Painless Telangiectatic Lesion on the Wrist

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Painless Telangiectatic Lesion on the Wrist
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Kristen Wