):426-34.
Dr. Stubblefield is a pediatric hospitalist at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Del., and a clinical assistant professor of pediatrics at Jefferson Medical College in Philadelphia.
Clinical question
What are the differences in rates of treatment failure, duration of hospitalization, and cost between nonoperative treatment (NOT) for acute uncomplicated appendicitis versus urgent appendectomy?
Background
Acute appendicitis is found in around 5% of children presenting for urgent or emergent evaluation of abdominal pain. It is the most common illness prompting emergency abdominal surgery in children.
Several trials in adults have shown that urgent surgery may not be necessary, and NOT of uncomplicated appendicitis may be both effective and safe. NOT involves a course of IV antibiotics and careful clinical monitoring while hospitalized, then a course of oral antibiotics after discharge. Regimens vary but include coverage for aerobic and anaerobic gut flora, such as piperacillin-tazobactam followed by amoxicillin. Little is known about the safety and efficacy of NOT in children.
Study design
Meta-analysis.
Search strategy
PubMed, MEDLINE, EMBASE, and Cochrane Library were searched for relevant studies. This search identified 527 potential articles, of which the authors examined the full text of 68 and ultimately identified 5 single-center trials for analysis (4 prospective cohort trials and 1 randomized, controlled trial).
Synopsis
A total of 404 patients with uncomplicated appendicitis were seen in all trials: 168 received NOT and 236 received standard surgical care (urgent appendectomy). In the single randomized, controlled trial, patients were assigned NOT or surgical care randomly. In the other trials parental preference directed therapy.
The heterogeneity of the design, populations, definitions of illness, duration of follow-up, and NOT treatment regimens made the meta-analysis challenging. Antibiotic options for NOT varied by center but included a course of IV antibiotics followed by 7-10 days of oral antibiotics. NOT success was defined as no need for surgery within 48 hours and no recurrence of appendicitis within 1 month. Of the 236 patients who received standard surgical care, all had appendicitis and 1 had a complication requiring repeat operation. Of the NOT group, 16 (9.5%) had treatment failures, including 3 with perforated appendicitis, and 45 (27%) went on to have an appendectomy within the following year, yielding a risk ratio of failure versus standard treatment of 8.9 (95% confidence interval, 2.7-29.8). A subgroup analysis of patients with appendicoliths who received NOT found that these patients experienced a substantially increased risk of treatment failures and recurrent appendicitis with the risk ratio versus NOT without appendicolith of 10.4 (95% CI, 1.5-74). Of the 30 patients who experienced treatment failure with NOT, 15 had appendicoliths. NOT lengthened hospital stays by 14.3 hours (95% CI, 7.5-21.1) but led to lower total costs by $1,310 (95% CI, $920-$1,690).
Bottom line
NOT may be a reasonable alternative to standard surgical management for acute uncomplicated appendicitis without appendicolith in children, with a success rate of greater than 90%. Further larger, randomized prospective studies are required to establish its safety and efficacy.
Citation
Huang L et al. Comparison of antibiotic therapy and appendectomy for acute uncomplicated appendicitis in children: A meta-analysis. JAMA Pediatr. 2017;171(5):426-34.
Dr. Stubblefield is a pediatric hospitalist at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Del., and a clinical assistant professor of pediatrics at Jefferson Medical College in Philadelphia.
Clinical question
What are the differences in rates of treatment failure, duration of hospitalization, and cost between nonoperative treatment (NOT) for acute uncomplicated appendicitis versus urgent appendectomy?
Background
Acute appendicitis is found in around 5% of children presenting for urgent or emergent evaluation of abdominal pain. It is the most common illness prompting emergency abdominal surgery in children.
Several trials in adults have shown that urgent surgery may not be necessary, and NOT of uncomplicated appendicitis may be both effective and safe. NOT involves a course of IV antibiotics and careful clinical monitoring while hospitalized, then a course of oral antibiotics after discharge. Regimens vary but include coverage for aerobic and anaerobic gut flora, such as piperacillin-tazobactam followed by amoxicillin. Little is known about the safety and efficacy of NOT in children.
Study design
Meta-analysis.
Search strategy
PubMed, MEDLINE, EMBASE, and Cochrane Library were searched for relevant studies. This search identified 527 potential articles, of which the authors examined the full text of 68 and ultimately identified 5 single-center trials for analysis (4 prospective cohort trials and 1 randomized, controlled trial).
Synopsis
A total of 404 patients with uncomplicated appendicitis were seen in all trials: 168 received NOT and 236 received standard surgical care (urgent appendectomy). In the single randomized, controlled trial, patients were assigned NOT or surgical care randomly. In the other trials parental preference directed therapy.
The heterogeneity of the design, populations, definitions of illness, duration of follow-up, and NOT treatment regimens made the meta-analysis challenging. Antibiotic options for NOT varied by center but included a course of IV antibiotics followed by 7-10 days of oral antibiotics. NOT success was defined as no need for surgery within 48 hours and no recurrence of appendicitis within 1 month. Of the 236 patients who received standard surgical care, all had appendicitis and 1 had a complication requiring repeat operation. Of the NOT group, 16 (9.5%) had treatment failures, including 3 with perforated appendicitis, and 45 (27%) went on to have an appendectomy within the following year, yielding a risk ratio of failure versus standard treatment of 8.9 (95% confidence interval, 2.7-29.8). A subgroup analysis of patients with appendicoliths who received NOT found that these patients experienced a substantially increased risk of treatment failures and recurrent appendicitis with the risk ratio versus NOT without appendicolith of 10.4 (95% CI, 1.5-74). Of the 30 patients who experienced treatment failure with NOT, 15 had appendicoliths. NOT lengthened hospital stays by 14.3 hours (95% CI, 7.5-21.1) but led to lower total costs by $1,310 (95% CI, $920-$1,690).
Bottom line
NOT may be a reasonable alternative to standard surgical management for acute uncomplicated appendicitis without appendicolith in children, with a success rate of greater than 90%. Further larger, randomized prospective studies are required to establish its safety and efficacy.
Citation
Huang L et al. Comparison of antibiotic therapy and appendectomy for acute uncomplicated appendicitis in children: A meta-analysis. JAMA Pediatr. 2017;171(5):426-34.
Dr. Stubblefield is a pediatric hospitalist at Nemours/Alfred I. duPont Hospital for Children in Wilmington, Del., and a clinical assistant professor of pediatrics at Jefferson Medical College in Philadelphia.
Emerging treatments tackling hair loss challenges include light therapies
according to Maria Hordinsky, MD.
“Although the precise mechanism remains unclear, it has been postulated that photobiomodulation acts through oxidative metabolism and transcription factor stimulation,” Dr. Hordinsky said in a presentation at Skin Disease Education Foundation’s Women’s & Pediatric Dermatology Seminar.
She referred to one trial, which found that men with androgenetic alopecia who used the HairMax Lasercomb showed an increase in mean hair density at 26 weeks of daily use, compared with a group that used a sham device.
Photobiomodulation devices use either laser light or light-emitting diodes. Comparing the two types is a challenge, and the question of which is more effective remains unanswered, Dr. Hordinsky said.
Other issues to be addressed in future research include finding the optimal wavelength to use for different indications for light-based treatments, determining whether pulse or continuous wave is more effective, and evaluating the potential for systemic side effects of these therapies, she noted.
No treatment for alopecia areata is currently approved by the Food and Drug Administration, but factors to consider when choosing a treatment include the patient’s age, location and extent of hair loss, and the presence of other medical problems, as well as a scalp biopsy report with information on the hair cycle and inflammation. Patients and/or their parents should understand the risks and benefits associated with various treatments to make an informed decision, Dr. Hordinsky said.
Patients and their families “have heard the ‘buzz’ about potential new treatments for alopecia areata, and the discussion needs to include a conversation about ongoing and future clinical research opportunities, as well as off-label use of Janus kinase inhibitors,” particularly oral tofacitinib, she said.
Approximately two-thirds of patients in recent studies of oral tofacitinib have had clinically acceptable hair regrowth after 6 months, Dr. Hordinsky said. Ruxolitinib is also being studied. However, “until clinical research studies are completed, there will be ongoing debate regarding the risks and benefits, cost, and sustainability” of JAK inhibitors or other new treatments, she said.
Dr. Hordinsky disclosed that she is a consultant for companies including Procter & Gamble and Concert, and has received grant/research support from Incyte, Allergan, and the National Alopecia Areata Foundation.
SDEF and this news organization are owned by Frontline Medical Communications.
according to Maria Hordinsky, MD.
“Although the precise mechanism remains unclear, it has been postulated that photobiomodulation acts through oxidative metabolism and transcription factor stimulation,” Dr. Hordinsky said in a presentation at Skin Disease Education Foundation’s Women’s & Pediatric Dermatology Seminar.
She referred to one trial, which found that men with androgenetic alopecia who used the HairMax Lasercomb showed an increase in mean hair density at 26 weeks of daily use, compared with a group that used a sham device.
Photobiomodulation devices use either laser light or light-emitting diodes. Comparing the two types is a challenge, and the question of which is more effective remains unanswered, Dr. Hordinsky said.
Other issues to be addressed in future research include finding the optimal wavelength to use for different indications for light-based treatments, determining whether pulse or continuous wave is more effective, and evaluating the potential for systemic side effects of these therapies, she noted.
No treatment for alopecia areata is currently approved by the Food and Drug Administration, but factors to consider when choosing a treatment include the patient’s age, location and extent of hair loss, and the presence of other medical problems, as well as a scalp biopsy report with information on the hair cycle and inflammation. Patients and/or their parents should understand the risks and benefits associated with various treatments to make an informed decision, Dr. Hordinsky said.
Patients and their families “have heard the ‘buzz’ about potential new treatments for alopecia areata, and the discussion needs to include a conversation about ongoing and future clinical research opportunities, as well as off-label use of Janus kinase inhibitors,” particularly oral tofacitinib, she said.
Approximately two-thirds of patients in recent studies of oral tofacitinib have had clinically acceptable hair regrowth after 6 months, Dr. Hordinsky said. Ruxolitinib is also being studied. However, “until clinical research studies are completed, there will be ongoing debate regarding the risks and benefits, cost, and sustainability” of JAK inhibitors or other new treatments, she said.
Dr. Hordinsky disclosed that she is a consultant for companies including Procter & Gamble and Concert, and has received grant/research support from Incyte, Allergan, and the National Alopecia Areata Foundation.
SDEF and this news organization are owned by Frontline Medical Communications.
according to Maria Hordinsky, MD.
“Although the precise mechanism remains unclear, it has been postulated that photobiomodulation acts through oxidative metabolism and transcription factor stimulation,” Dr. Hordinsky said in a presentation at Skin Disease Education Foundation’s Women’s & Pediatric Dermatology Seminar.
She referred to one trial, which found that men with androgenetic alopecia who used the HairMax Lasercomb showed an increase in mean hair density at 26 weeks of daily use, compared with a group that used a sham device.
Photobiomodulation devices use either laser light or light-emitting diodes. Comparing the two types is a challenge, and the question of which is more effective remains unanswered, Dr. Hordinsky said.
Other issues to be addressed in future research include finding the optimal wavelength to use for different indications for light-based treatments, determining whether pulse or continuous wave is more effective, and evaluating the potential for systemic side effects of these therapies, she noted.
No treatment for alopecia areata is currently approved by the Food and Drug Administration, but factors to consider when choosing a treatment include the patient’s age, location and extent of hair loss, and the presence of other medical problems, as well as a scalp biopsy report with information on the hair cycle and inflammation. Patients and/or their parents should understand the risks and benefits associated with various treatments to make an informed decision, Dr. Hordinsky said.
Patients and their families “have heard the ‘buzz’ about potential new treatments for alopecia areata, and the discussion needs to include a conversation about ongoing and future clinical research opportunities, as well as off-label use of Janus kinase inhibitors,” particularly oral tofacitinib, she said.
Approximately two-thirds of patients in recent studies of oral tofacitinib have had clinically acceptable hair regrowth after 6 months, Dr. Hordinsky said. Ruxolitinib is also being studied. However, “until clinical research studies are completed, there will be ongoing debate regarding the risks and benefits, cost, and sustainability” of JAK inhibitors or other new treatments, she said.
Dr. Hordinsky disclosed that she is a consultant for companies including Procter & Gamble and Concert, and has received grant/research support from Incyte, Allergan, and the National Alopecia Areata Foundation.
SDEF and this news organization are owned by Frontline Medical Communications.
FROM SDEF WOMEN’S & PEDIATRIC DERMATOLOGY SEMINAR
FDA: Puerto Rico’s medical supply shortage issues continue
“In addition to our ongoing concerns related to IV saline products, we also are particularly focused on the shortage of amino acids for injection,” FDA Commissioner Scott Gottlieb, MD, said in a statement.
The shortage of amino acids for injection predates Hurricane Maria, but the hurricane’s impact worsened the situation by disrupting Baxter’s ability to manufacture amino acids in Puerto Rico. The FDA has worked with Baxter to temporarily import pediatric and adult amino acid injections from the United Kingdom and Italy, as well as working with other amino acid manufacturers to increase supplies.
“We continue to work closely with federal and Puerto Rican authorities to address the needs of manufacturers on the island for power and other resources. These efforts have been focused on the needs of patients – to prevent potential shortages of medically important products where possible, and help ensure that any shortages that do occur are mitigated as quickly as possible,” Dr. Gottlieb said.
Read the full statement on the FDA website.
“In addition to our ongoing concerns related to IV saline products, we also are particularly focused on the shortage of amino acids for injection,” FDA Commissioner Scott Gottlieb, MD, said in a statement.
The shortage of amino acids for injection predates Hurricane Maria, but the hurricane’s impact worsened the situation by disrupting Baxter’s ability to manufacture amino acids in Puerto Rico. The FDA has worked with Baxter to temporarily import pediatric and adult amino acid injections from the United Kingdom and Italy, as well as working with other amino acid manufacturers to increase supplies.
“We continue to work closely with federal and Puerto Rican authorities to address the needs of manufacturers on the island for power and other resources. These efforts have been focused on the needs of patients – to prevent potential shortages of medically important products where possible, and help ensure that any shortages that do occur are mitigated as quickly as possible,” Dr. Gottlieb said.
Read the full statement on the FDA website.
“In addition to our ongoing concerns related to IV saline products, we also are particularly focused on the shortage of amino acids for injection,” FDA Commissioner Scott Gottlieb, MD, said in a statement.
The shortage of amino acids for injection predates Hurricane Maria, but the hurricane’s impact worsened the situation by disrupting Baxter’s ability to manufacture amino acids in Puerto Rico. The FDA has worked with Baxter to temporarily import pediatric and adult amino acid injections from the United Kingdom and Italy, as well as working with other amino acid manufacturers to increase supplies.
“We continue to work closely with federal and Puerto Rican authorities to address the needs of manufacturers on the island for power and other resources. These efforts have been focused on the needs of patients – to prevent potential shortages of medically important products where possible, and help ensure that any shortages that do occur are mitigated as quickly as possible,” Dr. Gottlieb said.
Read the full statement on the FDA website.
How to decide which ‘birthmarks’ spell trouble
When evaluating lumps and bumps in infants, categorizing them can help determine whether they need immediate attention, said James R. Treat, MD, a pediatric dermatologist at Children’s Hospital of Philadelphia, Pennsylvania.
“Divide ‘birthmarks’ based on appearance, “then decide when to worry,” he said in a presentation at Skin Disease Education Foundation’s Women’s & Pediatric Dermatology Seminar.
For example, Spitz nevi occur in patients younger than 20 years, most often on the face and lower extremities, but most are benign, Dr. Treat said. However, he recommends a biopsy if the patient is pubertal or older, or if the lesions are larger than 8 mm, amelanotic, or show asymmetry, ulceration, or excessive growth.
By contrast, neurocutaneous melanosis is a rare but serious skin condition that occurs in children and can be fatal if it progresses to melanoma, he pointed out. The condition involves the migration of melanocytes into the spinal canal and cerebrospinal fluid during development. Symptoms may include headache, seizures, and paralysis, and clinicians should keep them in mind when seeing children with melanocytic nevi, he noted. The highest risk for melanoma transformation is increased for individuals with more than 20 congenital moles, and “the second-highest risk is having a giant nevus lying overtop of the midline spine or scalp,” he said.
In some cases, yellow or tan lesions in children are benign and will resolve on their own, Dr. Treat said.
Juvenile xanthogranuloma (JXG), characterized by yellow-brown asymptomatic papules and nodules, develops most often within the first year of life, but the lesions usually resolve spontaneously by school age, he added.
Mastocytosis, localized collections of mast cells, presents as yellow/tan lesions that develop within the first 2 years of life. The condition can be systemic; patients may experience flushing and diarrhea because of localized release of histamines, and those with a history of weight loss, easy bruising or bleeding, hepatosplenomegaly, or lymphadenopathy may have systemic disease, Dr. Treat explained.
Subcutaneous fat necrosis can present within the first 2 weeks of life as firm, red-purple tender nodules that may be disturbing to parents. These lesions are most likely to appear on the cheeks, arms, back, and thighs, and are related to hypoxia or trauma, he added. The lesions usually resolve spontaneously within a period of weeks to months, although they may heal with some atrophy and scarring, he said. Subcutaneous fat necrosis is associated with hypercalcemia, so “it is important to check frequently, as hypercalcemia can occur weeks after the nodules resolve,” he commented.
Dr. Treat disclosed serving as a consultant to Procter & Gamble. SDEF and this news organization are owned by Frontline Medical Communications.
When evaluating lumps and bumps in infants, categorizing them can help determine whether they need immediate attention, said James R. Treat, MD, a pediatric dermatologist at Children’s Hospital of Philadelphia, Pennsylvania.
“Divide ‘birthmarks’ based on appearance, “then decide when to worry,” he said in a presentation at Skin Disease Education Foundation’s Women’s & Pediatric Dermatology Seminar.
For example, Spitz nevi occur in patients younger than 20 years, most often on the face and lower extremities, but most are benign, Dr. Treat said. However, he recommends a biopsy if the patient is pubertal or older, or if the lesions are larger than 8 mm, amelanotic, or show asymmetry, ulceration, or excessive growth.
By contrast, neurocutaneous melanosis is a rare but serious skin condition that occurs in children and can be fatal if it progresses to melanoma, he pointed out. The condition involves the migration of melanocytes into the spinal canal and cerebrospinal fluid during development. Symptoms may include headache, seizures, and paralysis, and clinicians should keep them in mind when seeing children with melanocytic nevi, he noted. The highest risk for melanoma transformation is increased for individuals with more than 20 congenital moles, and “the second-highest risk is having a giant nevus lying overtop of the midline spine or scalp,” he said.
In some cases, yellow or tan lesions in children are benign and will resolve on their own, Dr. Treat said.
Juvenile xanthogranuloma (JXG), characterized by yellow-brown asymptomatic papules and nodules, develops most often within the first year of life, but the lesions usually resolve spontaneously by school age, he added.
Mastocytosis, localized collections of mast cells, presents as yellow/tan lesions that develop within the first 2 years of life. The condition can be systemic; patients may experience flushing and diarrhea because of localized release of histamines, and those with a history of weight loss, easy bruising or bleeding, hepatosplenomegaly, or lymphadenopathy may have systemic disease, Dr. Treat explained.
Subcutaneous fat necrosis can present within the first 2 weeks of life as firm, red-purple tender nodules that may be disturbing to parents. These lesions are most likely to appear on the cheeks, arms, back, and thighs, and are related to hypoxia or trauma, he added. The lesions usually resolve spontaneously within a period of weeks to months, although they may heal with some atrophy and scarring, he said. Subcutaneous fat necrosis is associated with hypercalcemia, so “it is important to check frequently, as hypercalcemia can occur weeks after the nodules resolve,” he commented.
Dr. Treat disclosed serving as a consultant to Procter & Gamble. SDEF and this news organization are owned by Frontline Medical Communications.
When evaluating lumps and bumps in infants, categorizing them can help determine whether they need immediate attention, said James R. Treat, MD, a pediatric dermatologist at Children’s Hospital of Philadelphia, Pennsylvania.
“Divide ‘birthmarks’ based on appearance, “then decide when to worry,” he said in a presentation at Skin Disease Education Foundation’s Women’s & Pediatric Dermatology Seminar.
For example, Spitz nevi occur in patients younger than 20 years, most often on the face and lower extremities, but most are benign, Dr. Treat said. However, he recommends a biopsy if the patient is pubertal or older, or if the lesions are larger than 8 mm, amelanotic, or show asymmetry, ulceration, or excessive growth.
By contrast, neurocutaneous melanosis is a rare but serious skin condition that occurs in children and can be fatal if it progresses to melanoma, he pointed out. The condition involves the migration of melanocytes into the spinal canal and cerebrospinal fluid during development. Symptoms may include headache, seizures, and paralysis, and clinicians should keep them in mind when seeing children with melanocytic nevi, he noted. The highest risk for melanoma transformation is increased for individuals with more than 20 congenital moles, and “the second-highest risk is having a giant nevus lying overtop of the midline spine or scalp,” he said.
In some cases, yellow or tan lesions in children are benign and will resolve on their own, Dr. Treat said.
Juvenile xanthogranuloma (JXG), characterized by yellow-brown asymptomatic papules and nodules, develops most often within the first year of life, but the lesions usually resolve spontaneously by school age, he added.
Mastocytosis, localized collections of mast cells, presents as yellow/tan lesions that develop within the first 2 years of life. The condition can be systemic; patients may experience flushing and diarrhea because of localized release of histamines, and those with a history of weight loss, easy bruising or bleeding, hepatosplenomegaly, or lymphadenopathy may have systemic disease, Dr. Treat explained.
Subcutaneous fat necrosis can present within the first 2 weeks of life as firm, red-purple tender nodules that may be disturbing to parents. These lesions are most likely to appear on the cheeks, arms, back, and thighs, and are related to hypoxia or trauma, he added. The lesions usually resolve spontaneously within a period of weeks to months, although they may heal with some atrophy and scarring, he said. Subcutaneous fat necrosis is associated with hypercalcemia, so “it is important to check frequently, as hypercalcemia can occur weeks after the nodules resolve,” he commented.
Dr. Treat disclosed serving as a consultant to Procter & Gamble. SDEF and this news organization are owned by Frontline Medical Communications.
FROM SDEF WOMEN’S & PEDIATRIC DERMATOLOGY SEMINAR
Hospitalist movers and shakers – Nov. 2017
Pediatric hospitalist Patrick Conway, MD, has been named president and chief executive officer of Blue Cross and Blue Shield of North Carolina. Dr. Conway will take over for the retiring Brad Wilson on Oct. 1.
Dr. Conway is currently the deputy administrator for Innovation and Quality, and the director of the Center for Medicare and Medicaid Innovation for the Centers for Medicare and Medicaid Services (CMS). Previously, he was CMO at CMS, having served both the Obama and Trump administrations.
Hossam Hafez, MD, recently claimed the role of chief of Hospitalist Service with Health Quest Medical Practice (LaGrangeville, N.Y.). Dr. Hafez will be based out of Health Quest’s Vassar Brothers Medical Center in Poughkeepsie, N.Y., coordinating care in that hospital and throughout the Health Quest system.
Dr. Hafez has served full-time hospitalist stints with MidMichigan Health’s Physician Hospitalist Group, as well as with RiteMed Urgent Care. A native of Egypt, Dr. Hafez is fluent in both English and Arabic.
Caldwell UNC Healthcare (Lenoir, N.C.) has promoted David Lowry, MD, to chief medical officer as of Aug. 1, 2017.
Dr. Lowry, a longtime hospitalist and veteran in hospital medicine in general, will lead the building’s hospitalist program, support the chief of staff, and provide direct patient care, as well. He will serve as physician advisor for Caldwell’s Clinical Documentation, Utilization Review, Respiratory Care, and Rehabilitation departments.
Dr. Lowry boasts more than 25 years experience in hospital medicine and led in the creation of Caldwell’s hospitalist program. Since joining Caldwell, he has held leadership positions including chief of medicine. He received the hospital’s Donald D. McNeill Jr. Award for Outstanding Physician Leadership in 2014, as voted by his peers.
Joahd Toure, MD, recently was hired by Adirondack Health (Saranac Lake, N.Y.) as its new chief medical officer. He started his new position in late June 2017.
A Massachusetts native, Dr. Toure most recently worked as chief of hospitalist medicine with AdvantageCare Physicians in New York City. There, he helped manage care for patients in that system’s 16 hospitals in the New York metro area. Previously, he was regional medical director for Essex Inpatient Physicians (Boxford, Mass.) and a staff hospitalist at South Shore Hospital (South Weymouth, Mass.).
Longtime employee Emily Chapman, MD, has been promoted to chief medical officer and vice president of medical affairs at Children’s Minnesota Hospital (Minneapolis). The former vice CMO took on her new role on July 5, 2017.
Previously, Dr. Chapman served Children’s as its hospitalist program director, and as director of graduate medical education. She is an American Academy of Pediatrics Fellow.
Mark Sockell, MD, is the new chief medical officer at Meritage Medical Network in Novato, Calif. Meritage is a physician-run network that includes more than 700 board-certified physicians in both primary care and specialist fields.
Dr. Sockell has been a member of Meritage’s Board of Directors since 2014, and he specializes in risk adjustment and quality measures. His career has focused on medical education, headed by a stint as director of medical education at St. Mary’s Medical Center (San Francisco). There, he created and ran the inpatient hospitalist program.
Business Moves
Hammond-Henry Hospital (Geneseo, Ill.) recently announced the creation of a hospitalist program, utilizing the facility’s own emergency room physicians. Hammond-Henry will staff one emergency room doctor available for rounds outside of their ER work throughout the day.
The center’s hospitalist program will be led by medical director Kevin Jeffries, MD, who also will serve as one of the hospital’s ER physicians/hospitalists.
Avera Queen of Peace Hospital (Mitchell, S.D.) started its own hospitalist program on Aug. 1, 2017, launching with the goal of improving patient experience within the building. Avera’s hospitalists will be on-site for 12 hours each day, assisting specialists and working with patients who do not have a local primary care physician.
Queen of Peace is the regional referral center for an 11-county area, part of Avera Health System’s 330 facilities across North and South Dakota, Minnesota, Iowa, and Nebraska.
Pediatric hospitalist Patrick Conway, MD, has been named president and chief executive officer of Blue Cross and Blue Shield of North Carolina. Dr. Conway will take over for the retiring Brad Wilson on Oct. 1.
Dr. Conway is currently the deputy administrator for Innovation and Quality, and the director of the Center for Medicare and Medicaid Innovation for the Centers for Medicare and Medicaid Services (CMS). Previously, he was CMO at CMS, having served both the Obama and Trump administrations.
Hossam Hafez, MD, recently claimed the role of chief of Hospitalist Service with Health Quest Medical Practice (LaGrangeville, N.Y.). Dr. Hafez will be based out of Health Quest’s Vassar Brothers Medical Center in Poughkeepsie, N.Y., coordinating care in that hospital and throughout the Health Quest system.
Dr. Hafez has served full-time hospitalist stints with MidMichigan Health’s Physician Hospitalist Group, as well as with RiteMed Urgent Care. A native of Egypt, Dr. Hafez is fluent in both English and Arabic.
Caldwell UNC Healthcare (Lenoir, N.C.) has promoted David Lowry, MD, to chief medical officer as of Aug. 1, 2017.
Dr. Lowry, a longtime hospitalist and veteran in hospital medicine in general, will lead the building’s hospitalist program, support the chief of staff, and provide direct patient care, as well. He will serve as physician advisor for Caldwell’s Clinical Documentation, Utilization Review, Respiratory Care, and Rehabilitation departments.
Dr. Lowry boasts more than 25 years experience in hospital medicine and led in the creation of Caldwell’s hospitalist program. Since joining Caldwell, he has held leadership positions including chief of medicine. He received the hospital’s Donald D. McNeill Jr. Award for Outstanding Physician Leadership in 2014, as voted by his peers.
Joahd Toure, MD, recently was hired by Adirondack Health (Saranac Lake, N.Y.) as its new chief medical officer. He started his new position in late June 2017.
A Massachusetts native, Dr. Toure most recently worked as chief of hospitalist medicine with AdvantageCare Physicians in New York City. There, he helped manage care for patients in that system’s 16 hospitals in the New York metro area. Previously, he was regional medical director for Essex Inpatient Physicians (Boxford, Mass.) and a staff hospitalist at South Shore Hospital (South Weymouth, Mass.).
Longtime employee Emily Chapman, MD, has been promoted to chief medical officer and vice president of medical affairs at Children’s Minnesota Hospital (Minneapolis). The former vice CMO took on her new role on July 5, 2017.
Previously, Dr. Chapman served Children’s as its hospitalist program director, and as director of graduate medical education. She is an American Academy of Pediatrics Fellow.
Mark Sockell, MD, is the new chief medical officer at Meritage Medical Network in Novato, Calif. Meritage is a physician-run network that includes more than 700 board-certified physicians in both primary care and specialist fields.
Dr. Sockell has been a member of Meritage’s Board of Directors since 2014, and he specializes in risk adjustment and quality measures. His career has focused on medical education, headed by a stint as director of medical education at St. Mary’s Medical Center (San Francisco). There, he created and ran the inpatient hospitalist program.
Business Moves
Hammond-Henry Hospital (Geneseo, Ill.) recently announced the creation of a hospitalist program, utilizing the facility’s own emergency room physicians. Hammond-Henry will staff one emergency room doctor available for rounds outside of their ER work throughout the day.
The center’s hospitalist program will be led by medical director Kevin Jeffries, MD, who also will serve as one of the hospital’s ER physicians/hospitalists.
Avera Queen of Peace Hospital (Mitchell, S.D.) started its own hospitalist program on Aug. 1, 2017, launching with the goal of improving patient experience within the building. Avera’s hospitalists will be on-site for 12 hours each day, assisting specialists and working with patients who do not have a local primary care physician.
Queen of Peace is the regional referral center for an 11-county area, part of Avera Health System’s 330 facilities across North and South Dakota, Minnesota, Iowa, and Nebraska.
Pediatric hospitalist Patrick Conway, MD, has been named president and chief executive officer of Blue Cross and Blue Shield of North Carolina. Dr. Conway will take over for the retiring Brad Wilson on Oct. 1.
Dr. Conway is currently the deputy administrator for Innovation and Quality, and the director of the Center for Medicare and Medicaid Innovation for the Centers for Medicare and Medicaid Services (CMS). Previously, he was CMO at CMS, having served both the Obama and Trump administrations.
Hossam Hafez, MD, recently claimed the role of chief of Hospitalist Service with Health Quest Medical Practice (LaGrangeville, N.Y.). Dr. Hafez will be based out of Health Quest’s Vassar Brothers Medical Center in Poughkeepsie, N.Y., coordinating care in that hospital and throughout the Health Quest system.
Dr. Hafez has served full-time hospitalist stints with MidMichigan Health’s Physician Hospitalist Group, as well as with RiteMed Urgent Care. A native of Egypt, Dr. Hafez is fluent in both English and Arabic.
Caldwell UNC Healthcare (Lenoir, N.C.) has promoted David Lowry, MD, to chief medical officer as of Aug. 1, 2017.
Dr. Lowry, a longtime hospitalist and veteran in hospital medicine in general, will lead the building’s hospitalist program, support the chief of staff, and provide direct patient care, as well. He will serve as physician advisor for Caldwell’s Clinical Documentation, Utilization Review, Respiratory Care, and Rehabilitation departments.
Dr. Lowry boasts more than 25 years experience in hospital medicine and led in the creation of Caldwell’s hospitalist program. Since joining Caldwell, he has held leadership positions including chief of medicine. He received the hospital’s Donald D. McNeill Jr. Award for Outstanding Physician Leadership in 2014, as voted by his peers.
Joahd Toure, MD, recently was hired by Adirondack Health (Saranac Lake, N.Y.) as its new chief medical officer. He started his new position in late June 2017.
A Massachusetts native, Dr. Toure most recently worked as chief of hospitalist medicine with AdvantageCare Physicians in New York City. There, he helped manage care for patients in that system’s 16 hospitals in the New York metro area. Previously, he was regional medical director for Essex Inpatient Physicians (Boxford, Mass.) and a staff hospitalist at South Shore Hospital (South Weymouth, Mass.).
Longtime employee Emily Chapman, MD, has been promoted to chief medical officer and vice president of medical affairs at Children’s Minnesota Hospital (Minneapolis). The former vice CMO took on her new role on July 5, 2017.
Previously, Dr. Chapman served Children’s as its hospitalist program director, and as director of graduate medical education. She is an American Academy of Pediatrics Fellow.
Mark Sockell, MD, is the new chief medical officer at Meritage Medical Network in Novato, Calif. Meritage is a physician-run network that includes more than 700 board-certified physicians in both primary care and specialist fields.
Dr. Sockell has been a member of Meritage’s Board of Directors since 2014, and he specializes in risk adjustment and quality measures. His career has focused on medical education, headed by a stint as director of medical education at St. Mary’s Medical Center (San Francisco). There, he created and ran the inpatient hospitalist program.
Business Moves
Hammond-Henry Hospital (Geneseo, Ill.) recently announced the creation of a hospitalist program, utilizing the facility’s own emergency room physicians. Hammond-Henry will staff one emergency room doctor available for rounds outside of their ER work throughout the day.
The center’s hospitalist program will be led by medical director Kevin Jeffries, MD, who also will serve as one of the hospital’s ER physicians/hospitalists.
Avera Queen of Peace Hospital (Mitchell, S.D.) started its own hospitalist program on Aug. 1, 2017, launching with the goal of improving patient experience within the building. Avera’s hospitalists will be on-site for 12 hours each day, assisting specialists and working with patients who do not have a local primary care physician.
Queen of Peace is the regional referral center for an 11-county area, part of Avera Health System’s 330 facilities across North and South Dakota, Minnesota, Iowa, and Nebraska.
5 big ways the tax bill could affect health policy
Having failed to repeal and replace the Affordable Care Act, Congress is now working on a tax overhaul. But it turns out the tax bills in the House and Senate also aim to reshape health care.
Here are five big ways the tax bill could affect health policy:
1. Repeal the requirement for most people to have health insurance or pay a tax penalty
Republicans tried and failed to end the so-called individual mandate this year when they attempted to advance their health overhaul legislation. Now the idea is back, at least in the Senate’s version of the tax bill. The measure would not technically remove the requirement for people to have insurance, but it would eliminate the fine people would face if they choose to remain uninsured.
The Congressional Budget Office has estimated that dropping the requirement would result in 13 million fewer people having insurance over 10 years.
It also estimates that premiums would rise 10% more per year than they would without this change. That is because healthier people would be most likely to drop insurance in the absence of a fine, so insurers would have to raise premiums to compensate for a sicker group of customers. Those consumers, in turn, would be left with fewer affordable choices, according to the CBO.
State insurance officials are concerned that insurers will drop out of the individual market entirely if there is no requirement for healthy people to sign up, but they still have to sell to people who know they will need medical care.
Ironically, the states most likely to see this kind of insurance-market disruption are those that are reliably Republican. An analysis by the Los Angeles Times suggested that the states with the fewest insurers and the highest premiums – including Alaska, Iowa, Missouri, Nebraska, Nevada, and Wyoming – would be the ones left with either no coverage options or options too expensive for most consumers in the individual market.
2. Repeal the medical expense deduction
The House-passed tax bill, although not the Senate’s, would eliminate taxpayers’ ability to deduct medical expenses that exceed 10% of their adjusted gross income.
The medical expense deduction is not widely used – just under 9 million tax filers took it on their 2015 tax returns, according to the Internal Revenue Service. But those who do use it generally have very high medical expenses, often for a disabled child, a serious chronic illness, or expensive long-term care not covered by health insurance.
Among those most vehemently against getting rid of the deduction is the senior advocacy group AARP. Eliminating the deduction, the group said in a statement, “amounts to a health tax on millions of Americans with high medical costs – especially middle income seniors.”
3. Trigger major cuts to the Medicare program
The tax bill includes no specific Medicare changes, but budget analysts point out that passing it in its current form would trigger another law to kick in. That measure requires cuts to federal programs if the federal budget deficit is increased.
Because the tax bills in both the House and Senate would add an additional $1.5 trillion to the deficit over the next 10 years, both would result in automatic cuts under the Statutory Pay-As-You-Go Act of 2010 (PAYGO). According to the CBO, if Congress passes the tax bill and does not waive the PAYGO law, federal officials “would be required to issue a sequestration order within 15 days of the end of the session of Congress to reduce spending in fiscal year 2018 by the resultant total of $136 billion.”
Cuts to Medicare are limited under the PAYGO law, so the Medicare reduction would be limited to 4% of program spending, which is roughly $25 billion of that total. Cuts of a similar size would be required in future years. Most of that would likely come from payments to providers.
4. Change tax treatment for graduate students and those paying back student loans
The House bill, though not the Senate’s, would for the first time require graduate students to pay tax on the value of tuition that universities do not require them to pay.
Currently, graduate students in many fields, including science, often are paid a small stipend for teaching while they pursue advanced degrees. Many are technically charged tuition, but it is “waived” as long as they are working for the university.
The House tax bill would eliminate that waiver and require them to pay taxes on the full value of the tuition they don’t have to pay, which would result in many students with fairly low incomes seeing very large tax bills.
At the same time, the House tax bill would eliminate the deduction for interest paid on student loans. This would disproportionately affect young doctors.
According to the Association of American Medical Colleges, 75% of the medical school class of 2017 graduated with student loan debt, with nearly half owing $200,000 or more.
5. Change or eliminate the tax credit for rare disease drug development
Congress created the so-called Orphan Drug Credit in 1983, as part of a package of incentives intended to entice drugmakers to study and develop drugs to treat rare diseases, defined as those affecting fewer than 200,000 people. With such a small potential market, it does not otherwise make financial sense for the companies to spend the millions of dollars necessary to develop treatments for such ailments.
To date, about 500 drugs have come to market using the incentives, although in some cases drugmakers have manipulated the credit for extra financial gain.
The House tax bill would eliminate the tax credit; the Senate bill would scale it back. Sen. Orrin Hatch (R-Utah), chairman of the tax-writing Finance Committee, is one of the original sponsors of the orphan drug law.
The drug industry has been relatively quiet about the potential loss of the credit, but the National Organization for Rare Disorders called the change “wholly unacceptable” and said it “would directly result in 33% fewer orphan drugs coming to market.”
Kaiser Health News is a nonprofit news service covering health issues. It is an editorially independent program of the Kaiser Family Foundation that is not affiliated with Kaiser Permanente.
Having failed to repeal and replace the Affordable Care Act, Congress is now working on a tax overhaul. But it turns out the tax bills in the House and Senate also aim to reshape health care.
Here are five big ways the tax bill could affect health policy:
1. Repeal the requirement for most people to have health insurance or pay a tax penalty
Republicans tried and failed to end the so-called individual mandate this year when they attempted to advance their health overhaul legislation. Now the idea is back, at least in the Senate’s version of the tax bill. The measure would not technically remove the requirement for people to have insurance, but it would eliminate the fine people would face if they choose to remain uninsured.
The Congressional Budget Office has estimated that dropping the requirement would result in 13 million fewer people having insurance over 10 years.
It also estimates that premiums would rise 10% more per year than they would without this change. That is because healthier people would be most likely to drop insurance in the absence of a fine, so insurers would have to raise premiums to compensate for a sicker group of customers. Those consumers, in turn, would be left with fewer affordable choices, according to the CBO.
State insurance officials are concerned that insurers will drop out of the individual market entirely if there is no requirement for healthy people to sign up, but they still have to sell to people who know they will need medical care.
Ironically, the states most likely to see this kind of insurance-market disruption are those that are reliably Republican. An analysis by the Los Angeles Times suggested that the states with the fewest insurers and the highest premiums – including Alaska, Iowa, Missouri, Nebraska, Nevada, and Wyoming – would be the ones left with either no coverage options or options too expensive for most consumers in the individual market.
2. Repeal the medical expense deduction
The House-passed tax bill, although not the Senate’s, would eliminate taxpayers’ ability to deduct medical expenses that exceed 10% of their adjusted gross income.
The medical expense deduction is not widely used – just under 9 million tax filers took it on their 2015 tax returns, according to the Internal Revenue Service. But those who do use it generally have very high medical expenses, often for a disabled child, a serious chronic illness, or expensive long-term care not covered by health insurance.
Among those most vehemently against getting rid of the deduction is the senior advocacy group AARP. Eliminating the deduction, the group said in a statement, “amounts to a health tax on millions of Americans with high medical costs – especially middle income seniors.”
3. Trigger major cuts to the Medicare program
The tax bill includes no specific Medicare changes, but budget analysts point out that passing it in its current form would trigger another law to kick in. That measure requires cuts to federal programs if the federal budget deficit is increased.
Because the tax bills in both the House and Senate would add an additional $1.5 trillion to the deficit over the next 10 years, both would result in automatic cuts under the Statutory Pay-As-You-Go Act of 2010 (PAYGO). According to the CBO, if Congress passes the tax bill and does not waive the PAYGO law, federal officials “would be required to issue a sequestration order within 15 days of the end of the session of Congress to reduce spending in fiscal year 2018 by the resultant total of $136 billion.”
Cuts to Medicare are limited under the PAYGO law, so the Medicare reduction would be limited to 4% of program spending, which is roughly $25 billion of that total. Cuts of a similar size would be required in future years. Most of that would likely come from payments to providers.
4. Change tax treatment for graduate students and those paying back student loans
The House bill, though not the Senate’s, would for the first time require graduate students to pay tax on the value of tuition that universities do not require them to pay.
Currently, graduate students in many fields, including science, often are paid a small stipend for teaching while they pursue advanced degrees. Many are technically charged tuition, but it is “waived” as long as they are working for the university.
The House tax bill would eliminate that waiver and require them to pay taxes on the full value of the tuition they don’t have to pay, which would result in many students with fairly low incomes seeing very large tax bills.
At the same time, the House tax bill would eliminate the deduction for interest paid on student loans. This would disproportionately affect young doctors.
According to the Association of American Medical Colleges, 75% of the medical school class of 2017 graduated with student loan debt, with nearly half owing $200,000 or more.
5. Change or eliminate the tax credit for rare disease drug development
Congress created the so-called Orphan Drug Credit in 1983, as part of a package of incentives intended to entice drugmakers to study and develop drugs to treat rare diseases, defined as those affecting fewer than 200,000 people. With such a small potential market, it does not otherwise make financial sense for the companies to spend the millions of dollars necessary to develop treatments for such ailments.
To date, about 500 drugs have come to market using the incentives, although in some cases drugmakers have manipulated the credit for extra financial gain.
The House tax bill would eliminate the tax credit; the Senate bill would scale it back. Sen. Orrin Hatch (R-Utah), chairman of the tax-writing Finance Committee, is one of the original sponsors of the orphan drug law.
The drug industry has been relatively quiet about the potential loss of the credit, but the National Organization for Rare Disorders called the change “wholly unacceptable” and said it “would directly result in 33% fewer orphan drugs coming to market.”
Kaiser Health News is a nonprofit news service covering health issues. It is an editorially independent program of the Kaiser Family Foundation that is not affiliated with Kaiser Permanente.
Having failed to repeal and replace the Affordable Care Act, Congress is now working on a tax overhaul. But it turns out the tax bills in the House and Senate also aim to reshape health care.
Here are five big ways the tax bill could affect health policy:
1. Repeal the requirement for most people to have health insurance or pay a tax penalty
Republicans tried and failed to end the so-called individual mandate this year when they attempted to advance their health overhaul legislation. Now the idea is back, at least in the Senate’s version of the tax bill. The measure would not technically remove the requirement for people to have insurance, but it would eliminate the fine people would face if they choose to remain uninsured.
The Congressional Budget Office has estimated that dropping the requirement would result in 13 million fewer people having insurance over 10 years.
It also estimates that premiums would rise 10% more per year than they would without this change. That is because healthier people would be most likely to drop insurance in the absence of a fine, so insurers would have to raise premiums to compensate for a sicker group of customers. Those consumers, in turn, would be left with fewer affordable choices, according to the CBO.
State insurance officials are concerned that insurers will drop out of the individual market entirely if there is no requirement for healthy people to sign up, but they still have to sell to people who know they will need medical care.
Ironically, the states most likely to see this kind of insurance-market disruption are those that are reliably Republican. An analysis by the Los Angeles Times suggested that the states with the fewest insurers and the highest premiums – including Alaska, Iowa, Missouri, Nebraska, Nevada, and Wyoming – would be the ones left with either no coverage options or options too expensive for most consumers in the individual market.
2. Repeal the medical expense deduction
The House-passed tax bill, although not the Senate’s, would eliminate taxpayers’ ability to deduct medical expenses that exceed 10% of their adjusted gross income.
The medical expense deduction is not widely used – just under 9 million tax filers took it on their 2015 tax returns, according to the Internal Revenue Service. But those who do use it generally have very high medical expenses, often for a disabled child, a serious chronic illness, or expensive long-term care not covered by health insurance.
Among those most vehemently against getting rid of the deduction is the senior advocacy group AARP. Eliminating the deduction, the group said in a statement, “amounts to a health tax on millions of Americans with high medical costs – especially middle income seniors.”
3. Trigger major cuts to the Medicare program
The tax bill includes no specific Medicare changes, but budget analysts point out that passing it in its current form would trigger another law to kick in. That measure requires cuts to federal programs if the federal budget deficit is increased.
Because the tax bills in both the House and Senate would add an additional $1.5 trillion to the deficit over the next 10 years, both would result in automatic cuts under the Statutory Pay-As-You-Go Act of 2010 (PAYGO). According to the CBO, if Congress passes the tax bill and does not waive the PAYGO law, federal officials “would be required to issue a sequestration order within 15 days of the end of the session of Congress to reduce spending in fiscal year 2018 by the resultant total of $136 billion.”
Cuts to Medicare are limited under the PAYGO law, so the Medicare reduction would be limited to 4% of program spending, which is roughly $25 billion of that total. Cuts of a similar size would be required in future years. Most of that would likely come from payments to providers.
4. Change tax treatment for graduate students and those paying back student loans
The House bill, though not the Senate’s, would for the first time require graduate students to pay tax on the value of tuition that universities do not require them to pay.
Currently, graduate students in many fields, including science, often are paid a small stipend for teaching while they pursue advanced degrees. Many are technically charged tuition, but it is “waived” as long as they are working for the university.
The House tax bill would eliminate that waiver and require them to pay taxes on the full value of the tuition they don’t have to pay, which would result in many students with fairly low incomes seeing very large tax bills.
At the same time, the House tax bill would eliminate the deduction for interest paid on student loans. This would disproportionately affect young doctors.
According to the Association of American Medical Colleges, 75% of the medical school class of 2017 graduated with student loan debt, with nearly half owing $200,000 or more.
5. Change or eliminate the tax credit for rare disease drug development
Congress created the so-called Orphan Drug Credit in 1983, as part of a package of incentives intended to entice drugmakers to study and develop drugs to treat rare diseases, defined as those affecting fewer than 200,000 people. With such a small potential market, it does not otherwise make financial sense for the companies to spend the millions of dollars necessary to develop treatments for such ailments.
To date, about 500 drugs have come to market using the incentives, although in some cases drugmakers have manipulated the credit for extra financial gain.
The House tax bill would eliminate the tax credit; the Senate bill would scale it back. Sen. Orrin Hatch (R-Utah), chairman of the tax-writing Finance Committee, is one of the original sponsors of the orphan drug law.
The drug industry has been relatively quiet about the potential loss of the credit, but the National Organization for Rare Disorders called the change “wholly unacceptable” and said it “would directly result in 33% fewer orphan drugs coming to market.”
Kaiser Health News is a nonprofit news service covering health issues. It is an editorially independent program of the Kaiser Family Foundation that is not affiliated with Kaiser Permanente.
Introducing The Sarcoma Journal—The Official Journal of the Sarcoma Foundation of America ™ : An Exciting Initiative in Peer-Reviewed Professional Education and Patient Advocacy
The Sarcoma Journal — Official Journal of the Sarcoma Foundation of America™ represents a new and exciting initiative in professional education. We invite you to share in the excitement surrounding the launch of a medical journal designed to be your most authoritative and comprehensive source of scientific information on the diagnosis and treatment of sarcomas and sarcoma sub-types.
On behalf of myself, our editorial board, and editorial staff, I welcome you to this journal as we explore new treatment paradigms for this disease, translational research that bridges the bench and the clinic, and a broad range of science to encompass the many facets of sarcoma. In my opinion, the startup of this publication could not come at a better time.
As cancer specialists and allied health care professionals who attend regular meetings of your peers, including ASCO and CTOS, we have seen a dramatic shift in management within the last few years. In many ways we are at a threshold of a new era in sarcoma management, and the spectrum of treatment is expanding across subspecialties, promising more effective strategies for our patients that are based on an improved understanding of disease biology. We need a resource to maintain and clarify our focus on this disease as research opens new avenues for us to consider in the management of patients with sarcoma.
When I was approached to serve as Editor-in-Chief of The Sarcoma Journal by the Sarcoma Foundation of America, I began to recruit an esteemed group of colleagues whose knowledge, worldwide reputation as thought leaders, and dedicated work as researchers would reflect our commitment toward finding a cure for sarcoma. Many of the colleagues who will join me on the Editorial Advisory Board have long-standing affiliations with the Sarcoma Foundation of America and its comprehensive program of sarcoma research, patient support and education and advocacy. As you explore the first issue of the journal, you will discover how our editorial content is an extension of this three-tiered approach. The SFA program is characterized by a multi-dimensional and uniquely coordinated outreach program of videos and webinars, websites (a new journal website is launching as well) a sarcoma-specific clinical trials database, newsletters and related materials— all aimed ultimately at finding a cure for this disease. This professional journal complements and extends the SFA’s mission.
Although The Sarcoma Journal has a position within the SFA umbrella, my focus is foremost on ensuring that The Sarcoma Journal contains the most accurate, relevant and up to date information available. I urge you to explore our highly informative and relevant sarcoma-specific content—including original reports, review articles, a Journal Club, expert opinion, meeting reports, and patient advocacy that encapsulates the latest findings from the bench with implications for the bedside.
Whether it is discussing the latest findings in advanced sarcoma sub-types or implications of genetics as a prognostic factor, you will find the information in this journal, reliably analyzed by our team of experts who are leading sarcoma clinicians and investigators. All of the content we provide is presented in a thought-provoking, lively and peer-reviewed format; we welcome your comments and suggestions to keep us on the forefront of patient care as we cover a rapidly evolving landscape of new information in the treatment of sarcomas and frame it within a context directly applicable to enhancing the quality of patient care.
The Sarcoma Journal — Official Journal of the Sarcoma Foundation of America™ represents a new and exciting initiative in professional education. We invite you to share in the excitement surrounding the launch of a medical journal designed to be your most authoritative and comprehensive source of scientific information on the diagnosis and treatment of sarcomas and sarcoma sub-types.
On behalf of myself, our editorial board, and editorial staff, I welcome you to this journal as we explore new treatment paradigms for this disease, translational research that bridges the bench and the clinic, and a broad range of science to encompass the many facets of sarcoma. In my opinion, the startup of this publication could not come at a better time.
As cancer specialists and allied health care professionals who attend regular meetings of your peers, including ASCO and CTOS, we have seen a dramatic shift in management within the last few years. In many ways we are at a threshold of a new era in sarcoma management, and the spectrum of treatment is expanding across subspecialties, promising more effective strategies for our patients that are based on an improved understanding of disease biology. We need a resource to maintain and clarify our focus on this disease as research opens new avenues for us to consider in the management of patients with sarcoma.
When I was approached to serve as Editor-in-Chief of The Sarcoma Journal by the Sarcoma Foundation of America, I began to recruit an esteemed group of colleagues whose knowledge, worldwide reputation as thought leaders, and dedicated work as researchers would reflect our commitment toward finding a cure for sarcoma. Many of the colleagues who will join me on the Editorial Advisory Board have long-standing affiliations with the Sarcoma Foundation of America and its comprehensive program of sarcoma research, patient support and education and advocacy. As you explore the first issue of the journal, you will discover how our editorial content is an extension of this three-tiered approach. The SFA program is characterized by a multi-dimensional and uniquely coordinated outreach program of videos and webinars, websites (a new journal website is launching as well) a sarcoma-specific clinical trials database, newsletters and related materials— all aimed ultimately at finding a cure for this disease. This professional journal complements and extends the SFA’s mission.
Although The Sarcoma Journal has a position within the SFA umbrella, my focus is foremost on ensuring that The Sarcoma Journal contains the most accurate, relevant and up to date information available. I urge you to explore our highly informative and relevant sarcoma-specific content—including original reports, review articles, a Journal Club, expert opinion, meeting reports, and patient advocacy that encapsulates the latest findings from the bench with implications for the bedside.
Whether it is discussing the latest findings in advanced sarcoma sub-types or implications of genetics as a prognostic factor, you will find the information in this journal, reliably analyzed by our team of experts who are leading sarcoma clinicians and investigators. All of the content we provide is presented in a thought-provoking, lively and peer-reviewed format; we welcome your comments and suggestions to keep us on the forefront of patient care as we cover a rapidly evolving landscape of new information in the treatment of sarcomas and frame it within a context directly applicable to enhancing the quality of patient care.
The Sarcoma Journal — Official Journal of the Sarcoma Foundation of America™ represents a new and exciting initiative in professional education. We invite you to share in the excitement surrounding the launch of a medical journal designed to be your most authoritative and comprehensive source of scientific information on the diagnosis and treatment of sarcomas and sarcoma sub-types.
On behalf of myself, our editorial board, and editorial staff, I welcome you to this journal as we explore new treatment paradigms for this disease, translational research that bridges the bench and the clinic, and a broad range of science to encompass the many facets of sarcoma. In my opinion, the startup of this publication could not come at a better time.
As cancer specialists and allied health care professionals who attend regular meetings of your peers, including ASCO and CTOS, we have seen a dramatic shift in management within the last few years. In many ways we are at a threshold of a new era in sarcoma management, and the spectrum of treatment is expanding across subspecialties, promising more effective strategies for our patients that are based on an improved understanding of disease biology. We need a resource to maintain and clarify our focus on this disease as research opens new avenues for us to consider in the management of patients with sarcoma.
When I was approached to serve as Editor-in-Chief of The Sarcoma Journal by the Sarcoma Foundation of America, I began to recruit an esteemed group of colleagues whose knowledge, worldwide reputation as thought leaders, and dedicated work as researchers would reflect our commitment toward finding a cure for sarcoma. Many of the colleagues who will join me on the Editorial Advisory Board have long-standing affiliations with the Sarcoma Foundation of America and its comprehensive program of sarcoma research, patient support and education and advocacy. As you explore the first issue of the journal, you will discover how our editorial content is an extension of this three-tiered approach. The SFA program is characterized by a multi-dimensional and uniquely coordinated outreach program of videos and webinars, websites (a new journal website is launching as well) a sarcoma-specific clinical trials database, newsletters and related materials— all aimed ultimately at finding a cure for this disease. This professional journal complements and extends the SFA’s mission.
Although The Sarcoma Journal has a position within the SFA umbrella, my focus is foremost on ensuring that The Sarcoma Journal contains the most accurate, relevant and up to date information available. I urge you to explore our highly informative and relevant sarcoma-specific content—including original reports, review articles, a Journal Club, expert opinion, meeting reports, and patient advocacy that encapsulates the latest findings from the bench with implications for the bedside.
Whether it is discussing the latest findings in advanced sarcoma sub-types or implications of genetics as a prognostic factor, you will find the information in this journal, reliably analyzed by our team of experts who are leading sarcoma clinicians and investigators. All of the content we provide is presented in a thought-provoking, lively and peer-reviewed format; we welcome your comments and suggestions to keep us on the forefront of patient care as we cover a rapidly evolving landscape of new information in the treatment of sarcomas and frame it within a context directly applicable to enhancing the quality of patient care.
Pulmonary sarcomatoid carcinoma presenting as a necrotizing cavitary lung lesion: diagnostic dilemma
Pulmonary sarcomatoid carcinoma (PSC) is a rare histological subtype that has an aggressive course with average survival of 11-13 months.1 In clinical practice, the possible presentations of this rare cancer are not widely known, resulting in a misdiagnosis. That is what happened with our patient, who presented with necrotizing cavitary lung lesion and soft tissue necrotizing lymphadenitis. The clinical picture was reminiscent of tuberculosis or granulomatosis with polyangiitis and was further confounded by negative computed-tomography (CT)-guided biopsy and bronchoscopy findings, which added to the delay in diagnosis. With the currently available knowledge, the diagnosis of PSC depends largely on evaluation of the surgically resected specimen, which in most cases is avoided until there is a high suspicion of PSC. Biopsy is not useful due to extensive necrosis, as will be seen in our case. Consequently, most of the data in the literature is based on case series of autopsy specimen, and the clinical characteristics of PSC remain unclear. The rarity of PSC has prevented its characterization in literature. We report here a rare presentation of PSC with necrotizing lung lesion, to add to the paucity of the current data.
Case presentation and summary
A 58-year-old homeless man presented to the Upstate University Hospital, Syracuse, New York, with a 25-pound weight loss during the previous month and associated productive cough and hemoptysis for a week and a painful mass in the nape of his neck. He denied any fever, chest pain, sick contacts, or joint pain. He had a history of about 40 pack-years of smoking, and his brother had recently been diagnosed with lung cancer. A tender fluctuant mass was detected in the nape of his neck on examination (Figure 1).
The patient had presented 9 months earlier with persistent cough and hemoptysis, and at that visit was found to have a cavitary lesion in the right lung measuring 2 cm (0.8 in). He had undergone a computed-tomograpghy (CT)-guided biopsy of the lesion, which had shown acute and chronic inflammation with fibrosis, and he had negative bronchoscopy findings. The patient tested negative for tuberculosis during the first visit but he left the hospital against the medical advice of the physicians and he was lost to follow-up until his re-presentation.
On physical examination at his re-presentation, the patient seemed cachectic, with a blood pressure of 94/62 mm of Hg. The mass in the nape of his neck was about 3 cm (1.2 in) long, with erythema of the surrounding skin (Figure 1). Bronchial breath sounds were heard in the right upper lobe of the lung, likely due to the underlying cavitary lesion (Figure 2B). Relevant lab findings included a negative HIV test and repeat AFB (acid-fast bacilli) sputum cultures. A CT-guided biopsy with contrast of the thorax showed an interval increase in the size of the cavitary lesion in the patient’s right upper lobe, now measuring about 10 cm (4 in). Also seen were multiple nodules elsewhere in both lungs, with the largest measuring 8 mm (0.3 in). A CT scan of the neck showed 3 cm cystic mass within the posterior subcutaneous soft tissue of the C3 level, confirming the examination finding of the neck mass (Figure 2A) with peripheral enhancement and surrounding infiltrative changes, likely abscess or malignant lymph node versus necrotic infection. He underwent bronchoscopy, which again failed to reveal any endobronchial lesions. Bronchoalveolar lavage was sent for microbiological analysis, including AFB and fungus, but came back negative. Transbronchial biopsy cytology revealed fragments of tumor composed of large pleomorphic cells without glandular or squamous differentiation, within large areas of necrosis (Figure 3). Immunohistochemical studies showed strong reactivity with cytokeratin CAM5.2 (Figure 4), weak and focal reactivity with cytokeratin AE1/AE3 (Figure 5), and lack of reactivity with CD20, CD3, CD30, S-100, MART-1, TTF-1 and p63, all findings consistent with sarcomatoid carcinoma.
The patient underwent fine-needle aspiration and drainage of the neck lesion and the culture grew mixed organisms The results of a bone scan, which was done within a week, showed multiple foci of uptake in the ribs and cervical spine. Given the patient’s advanced disease, he was started on palliative radiotherapy with radiosensitizing chemotherapy with carboplatin (target AUC 6) and paclitaxel (135 mg/m2 over 24 hours). His symptoms of hemoptysis improved transiently after the first cycle, but he became hypotensive and drowsy during the second cycle of therapy, and the family decided to make the patient comfort care and withdraw all further treatment. He was discharged to hospice.
Discussion
PSC is a rare variant of non-small-cell carcinoma lung cancer, accounting for up to 0.4% of lung malignancy.1 It was
recently subtyped by the World Health Organization as a non-small cell lung carcinoma with certain amount of differentiation resembling sarcoma or containing elements of sarcoma.2-4 It is not known why both elements co-exist in the tumor, but Franks and colleagues some theories have been postulated in the literature, including possible origin from a single, aberrant stem cell with progenies differentiating in two separate pathways.3
Sarcomatoid carcinoma consists of spectrum of tumors including pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and blastoma.3,4 It usually shows male preponderance, and association with smoking.3 The diagnosis commonly occurs in the sixth decade of life, except for pulmonary blastoma, which is more common in the fourth decade andnwith equal gender distribution.4
The presenting symptoms can be variable and nonspecific, but predominantly include chest pain, cough, hemoptysis, and/or weight loss.5 Radiologically, pulmonary sarcomatoid cancer presenting as a necrotizing cavitary lesion in the lung is a rare finding, seldom reported in the past.6,7 The presentation in our case, with necrotizing lymphadenitis, was reminiscent of an infectious or autoimmune etiology such as tuberculosis or granulomatosis with polyangiitis. The presence of extensive necrosis in the lesion and the characteristic heterogeneity of the tumor had resulted in inconclusive biopsy findings during the previous presentation. In clinical practice, there is over-reliance on biopsy findings to make the distinction between cancer and other mimicking conditions. This is especially true for rare tumors such as PSC, which often results in misdiagnosis and a delay in administering the proper treatment. Transbronchial biopsy in cases such as the present case, carries little benefit because the diagnosis depends on the site from which the biopsy is taken and whether the biopsied tissue is representative of the entire mass. The diagnosis can be suspected based on the clinical and radiological findings but confirmation requires a surgical resection to delineate the accurate cytology and architecture.5,6,8 Huang and colleagues showed a misdiagnosis rate of PSC of >70% preoperatively.4 Resective surgery is feasible only in patients with high index of suspicion for a malignancy, which in most cases requires previous confirmation with a biopsy. The rarity of this cancer, its unusual presentations, and the lack of specific testing preclude early diagnosis and timely treatment of this fatal condition.
Initial treatment options for localized or with limited spread disease is resective surgery. The role of chemo- or radiation therapy is not known, but they have not previously shown promising results,6,8 except in some cases when they are used as postoperative adjuvant chemotherapy4 or in bulky, locally invasive tumors.1 The recurrence rate after surgery is very high, resulting in a poor 5-year survival rate.1,8 Experimental therapies, such as antibodies that target epidermal growth factor receptor mutations, have not shown much success either.8 In conclusion, the outlook for patients with PSC with the current available knowledge and treatment protocols, is dismal.
Most of the current knowledge and data in the literature is based on cases from autopsy or early-stage surgical resections rather than on patients with advanced cancer.5 Moreover, the role of surgical resection in PSC is questionable, given the high recurrence rate. Subsequently, the clinical and pathological manifestations have yet to be well characterized.4 There has been advance with the publication of more studies recently. Cytokeratin markers such as CAM 5.2 and AE1/AE3 are commonly useful to support the diagnosis when suspected.3 Other markers, including the carcinoembryonic antigen, CD15, and thyroid transcription factor-1 may be variably positive, based on the differentiation of the cancer. Other exciting prospects in the study of PSC include the suggestion of a modified vimentin histologic score for better characterization of the cancer and the discovery of high plateletderived growth factor receptor beta immunohistochemistry expression in PSC as a potential target for future therapy.
Conclusion
Pulmonary sarcomatoid lung cancer can present with a predominant necrotizing picture that mimics diseases such as tuberculosis. In such case, transbronchial biopsy carries little benefit because the diagnosis depends on whether the biopsied tissue is representative of the entire mass, often confounded by the extensive necrosis. More data is needed to determine prognostic factors and appropriate therapeutic strategies. TSJ
Correspondence
Gaurang Nandkishor Vaidya, MD
References
1. Martin LW, Correa AM, Ordonez NG, et al. Sarcomatoid carcinoma of the lung: a predictor of poor prognosis. Ann Thorac Surg. 2007;84(3):973-980.
2. Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y. The new World Health Organization classification of lung tumours. Eur Respir J. 2001;18(6):1059-1068.
3. Franks TJ, Galvin JR. Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis. Arch Pathol Lab Med. 2010;134(1):49-54.
4. Huang SY, Shen SJ, Li XY. Pulmonary sarcomatoid carcinoma: a clinicopathologic study and prognostic analysis of 51 cases. http://wjso. biomedcentral.com/articles/10.1186/1477-7819-11-252. Published 2013. Accessed March 12, 2017.
5. Travis WD. Sarcomatoid neoplasms of the lung and pleura. Arch Pathol Lab Med. 2010;134(11):1645-1658.
6. Pelosi G, Sonzogni A, De Pas T, et al. Review article: pulmonary sarcomatoid carcinomas: a practical overview. Int J Surg Pathol. 2010;18(2):103-120.
7. Chang YL, Lee YC, Shih JY, Wu CT. Pulmonary pleomorphic (spindle) cell carcinoma: peculiar clinicopathologic manifestations different from ordinary non-small cell carcinoma. Lung Cancer. 2001;34(1):91-97.
8. Park JS, Lee Y, Han J, et al. Clinicopathologic outcomes of curative resection for sarcomatoid carcinoma of the lung. Oncology. 2011;81(3-4):206-213.
Pulmonary sarcomatoid carcinoma (PSC) is a rare histological subtype that has an aggressive course with average survival of 11-13 months.1 In clinical practice, the possible presentations of this rare cancer are not widely known, resulting in a misdiagnosis. That is what happened with our patient, who presented with necrotizing cavitary lung lesion and soft tissue necrotizing lymphadenitis. The clinical picture was reminiscent of tuberculosis or granulomatosis with polyangiitis and was further confounded by negative computed-tomography (CT)-guided biopsy and bronchoscopy findings, which added to the delay in diagnosis. With the currently available knowledge, the diagnosis of PSC depends largely on evaluation of the surgically resected specimen, which in most cases is avoided until there is a high suspicion of PSC. Biopsy is not useful due to extensive necrosis, as will be seen in our case. Consequently, most of the data in the literature is based on case series of autopsy specimen, and the clinical characteristics of PSC remain unclear. The rarity of PSC has prevented its characterization in literature. We report here a rare presentation of PSC with necrotizing lung lesion, to add to the paucity of the current data.
Case presentation and summary
A 58-year-old homeless man presented to the Upstate University Hospital, Syracuse, New York, with a 25-pound weight loss during the previous month and associated productive cough and hemoptysis for a week and a painful mass in the nape of his neck. He denied any fever, chest pain, sick contacts, or joint pain. He had a history of about 40 pack-years of smoking, and his brother had recently been diagnosed with lung cancer. A tender fluctuant mass was detected in the nape of his neck on examination (Figure 1).
The patient had presented 9 months earlier with persistent cough and hemoptysis, and at that visit was found to have a cavitary lesion in the right lung measuring 2 cm (0.8 in). He had undergone a computed-tomograpghy (CT)-guided biopsy of the lesion, which had shown acute and chronic inflammation with fibrosis, and he had negative bronchoscopy findings. The patient tested negative for tuberculosis during the first visit but he left the hospital against the medical advice of the physicians and he was lost to follow-up until his re-presentation.
On physical examination at his re-presentation, the patient seemed cachectic, with a blood pressure of 94/62 mm of Hg. The mass in the nape of his neck was about 3 cm (1.2 in) long, with erythema of the surrounding skin (Figure 1). Bronchial breath sounds were heard in the right upper lobe of the lung, likely due to the underlying cavitary lesion (Figure 2B). Relevant lab findings included a negative HIV test and repeat AFB (acid-fast bacilli) sputum cultures. A CT-guided biopsy with contrast of the thorax showed an interval increase in the size of the cavitary lesion in the patient’s right upper lobe, now measuring about 10 cm (4 in). Also seen were multiple nodules elsewhere in both lungs, with the largest measuring 8 mm (0.3 in). A CT scan of the neck showed 3 cm cystic mass within the posterior subcutaneous soft tissue of the C3 level, confirming the examination finding of the neck mass (Figure 2A) with peripheral enhancement and surrounding infiltrative changes, likely abscess or malignant lymph node versus necrotic infection. He underwent bronchoscopy, which again failed to reveal any endobronchial lesions. Bronchoalveolar lavage was sent for microbiological analysis, including AFB and fungus, but came back negative. Transbronchial biopsy cytology revealed fragments of tumor composed of large pleomorphic cells without glandular or squamous differentiation, within large areas of necrosis (Figure 3). Immunohistochemical studies showed strong reactivity with cytokeratin CAM5.2 (Figure 4), weak and focal reactivity with cytokeratin AE1/AE3 (Figure 5), and lack of reactivity with CD20, CD3, CD30, S-100, MART-1, TTF-1 and p63, all findings consistent with sarcomatoid carcinoma.
The patient underwent fine-needle aspiration and drainage of the neck lesion and the culture grew mixed organisms The results of a bone scan, which was done within a week, showed multiple foci of uptake in the ribs and cervical spine. Given the patient’s advanced disease, he was started on palliative radiotherapy with radiosensitizing chemotherapy with carboplatin (target AUC 6) and paclitaxel (135 mg/m2 over 24 hours). His symptoms of hemoptysis improved transiently after the first cycle, but he became hypotensive and drowsy during the second cycle of therapy, and the family decided to make the patient comfort care and withdraw all further treatment. He was discharged to hospice.
Discussion
PSC is a rare variant of non-small-cell carcinoma lung cancer, accounting for up to 0.4% of lung malignancy.1 It was
recently subtyped by the World Health Organization as a non-small cell lung carcinoma with certain amount of differentiation resembling sarcoma or containing elements of sarcoma.2-4 It is not known why both elements co-exist in the tumor, but Franks and colleagues some theories have been postulated in the literature, including possible origin from a single, aberrant stem cell with progenies differentiating in two separate pathways.3
Sarcomatoid carcinoma consists of spectrum of tumors including pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and blastoma.3,4 It usually shows male preponderance, and association with smoking.3 The diagnosis commonly occurs in the sixth decade of life, except for pulmonary blastoma, which is more common in the fourth decade andnwith equal gender distribution.4
The presenting symptoms can be variable and nonspecific, but predominantly include chest pain, cough, hemoptysis, and/or weight loss.5 Radiologically, pulmonary sarcomatoid cancer presenting as a necrotizing cavitary lesion in the lung is a rare finding, seldom reported in the past.6,7 The presentation in our case, with necrotizing lymphadenitis, was reminiscent of an infectious or autoimmune etiology such as tuberculosis or granulomatosis with polyangiitis. The presence of extensive necrosis in the lesion and the characteristic heterogeneity of the tumor had resulted in inconclusive biopsy findings during the previous presentation. In clinical practice, there is over-reliance on biopsy findings to make the distinction between cancer and other mimicking conditions. This is especially true for rare tumors such as PSC, which often results in misdiagnosis and a delay in administering the proper treatment. Transbronchial biopsy in cases such as the present case, carries little benefit because the diagnosis depends on the site from which the biopsy is taken and whether the biopsied tissue is representative of the entire mass. The diagnosis can be suspected based on the clinical and radiological findings but confirmation requires a surgical resection to delineate the accurate cytology and architecture.5,6,8 Huang and colleagues showed a misdiagnosis rate of PSC of >70% preoperatively.4 Resective surgery is feasible only in patients with high index of suspicion for a malignancy, which in most cases requires previous confirmation with a biopsy. The rarity of this cancer, its unusual presentations, and the lack of specific testing preclude early diagnosis and timely treatment of this fatal condition.
Initial treatment options for localized or with limited spread disease is resective surgery. The role of chemo- or radiation therapy is not known, but they have not previously shown promising results,6,8 except in some cases when they are used as postoperative adjuvant chemotherapy4 or in bulky, locally invasive tumors.1 The recurrence rate after surgery is very high, resulting in a poor 5-year survival rate.1,8 Experimental therapies, such as antibodies that target epidermal growth factor receptor mutations, have not shown much success either.8 In conclusion, the outlook for patients with PSC with the current available knowledge and treatment protocols, is dismal.
Most of the current knowledge and data in the literature is based on cases from autopsy or early-stage surgical resections rather than on patients with advanced cancer.5 Moreover, the role of surgical resection in PSC is questionable, given the high recurrence rate. Subsequently, the clinical and pathological manifestations have yet to be well characterized.4 There has been advance with the publication of more studies recently. Cytokeratin markers such as CAM 5.2 and AE1/AE3 are commonly useful to support the diagnosis when suspected.3 Other markers, including the carcinoembryonic antigen, CD15, and thyroid transcription factor-1 may be variably positive, based on the differentiation of the cancer. Other exciting prospects in the study of PSC include the suggestion of a modified vimentin histologic score for better characterization of the cancer and the discovery of high plateletderived growth factor receptor beta immunohistochemistry expression in PSC as a potential target for future therapy.
Conclusion
Pulmonary sarcomatoid lung cancer can present with a predominant necrotizing picture that mimics diseases such as tuberculosis. In such case, transbronchial biopsy carries little benefit because the diagnosis depends on whether the biopsied tissue is representative of the entire mass, often confounded by the extensive necrosis. More data is needed to determine prognostic factors and appropriate therapeutic strategies. TSJ
Correspondence
Gaurang Nandkishor Vaidya, MD
References
1. Martin LW, Correa AM, Ordonez NG, et al. Sarcomatoid carcinoma of the lung: a predictor of poor prognosis. Ann Thorac Surg. 2007;84(3):973-980.
2. Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y. The new World Health Organization classification of lung tumours. Eur Respir J. 2001;18(6):1059-1068.
3. Franks TJ, Galvin JR. Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis. Arch Pathol Lab Med. 2010;134(1):49-54.
4. Huang SY, Shen SJ, Li XY. Pulmonary sarcomatoid carcinoma: a clinicopathologic study and prognostic analysis of 51 cases. http://wjso. biomedcentral.com/articles/10.1186/1477-7819-11-252. Published 2013. Accessed March 12, 2017.
5. Travis WD. Sarcomatoid neoplasms of the lung and pleura. Arch Pathol Lab Med. 2010;134(11):1645-1658.
6. Pelosi G, Sonzogni A, De Pas T, et al. Review article: pulmonary sarcomatoid carcinomas: a practical overview. Int J Surg Pathol. 2010;18(2):103-120.
7. Chang YL, Lee YC, Shih JY, Wu CT. Pulmonary pleomorphic (spindle) cell carcinoma: peculiar clinicopathologic manifestations different from ordinary non-small cell carcinoma. Lung Cancer. 2001;34(1):91-97.
8. Park JS, Lee Y, Han J, et al. Clinicopathologic outcomes of curative resection for sarcomatoid carcinoma of the lung. Oncology. 2011;81(3-4):206-213.
Pulmonary sarcomatoid carcinoma (PSC) is a rare histological subtype that has an aggressive course with average survival of 11-13 months.1 In clinical practice, the possible presentations of this rare cancer are not widely known, resulting in a misdiagnosis. That is what happened with our patient, who presented with necrotizing cavitary lung lesion and soft tissue necrotizing lymphadenitis. The clinical picture was reminiscent of tuberculosis or granulomatosis with polyangiitis and was further confounded by negative computed-tomography (CT)-guided biopsy and bronchoscopy findings, which added to the delay in diagnosis. With the currently available knowledge, the diagnosis of PSC depends largely on evaluation of the surgically resected specimen, which in most cases is avoided until there is a high suspicion of PSC. Biopsy is not useful due to extensive necrosis, as will be seen in our case. Consequently, most of the data in the literature is based on case series of autopsy specimen, and the clinical characteristics of PSC remain unclear. The rarity of PSC has prevented its characterization in literature. We report here a rare presentation of PSC with necrotizing lung lesion, to add to the paucity of the current data.
Case presentation and summary
A 58-year-old homeless man presented to the Upstate University Hospital, Syracuse, New York, with a 25-pound weight loss during the previous month and associated productive cough and hemoptysis for a week and a painful mass in the nape of his neck. He denied any fever, chest pain, sick contacts, or joint pain. He had a history of about 40 pack-years of smoking, and his brother had recently been diagnosed with lung cancer. A tender fluctuant mass was detected in the nape of his neck on examination (Figure 1).
The patient had presented 9 months earlier with persistent cough and hemoptysis, and at that visit was found to have a cavitary lesion in the right lung measuring 2 cm (0.8 in). He had undergone a computed-tomograpghy (CT)-guided biopsy of the lesion, which had shown acute and chronic inflammation with fibrosis, and he had negative bronchoscopy findings. The patient tested negative for tuberculosis during the first visit but he left the hospital against the medical advice of the physicians and he was lost to follow-up until his re-presentation.
On physical examination at his re-presentation, the patient seemed cachectic, with a blood pressure of 94/62 mm of Hg. The mass in the nape of his neck was about 3 cm (1.2 in) long, with erythema of the surrounding skin (Figure 1). Bronchial breath sounds were heard in the right upper lobe of the lung, likely due to the underlying cavitary lesion (Figure 2B). Relevant lab findings included a negative HIV test and repeat AFB (acid-fast bacilli) sputum cultures. A CT-guided biopsy with contrast of the thorax showed an interval increase in the size of the cavitary lesion in the patient’s right upper lobe, now measuring about 10 cm (4 in). Also seen were multiple nodules elsewhere in both lungs, with the largest measuring 8 mm (0.3 in). A CT scan of the neck showed 3 cm cystic mass within the posterior subcutaneous soft tissue of the C3 level, confirming the examination finding of the neck mass (Figure 2A) with peripheral enhancement and surrounding infiltrative changes, likely abscess or malignant lymph node versus necrotic infection. He underwent bronchoscopy, which again failed to reveal any endobronchial lesions. Bronchoalveolar lavage was sent for microbiological analysis, including AFB and fungus, but came back negative. Transbronchial biopsy cytology revealed fragments of tumor composed of large pleomorphic cells without glandular or squamous differentiation, within large areas of necrosis (Figure 3). Immunohistochemical studies showed strong reactivity with cytokeratin CAM5.2 (Figure 4), weak and focal reactivity with cytokeratin AE1/AE3 (Figure 5), and lack of reactivity with CD20, CD3, CD30, S-100, MART-1, TTF-1 and p63, all findings consistent with sarcomatoid carcinoma.
The patient underwent fine-needle aspiration and drainage of the neck lesion and the culture grew mixed organisms The results of a bone scan, which was done within a week, showed multiple foci of uptake in the ribs and cervical spine. Given the patient’s advanced disease, he was started on palliative radiotherapy with radiosensitizing chemotherapy with carboplatin (target AUC 6) and paclitaxel (135 mg/m2 over 24 hours). His symptoms of hemoptysis improved transiently after the first cycle, but he became hypotensive and drowsy during the second cycle of therapy, and the family decided to make the patient comfort care and withdraw all further treatment. He was discharged to hospice.
Discussion
PSC is a rare variant of non-small-cell carcinoma lung cancer, accounting for up to 0.4% of lung malignancy.1 It was
recently subtyped by the World Health Organization as a non-small cell lung carcinoma with certain amount of differentiation resembling sarcoma or containing elements of sarcoma.2-4 It is not known why both elements co-exist in the tumor, but Franks and colleagues some theories have been postulated in the literature, including possible origin from a single, aberrant stem cell with progenies differentiating in two separate pathways.3
Sarcomatoid carcinoma consists of spectrum of tumors including pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and blastoma.3,4 It usually shows male preponderance, and association with smoking.3 The diagnosis commonly occurs in the sixth decade of life, except for pulmonary blastoma, which is more common in the fourth decade andnwith equal gender distribution.4
The presenting symptoms can be variable and nonspecific, but predominantly include chest pain, cough, hemoptysis, and/or weight loss.5 Radiologically, pulmonary sarcomatoid cancer presenting as a necrotizing cavitary lesion in the lung is a rare finding, seldom reported in the past.6,7 The presentation in our case, with necrotizing lymphadenitis, was reminiscent of an infectious or autoimmune etiology such as tuberculosis or granulomatosis with polyangiitis. The presence of extensive necrosis in the lesion and the characteristic heterogeneity of the tumor had resulted in inconclusive biopsy findings during the previous presentation. In clinical practice, there is over-reliance on biopsy findings to make the distinction between cancer and other mimicking conditions. This is especially true for rare tumors such as PSC, which often results in misdiagnosis and a delay in administering the proper treatment. Transbronchial biopsy in cases such as the present case, carries little benefit because the diagnosis depends on the site from which the biopsy is taken and whether the biopsied tissue is representative of the entire mass. The diagnosis can be suspected based on the clinical and radiological findings but confirmation requires a surgical resection to delineate the accurate cytology and architecture.5,6,8 Huang and colleagues showed a misdiagnosis rate of PSC of >70% preoperatively.4 Resective surgery is feasible only in patients with high index of suspicion for a malignancy, which in most cases requires previous confirmation with a biopsy. The rarity of this cancer, its unusual presentations, and the lack of specific testing preclude early diagnosis and timely treatment of this fatal condition.
Initial treatment options for localized or with limited spread disease is resective surgery. The role of chemo- or radiation therapy is not known, but they have not previously shown promising results,6,8 except in some cases when they are used as postoperative adjuvant chemotherapy4 or in bulky, locally invasive tumors.1 The recurrence rate after surgery is very high, resulting in a poor 5-year survival rate.1,8 Experimental therapies, such as antibodies that target epidermal growth factor receptor mutations, have not shown much success either.8 In conclusion, the outlook for patients with PSC with the current available knowledge and treatment protocols, is dismal.
Most of the current knowledge and data in the literature is based on cases from autopsy or early-stage surgical resections rather than on patients with advanced cancer.5 Moreover, the role of surgical resection in PSC is questionable, given the high recurrence rate. Subsequently, the clinical and pathological manifestations have yet to be well characterized.4 There has been advance with the publication of more studies recently. Cytokeratin markers such as CAM 5.2 and AE1/AE3 are commonly useful to support the diagnosis when suspected.3 Other markers, including the carcinoembryonic antigen, CD15, and thyroid transcription factor-1 may be variably positive, based on the differentiation of the cancer. Other exciting prospects in the study of PSC include the suggestion of a modified vimentin histologic score for better characterization of the cancer and the discovery of high plateletderived growth factor receptor beta immunohistochemistry expression in PSC as a potential target for future therapy.
Conclusion
Pulmonary sarcomatoid lung cancer can present with a predominant necrotizing picture that mimics diseases such as tuberculosis. In such case, transbronchial biopsy carries little benefit because the diagnosis depends on whether the biopsied tissue is representative of the entire mass, often confounded by the extensive necrosis. More data is needed to determine prognostic factors and appropriate therapeutic strategies. TSJ
Correspondence
Gaurang Nandkishor Vaidya, MD
References
1. Martin LW, Correa AM, Ordonez NG, et al. Sarcomatoid carcinoma of the lung: a predictor of poor prognosis. Ann Thorac Surg. 2007;84(3):973-980.
2. Brambilla E, Travis WD, Colby TV, Corrin B, Shimosato Y. The new World Health Organization classification of lung tumours. Eur Respir J. 2001;18(6):1059-1068.
3. Franks TJ, Galvin JR. Sarcomatoid carcinoma of the lung: histologic criteria and common lesions in the differential diagnosis. Arch Pathol Lab Med. 2010;134(1):49-54.
4. Huang SY, Shen SJ, Li XY. Pulmonary sarcomatoid carcinoma: a clinicopathologic study and prognostic analysis of 51 cases. http://wjso. biomedcentral.com/articles/10.1186/1477-7819-11-252. Published 2013. Accessed March 12, 2017.
5. Travis WD. Sarcomatoid neoplasms of the lung and pleura. Arch Pathol Lab Med. 2010;134(11):1645-1658.
6. Pelosi G, Sonzogni A, De Pas T, et al. Review article: pulmonary sarcomatoid carcinomas: a practical overview. Int J Surg Pathol. 2010;18(2):103-120.
7. Chang YL, Lee YC, Shih JY, Wu CT. Pulmonary pleomorphic (spindle) cell carcinoma: peculiar clinicopathologic manifestations different from ordinary non-small cell carcinoma. Lung Cancer. 2001;34(1):91-97.
8. Park JS, Lee Y, Han J, et al. Clinicopathologic outcomes of curative resection for sarcomatoid carcinoma of the lung. Oncology. 2011;81(3-4):206-213.
This article was originally published in the Journal of Community and Supportive Oncology (JCSO 2017;15(2):103-105). doi: https://doi.org/10.12788/jcso.0259. It is reproduced here with permission of the copyright owner. Further reproduction is prohibited without permission.
Bilateral chylothorax in an AIDS patient with newly diagnosed Kaposi sarcoma
Kaposi sarcoma is an angioproliferative tumor that is associated with human herpes virus-B (HIV-B). Mucocutaneous disease is the most common site for manifestation of AIDS-related Kaposi sarcoma, commonly affecting the lower extremeties, oral mucosa, face, and genitalia. Pleural effusions can occur in 36%-60% of patients with Kaposi sarcoma, and it has been documented that chylothorax is a rare, but plausible presentation in patients with Kaposi sarcoma.1 We present here a case of bilateral chylothorax in a patient with AIDS-related Kaposi sarcoma.
Case presentation and summary
A 52-year-old MSM male with AIDS (CD4, <20 mm3 ; viral load, 58 copies/ml) presented to the emergency department with complaints of shortness of breath, productive cough, and diarrhea for 2 days prior to presentation. His medical history also included chronic obstructive pulmonary disease, coronary artery disease, and hyperlipidemia.The patient was not on HAART because of his history of noncompliance. The results of a chest X-ray and computed-tomography (CT) scan showed that the patient had bilateral pleural effusion and a spiculated 14-mm nodule in the left upper lobe.
The patient underwent ultrasound-guided placement of a 12-French left-sided chest catheter, and a milky white fluid was aspirated from the left pleural space. Laboratory analysis of the pleural fluid confirmed an exudate with an elevated triglyceride level of 120 mg/dL (chylous, >110 mg/dL) indicating chylothorax.
On close physical examination, the patient was found to have multiple irregular plaques on the back and lower extremities. As described by dermatology, there was a violaceous indurated plaque on the left axillae, violaceous indurated plaques with superficial scale grouped on the left midlateral back, and hyperpigmented lichenified plaques and papules on bilateral shins, with some with plate-like scale. Two punch biopsies were taken of the skin lesions, which confirmed Kaposi sarcoma, plaque stage from the lesion biopsied on the back, and patch stage from the lesion biopsied in the left axilla. Cytology of the pleural fluid was negative for malignant cells. On review by the radiologist of the CT scan of the chest, there was no indication of gross distention of the thoracic duct. Treatment options were offered to the patient, and the patient was considering options for chemotherapy and home hospice given his advanced disease state at the time of discharge.
Discussion
Chylothorax occurs with a thoracic duct obstruction, which results in leakage of lymphatic fluid into the pleural cavity. The two leading causes of chylothorax are trauma and malignancy, with lymphoma being the most common cause of chylothorax among those with malignancy.2 Chylothorax, however, is a rare but documented complication of Kaposi sarcoma. Marais and colleagues reported the case of a 3-year-old HIV-positive patient with newly diagnosed Kaposi sarcoma who was found to have tumor infiltration in the thoracic duct leading to bilateral chylothorax.3 Maradona and colleagues described a 40-year-old man with AIDS-related Kaposi sarcoma who was found to have pleural and pericardial Kaposi sarcoma with chylothorax.4 Priest and colleagues wrote about a 32-year-old patient with AIDS with biopsy-proven Kaposi sarcoma who required multiple therapeutic thoracenteses for rapidly recurrent left chylothorax effusions.5
There are two leading discussions as to the pathophysiology of chylothorax that is related to Kaposi sarcoma: chylothorax developing secondary to metastatic disease or the development of chylothorax secondary to primary Kaposi sarcoma arising from the pleural region.6 One case report examined pleural and lung biopsies in a 34-year-old patient with AIDS-related Kaposi sarcoma that showed immunohistochemical staining that was suggestive of early-stage Kaposi sarcoma of lymphatic endothelial origin. The authors were attempting to illustrate that Kaposi sarcoma may have a stem-cell origin which can differentiate into lymph cells. Kontantinopoulos and colleagues postulated that in situ Kaposi sarcoma can arise from the lymphatic system with a resultant clinical presentation of chylothorax.7 The more mainstream thought however, is that chylothorax has been found to develop secondary to metastatic disease. The present case, therefore, illustrates an unusual presentation of cytology negative chylothorax in a patient with AIDS-related Kaposi sarcoma. TSJ
Rebecca E Neril, MD; Department of Internal Medicine, SBH Health System, Bronx, New York.
References
1. Sridar S, Garza EG, Cox J, Rumbak MJ. Serosanguineous pleural effusions in a patient with HIV and Kaposi sarcoma: pleuroscopic findings. J Bronchology Interv Pulmonol. 2011;18(4):337-339.
2. Light RW. Chylothorax and pseudochylothorax. In: Light RW, ed. Pleural diseases. 6th ed. Philadelphia: Lippincott Williams & Wilkins, 2013:412-426.
3. Marais BJ, Pienaar J, Gie RP. Kaposi sarcoma with upper airway obstruction and bilateral chylothoraces. Pediatr Infect Dis J. 2003;22:926-928.
4. Maradona JA, Carton JA, Asensi V, Rodriguez-Guardado A. AIDSrelated Kaposi sarcoma with chylothorax and pericardial involvement satisfactorily treated with liposomal doxorubicin. AIDS. 2002;16(5):806.
5. Priest ER, Weiss R. Chylothorax with Kaposi sarcoma. South Med J. 1991;84:806-807.
6. Pantanowitz L, Dezube BJ. Kaposi sarcoma in unusual locations. BMC Cancer. 2008;8:190.
7. Konstantinopoulos PA, Dezube BJ, Pantanowitz L. Morphologic and immunophenotypic evidence of in situ Kaposi sarcoma. BMC Clin Pathol. 2006;30:6:7.
Kaposi sarcoma is an angioproliferative tumor that is associated with human herpes virus-B (HIV-B). Mucocutaneous disease is the most common site for manifestation of AIDS-related Kaposi sarcoma, commonly affecting the lower extremeties, oral mucosa, face, and genitalia. Pleural effusions can occur in 36%-60% of patients with Kaposi sarcoma, and it has been documented that chylothorax is a rare, but plausible presentation in patients with Kaposi sarcoma.1 We present here a case of bilateral chylothorax in a patient with AIDS-related Kaposi sarcoma.
Case presentation and summary
A 52-year-old MSM male with AIDS (CD4, <20 mm3 ; viral load, 58 copies/ml) presented to the emergency department with complaints of shortness of breath, productive cough, and diarrhea for 2 days prior to presentation. His medical history also included chronic obstructive pulmonary disease, coronary artery disease, and hyperlipidemia.The patient was not on HAART because of his history of noncompliance. The results of a chest X-ray and computed-tomography (CT) scan showed that the patient had bilateral pleural effusion and a spiculated 14-mm nodule in the left upper lobe.
The patient underwent ultrasound-guided placement of a 12-French left-sided chest catheter, and a milky white fluid was aspirated from the left pleural space. Laboratory analysis of the pleural fluid confirmed an exudate with an elevated triglyceride level of 120 mg/dL (chylous, >110 mg/dL) indicating chylothorax.
On close physical examination, the patient was found to have multiple irregular plaques on the back and lower extremities. As described by dermatology, there was a violaceous indurated plaque on the left axillae, violaceous indurated plaques with superficial scale grouped on the left midlateral back, and hyperpigmented lichenified plaques and papules on bilateral shins, with some with plate-like scale. Two punch biopsies were taken of the skin lesions, which confirmed Kaposi sarcoma, plaque stage from the lesion biopsied on the back, and patch stage from the lesion biopsied in the left axilla. Cytology of the pleural fluid was negative for malignant cells. On review by the radiologist of the CT scan of the chest, there was no indication of gross distention of the thoracic duct. Treatment options were offered to the patient, and the patient was considering options for chemotherapy and home hospice given his advanced disease state at the time of discharge.
Discussion
Chylothorax occurs with a thoracic duct obstruction, which results in leakage of lymphatic fluid into the pleural cavity. The two leading causes of chylothorax are trauma and malignancy, with lymphoma being the most common cause of chylothorax among those with malignancy.2 Chylothorax, however, is a rare but documented complication of Kaposi sarcoma. Marais and colleagues reported the case of a 3-year-old HIV-positive patient with newly diagnosed Kaposi sarcoma who was found to have tumor infiltration in the thoracic duct leading to bilateral chylothorax.3 Maradona and colleagues described a 40-year-old man with AIDS-related Kaposi sarcoma who was found to have pleural and pericardial Kaposi sarcoma with chylothorax.4 Priest and colleagues wrote about a 32-year-old patient with AIDS with biopsy-proven Kaposi sarcoma who required multiple therapeutic thoracenteses for rapidly recurrent left chylothorax effusions.5
There are two leading discussions as to the pathophysiology of chylothorax that is related to Kaposi sarcoma: chylothorax developing secondary to metastatic disease or the development of chylothorax secondary to primary Kaposi sarcoma arising from the pleural region.6 One case report examined pleural and lung biopsies in a 34-year-old patient with AIDS-related Kaposi sarcoma that showed immunohistochemical staining that was suggestive of early-stage Kaposi sarcoma of lymphatic endothelial origin. The authors were attempting to illustrate that Kaposi sarcoma may have a stem-cell origin which can differentiate into lymph cells. Kontantinopoulos and colleagues postulated that in situ Kaposi sarcoma can arise from the lymphatic system with a resultant clinical presentation of chylothorax.7 The more mainstream thought however, is that chylothorax has been found to develop secondary to metastatic disease. The present case, therefore, illustrates an unusual presentation of cytology negative chylothorax in a patient with AIDS-related Kaposi sarcoma. TSJ
Rebecca E Neril, MD; Department of Internal Medicine, SBH Health System, Bronx, New York.
Kaposi sarcoma is an angioproliferative tumor that is associated with human herpes virus-B (HIV-B). Mucocutaneous disease is the most common site for manifestation of AIDS-related Kaposi sarcoma, commonly affecting the lower extremeties, oral mucosa, face, and genitalia. Pleural effusions can occur in 36%-60% of patients with Kaposi sarcoma, and it has been documented that chylothorax is a rare, but plausible presentation in patients with Kaposi sarcoma.1 We present here a case of bilateral chylothorax in a patient with AIDS-related Kaposi sarcoma.
Case presentation and summary
A 52-year-old MSM male with AIDS (CD4, <20 mm3 ; viral load, 58 copies/ml) presented to the emergency department with complaints of shortness of breath, productive cough, and diarrhea for 2 days prior to presentation. His medical history also included chronic obstructive pulmonary disease, coronary artery disease, and hyperlipidemia.The patient was not on HAART because of his history of noncompliance. The results of a chest X-ray and computed-tomography (CT) scan showed that the patient had bilateral pleural effusion and a spiculated 14-mm nodule in the left upper lobe.
The patient underwent ultrasound-guided placement of a 12-French left-sided chest catheter, and a milky white fluid was aspirated from the left pleural space. Laboratory analysis of the pleural fluid confirmed an exudate with an elevated triglyceride level of 120 mg/dL (chylous, >110 mg/dL) indicating chylothorax.
On close physical examination, the patient was found to have multiple irregular plaques on the back and lower extremities. As described by dermatology, there was a violaceous indurated plaque on the left axillae, violaceous indurated plaques with superficial scale grouped on the left midlateral back, and hyperpigmented lichenified plaques and papules on bilateral shins, with some with plate-like scale. Two punch biopsies were taken of the skin lesions, which confirmed Kaposi sarcoma, plaque stage from the lesion biopsied on the back, and patch stage from the lesion biopsied in the left axilla. Cytology of the pleural fluid was negative for malignant cells. On review by the radiologist of the CT scan of the chest, there was no indication of gross distention of the thoracic duct. Treatment options were offered to the patient, and the patient was considering options for chemotherapy and home hospice given his advanced disease state at the time of discharge.
Discussion
Chylothorax occurs with a thoracic duct obstruction, which results in leakage of lymphatic fluid into the pleural cavity. The two leading causes of chylothorax are trauma and malignancy, with lymphoma being the most common cause of chylothorax among those with malignancy.2 Chylothorax, however, is a rare but documented complication of Kaposi sarcoma. Marais and colleagues reported the case of a 3-year-old HIV-positive patient with newly diagnosed Kaposi sarcoma who was found to have tumor infiltration in the thoracic duct leading to bilateral chylothorax.3 Maradona and colleagues described a 40-year-old man with AIDS-related Kaposi sarcoma who was found to have pleural and pericardial Kaposi sarcoma with chylothorax.4 Priest and colleagues wrote about a 32-year-old patient with AIDS with biopsy-proven Kaposi sarcoma who required multiple therapeutic thoracenteses for rapidly recurrent left chylothorax effusions.5
There are two leading discussions as to the pathophysiology of chylothorax that is related to Kaposi sarcoma: chylothorax developing secondary to metastatic disease or the development of chylothorax secondary to primary Kaposi sarcoma arising from the pleural region.6 One case report examined pleural and lung biopsies in a 34-year-old patient with AIDS-related Kaposi sarcoma that showed immunohistochemical staining that was suggestive of early-stage Kaposi sarcoma of lymphatic endothelial origin. The authors were attempting to illustrate that Kaposi sarcoma may have a stem-cell origin which can differentiate into lymph cells. Kontantinopoulos and colleagues postulated that in situ Kaposi sarcoma can arise from the lymphatic system with a resultant clinical presentation of chylothorax.7 The more mainstream thought however, is that chylothorax has been found to develop secondary to metastatic disease. The present case, therefore, illustrates an unusual presentation of cytology negative chylothorax in a patient with AIDS-related Kaposi sarcoma. TSJ
Rebecca E Neril, MD; Department of Internal Medicine, SBH Health System, Bronx, New York.
References
1. Sridar S, Garza EG, Cox J, Rumbak MJ. Serosanguineous pleural effusions in a patient with HIV and Kaposi sarcoma: pleuroscopic findings. J Bronchology Interv Pulmonol. 2011;18(4):337-339.
2. Light RW. Chylothorax and pseudochylothorax. In: Light RW, ed. Pleural diseases. 6th ed. Philadelphia: Lippincott Williams & Wilkins, 2013:412-426.
3. Marais BJ, Pienaar J, Gie RP. Kaposi sarcoma with upper airway obstruction and bilateral chylothoraces. Pediatr Infect Dis J. 2003;22:926-928.
4. Maradona JA, Carton JA, Asensi V, Rodriguez-Guardado A. AIDSrelated Kaposi sarcoma with chylothorax and pericardial involvement satisfactorily treated with liposomal doxorubicin. AIDS. 2002;16(5):806.
5. Priest ER, Weiss R. Chylothorax with Kaposi sarcoma. South Med J. 1991;84:806-807.
6. Pantanowitz L, Dezube BJ. Kaposi sarcoma in unusual locations. BMC Cancer. 2008;8:190.
7. Konstantinopoulos PA, Dezube BJ, Pantanowitz L. Morphologic and immunophenotypic evidence of in situ Kaposi sarcoma. BMC Clin Pathol. 2006;30:6:7.
References
1. Sridar S, Garza EG, Cox J, Rumbak MJ. Serosanguineous pleural effusions in a patient with HIV and Kaposi sarcoma: pleuroscopic findings. J Bronchology Interv Pulmonol. 2011;18(4):337-339.
2. Light RW. Chylothorax and pseudochylothorax. In: Light RW, ed. Pleural diseases. 6th ed. Philadelphia: Lippincott Williams & Wilkins, 2013:412-426.
3. Marais BJ, Pienaar J, Gie RP. Kaposi sarcoma with upper airway obstruction and bilateral chylothoraces. Pediatr Infect Dis J. 2003;22:926-928.
4. Maradona JA, Carton JA, Asensi V, Rodriguez-Guardado A. AIDSrelated Kaposi sarcoma with chylothorax and pericardial involvement satisfactorily treated with liposomal doxorubicin. AIDS. 2002;16(5):806.
5. Priest ER, Weiss R. Chylothorax with Kaposi sarcoma. South Med J. 1991;84:806-807.
6. Pantanowitz L, Dezube BJ. Kaposi sarcoma in unusual locations. BMC Cancer. 2008;8:190.
7. Konstantinopoulos PA, Dezube BJ, Pantanowitz L. Morphologic and immunophenotypic evidence of in situ Kaposi sarcoma. BMC Clin Pathol. 2006;30:6:7.
This article was originally published in the Journal of Community and Supportive Oncology (JCSO 2017;15(3):e174-e175). doi: https://doi.org/10.12788/jcso.0261. It is reproduced here with permission of the copyright owner. Further reproduction is prohibited without permission.
Cordlike Dermal Plaques and Nodules on the Neck and Hands
The Diagnosis: Fibroblastic Rheumatism
Routine histologic sections stained with hematoxylin and eosin demonstrated a noncircumscribed dermal proliferation of fibroblasts and myofibroblasts with thickened collagen bundles (Figure, A and B). Focally fragmented elastin fibers were noted with Verhoeff elastic tissue stain. Alcian blue stain did not show increased dermal mucin. With the clinical presentation and histologic findings described, we diagnosed the patient with fibroblastic rheumatism (FR). To date, the patient's condition has stabilized overall with skin lesions fading and minimal to no joint pain. Current therapies include adalimumab, mycophenolate mofetil 500 mg 3 times daily, and low-dose prednisone.
Fibroblastic rheumatism is a rare arthropathy with cutaneous findings initially described by Chaouat et al1 in 1980. Age of onset varies, and the condition also has been observed in pediatric patients.2 Fibroblastic rheumatism is characterized by sudden onset of firm, flesh-colored, subcutaneous nodules on periungual and periarticular surfaces.2 Neck lesions rarely are described,2-4 and cordlike plaques previously have not been reported in FR. Typically, patients develop diffusely swollen fingers, palmar thickening, sclerodactyly, and contractures. The eruption may be accompanied by Raynaud phenomenon as well as a progressive symmetric erosive arthropathy.2,5
The clinical course in FR is variable. The cutaneous findings spontaneously may regress in months to years.3,4 However, polyarthropathy often is destructive and progresses to disability.3 Response to therapy has been unpredictable, and the following treatments have been tried, generally with poor efficacy: aspirin, nonsteroidal anti-inflammatory drugs, hydroxychloroquine, colchicine, methotrexate, prednisone, infliximab, D-penicillamine, interferon alfa, and intensive physical therapy.2-4,6 Histologic characteristics may include thickened collagen bundles along with a fibroblastic and myofibroblastic proliferation. Elastic fibers may be decreased or absent.2,3,5
Clinical and histologic features in FR may mimic other entities; thus, clinical pathological correlation is essential in determining the correct diagnosis. Considerations in the differential diagnoses include multicentric reticulohistiocytosis (MRH), palisaded neutrophilic and granulomatous dermatitis, and scleroderma.
In MRH, a symmetric erosive arthritis of mainly distal interphalangeal joints typically precedes the cutaneous disease. Occurrence of arthritis mutilans is reported in approximately half of patients.4 Cutaneous manifestations typically include the presence of coral bead-like papules and nodules over the dorsal aspect of the hands, face, and neck. Unlike FR, MRH has a concomitant autoimmune disease in up to 20% of cases and an associated malignancy in up to 31% of cases, with breast and ovarian carcinomas most common. On histology, MRH is characterized by a nodular infiltrate of histiocytes and multinucleated giant cells with eosinophilic ground-glass cytoplasm.4 No notable collagen changes or fibroblastic proliferations typically are present.
Palisaded neutrophilic and granulomatous dermatitis, usually associated with rheumatoid arthritis or connective tissue disease, classically presents as annular plaques and indurated linear bands over the trunk and extremities. However, its clinical presentation is quite variable and may include pink to violaceous urticarialike; livedoid-appearing; or nonspecific papules, plaques, or nodules. Histology in palisaded neutrophilic and granulomatous dermatitis shows a dense dermal neutrophilic infiltrate associated with interstitial histiocytes having a palisading arrangement around degenerated collagen.7 No fibroblastic proliferation typically is present.
Scleroderma can be distinguished based on additional clinical and laboratory findings as well as histology showing thickened collagen bundles without fibroblastic proliferation.2 The histologic findings also may suggest inclusion of dermatofibroma or a scar in the differential diagnosis, though the clinical presentation of these entities would not support these diagnoses.
Acknowledgments
We thank the patient for granting permission to share this information. We also thank Sheng Chen, MD, PhD (Lake Success, New York), for his dermatopathological contributions to the case.
- Chaouat Y, Aron-Brunetiere R, Faures B, et al. Une nouvelle entité: le rhumatisme fibroblastique. a propos d'une observation [in French]. Rev Rhum Mal Osteoartic. 1980;47:34-35.
- Jurado SA, Alvin GG, Selim MA, et al. Fibroblastic rheumatism: a report of 4 cases with potential therapeutic implications. J Am Acad Dermatol. 2012;66:959-965.
- Colonna L, Barbieri C, Di Lella G, et al. Fibroblastic rheumatism: a case without rheumatological symptoms. Acta Derm Venereol. 2002;82:200-203.
- Trotta F, Colina M. Multicentric reticulohistiocytosis and fibroblastic rheumatism. Best Pract Res Clin Rheumatol. 2012;26:543-557.
- Lee JM, Sundel RP, Liang MG. Fibroblastic rheumatism: case report and review of the literature. Pediatr Dermatol. 2002;19:532-535.
- Kluger N, Dumas-Tesici A, Hamel D, et al. Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosis. J Cutan Pathol. 2010;37:587-592.
- Stephenson SR, Campbell M, Dre GS, et al. Palisaded neutrophilic and granulomatous dermatitis presenting in a patient with rheumatoid arthritis on adalimumab. J Cutan Pathol. 2011;38:644-648.
The Diagnosis: Fibroblastic Rheumatism
Routine histologic sections stained with hematoxylin and eosin demonstrated a noncircumscribed dermal proliferation of fibroblasts and myofibroblasts with thickened collagen bundles (Figure, A and B). Focally fragmented elastin fibers were noted with Verhoeff elastic tissue stain. Alcian blue stain did not show increased dermal mucin. With the clinical presentation and histologic findings described, we diagnosed the patient with fibroblastic rheumatism (FR). To date, the patient's condition has stabilized overall with skin lesions fading and minimal to no joint pain. Current therapies include adalimumab, mycophenolate mofetil 500 mg 3 times daily, and low-dose prednisone.
Fibroblastic rheumatism is a rare arthropathy with cutaneous findings initially described by Chaouat et al1 in 1980. Age of onset varies, and the condition also has been observed in pediatric patients.2 Fibroblastic rheumatism is characterized by sudden onset of firm, flesh-colored, subcutaneous nodules on periungual and periarticular surfaces.2 Neck lesions rarely are described,2-4 and cordlike plaques previously have not been reported in FR. Typically, patients develop diffusely swollen fingers, palmar thickening, sclerodactyly, and contractures. The eruption may be accompanied by Raynaud phenomenon as well as a progressive symmetric erosive arthropathy.2,5
The clinical course in FR is variable. The cutaneous findings spontaneously may regress in months to years.3,4 However, polyarthropathy often is destructive and progresses to disability.3 Response to therapy has been unpredictable, and the following treatments have been tried, generally with poor efficacy: aspirin, nonsteroidal anti-inflammatory drugs, hydroxychloroquine, colchicine, methotrexate, prednisone, infliximab, D-penicillamine, interferon alfa, and intensive physical therapy.2-4,6 Histologic characteristics may include thickened collagen bundles along with a fibroblastic and myofibroblastic proliferation. Elastic fibers may be decreased or absent.2,3,5
Clinical and histologic features in FR may mimic other entities; thus, clinical pathological correlation is essential in determining the correct diagnosis. Considerations in the differential diagnoses include multicentric reticulohistiocytosis (MRH), palisaded neutrophilic and granulomatous dermatitis, and scleroderma.
In MRH, a symmetric erosive arthritis of mainly distal interphalangeal joints typically precedes the cutaneous disease. Occurrence of arthritis mutilans is reported in approximately half of patients.4 Cutaneous manifestations typically include the presence of coral bead-like papules and nodules over the dorsal aspect of the hands, face, and neck. Unlike FR, MRH has a concomitant autoimmune disease in up to 20% of cases and an associated malignancy in up to 31% of cases, with breast and ovarian carcinomas most common. On histology, MRH is characterized by a nodular infiltrate of histiocytes and multinucleated giant cells with eosinophilic ground-glass cytoplasm.4 No notable collagen changes or fibroblastic proliferations typically are present.
Palisaded neutrophilic and granulomatous dermatitis, usually associated with rheumatoid arthritis or connective tissue disease, classically presents as annular plaques and indurated linear bands over the trunk and extremities. However, its clinical presentation is quite variable and may include pink to violaceous urticarialike; livedoid-appearing; or nonspecific papules, plaques, or nodules. Histology in palisaded neutrophilic and granulomatous dermatitis shows a dense dermal neutrophilic infiltrate associated with interstitial histiocytes having a palisading arrangement around degenerated collagen.7 No fibroblastic proliferation typically is present.
Scleroderma can be distinguished based on additional clinical and laboratory findings as well as histology showing thickened collagen bundles without fibroblastic proliferation.2 The histologic findings also may suggest inclusion of dermatofibroma or a scar in the differential diagnosis, though the clinical presentation of these entities would not support these diagnoses.
Acknowledgments
We thank the patient for granting permission to share this information. We also thank Sheng Chen, MD, PhD (Lake Success, New York), for his dermatopathological contributions to the case.
The Diagnosis: Fibroblastic Rheumatism
Routine histologic sections stained with hematoxylin and eosin demonstrated a noncircumscribed dermal proliferation of fibroblasts and myofibroblasts with thickened collagen bundles (Figure, A and B). Focally fragmented elastin fibers were noted with Verhoeff elastic tissue stain. Alcian blue stain did not show increased dermal mucin. With the clinical presentation and histologic findings described, we diagnosed the patient with fibroblastic rheumatism (FR). To date, the patient's condition has stabilized overall with skin lesions fading and minimal to no joint pain. Current therapies include adalimumab, mycophenolate mofetil 500 mg 3 times daily, and low-dose prednisone.
Fibroblastic rheumatism is a rare arthropathy with cutaneous findings initially described by Chaouat et al1 in 1980. Age of onset varies, and the condition also has been observed in pediatric patients.2 Fibroblastic rheumatism is characterized by sudden onset of firm, flesh-colored, subcutaneous nodules on periungual and periarticular surfaces.2 Neck lesions rarely are described,2-4 and cordlike plaques previously have not been reported in FR. Typically, patients develop diffusely swollen fingers, palmar thickening, sclerodactyly, and contractures. The eruption may be accompanied by Raynaud phenomenon as well as a progressive symmetric erosive arthropathy.2,5
The clinical course in FR is variable. The cutaneous findings spontaneously may regress in months to years.3,4 However, polyarthropathy often is destructive and progresses to disability.3 Response to therapy has been unpredictable, and the following treatments have been tried, generally with poor efficacy: aspirin, nonsteroidal anti-inflammatory drugs, hydroxychloroquine, colchicine, methotrexate, prednisone, infliximab, D-penicillamine, interferon alfa, and intensive physical therapy.2-4,6 Histologic characteristics may include thickened collagen bundles along with a fibroblastic and myofibroblastic proliferation. Elastic fibers may be decreased or absent.2,3,5
Clinical and histologic features in FR may mimic other entities; thus, clinical pathological correlation is essential in determining the correct diagnosis. Considerations in the differential diagnoses include multicentric reticulohistiocytosis (MRH), palisaded neutrophilic and granulomatous dermatitis, and scleroderma.
In MRH, a symmetric erosive arthritis of mainly distal interphalangeal joints typically precedes the cutaneous disease. Occurrence of arthritis mutilans is reported in approximately half of patients.4 Cutaneous manifestations typically include the presence of coral bead-like papules and nodules over the dorsal aspect of the hands, face, and neck. Unlike FR, MRH has a concomitant autoimmune disease in up to 20% of cases and an associated malignancy in up to 31% of cases, with breast and ovarian carcinomas most common. On histology, MRH is characterized by a nodular infiltrate of histiocytes and multinucleated giant cells with eosinophilic ground-glass cytoplasm.4 No notable collagen changes or fibroblastic proliferations typically are present.
Palisaded neutrophilic and granulomatous dermatitis, usually associated with rheumatoid arthritis or connective tissue disease, classically presents as annular plaques and indurated linear bands over the trunk and extremities. However, its clinical presentation is quite variable and may include pink to violaceous urticarialike; livedoid-appearing; or nonspecific papules, plaques, or nodules. Histology in palisaded neutrophilic and granulomatous dermatitis shows a dense dermal neutrophilic infiltrate associated with interstitial histiocytes having a palisading arrangement around degenerated collagen.7 No fibroblastic proliferation typically is present.
Scleroderma can be distinguished based on additional clinical and laboratory findings as well as histology showing thickened collagen bundles without fibroblastic proliferation.2 The histologic findings also may suggest inclusion of dermatofibroma or a scar in the differential diagnosis, though the clinical presentation of these entities would not support these diagnoses.
Acknowledgments
We thank the patient for granting permission to share this information. We also thank Sheng Chen, MD, PhD (Lake Success, New York), for his dermatopathological contributions to the case.
- Chaouat Y, Aron-Brunetiere R, Faures B, et al. Une nouvelle entité: le rhumatisme fibroblastique. a propos d'une observation [in French]. Rev Rhum Mal Osteoartic. 1980;47:34-35.
- Jurado SA, Alvin GG, Selim MA, et al. Fibroblastic rheumatism: a report of 4 cases with potential therapeutic implications. J Am Acad Dermatol. 2012;66:959-965.
- Colonna L, Barbieri C, Di Lella G, et al. Fibroblastic rheumatism: a case without rheumatological symptoms. Acta Derm Venereol. 2002;82:200-203.
- Trotta F, Colina M. Multicentric reticulohistiocytosis and fibroblastic rheumatism. Best Pract Res Clin Rheumatol. 2012;26:543-557.
- Lee JM, Sundel RP, Liang MG. Fibroblastic rheumatism: case report and review of the literature. Pediatr Dermatol. 2002;19:532-535.
- Kluger N, Dumas-Tesici A, Hamel D, et al. Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosis. J Cutan Pathol. 2010;37:587-592.
- Stephenson SR, Campbell M, Dre GS, et al. Palisaded neutrophilic and granulomatous dermatitis presenting in a patient with rheumatoid arthritis on adalimumab. J Cutan Pathol. 2011;38:644-648.
- Chaouat Y, Aron-Brunetiere R, Faures B, et al. Une nouvelle entité: le rhumatisme fibroblastique. a propos d'une observation [in French]. Rev Rhum Mal Osteoartic. 1980;47:34-35.
- Jurado SA, Alvin GG, Selim MA, et al. Fibroblastic rheumatism: a report of 4 cases with potential therapeutic implications. J Am Acad Dermatol. 2012;66:959-965.
- Colonna L, Barbieri C, Di Lella G, et al. Fibroblastic rheumatism: a case without rheumatological symptoms. Acta Derm Venereol. 2002;82:200-203.
- Trotta F, Colina M. Multicentric reticulohistiocytosis and fibroblastic rheumatism. Best Pract Res Clin Rheumatol. 2012;26:543-557.
- Lee JM, Sundel RP, Liang MG. Fibroblastic rheumatism: case report and review of the literature. Pediatr Dermatol. 2002;19:532-535.
- Kluger N, Dumas-Tesici A, Hamel D, et al. Fibroblastic rheumatism: fibromatosis rather than non-Langerhans cell histiocytosis. J Cutan Pathol. 2010;37:587-592.
- Stephenson SR, Campbell M, Dre GS, et al. Palisaded neutrophilic and granulomatous dermatitis presenting in a patient with rheumatoid arthritis on adalimumab. J Cutan Pathol. 2011;38:644-648.
A 67-year-old man presented with asymptomatic plaques on the neck of 4 months' duration and nodules scattered over the hands, elbows, ears, and forehead of 3 years' duration. The eruption was associated with progressive thickening and contractures of the fingers, hand morning stiffness lasting less than 45 minutes, and Raynaud phenomenon. Physical examination revealed flesh-colored, firm, cordlike plaques on the neck bilaterally (top), with firm subcutaneous nodules on the helix and antihelix of the ears, forehead, elbows, and on the dorsal and ventral aspects of the hands (bottom). The largest nodules were approximately 5 cm. All fingers and first toes were thickened and firm with few contractile bands on the fingers. The patient had a persistently elevated erythrocyte sedimentation rate (80 mm/h)(reference range, 0-20 mm/h) and C-reactive protein level (3.27 mg/dL)(reference range, 0.00-0.40 mg/dL). Serologic workup was remarkable only for an antinuclear antibody titer of 1:80 (speckled). Plain radiographs confirmed an erosive arthropathy of the hands and feet. Erosions on the hands predominantly involved distal interphalangeal articulations, as well as, to a lesser extent, the proximal interphalangeal articulations, carpus, and the left distal radius. Erosive changes on the feet involved metatarsophalangeal, proximal interphalangeal, and distal interphalangeal articulations. Biopsies from the neck were performed for histopathologic correlation.
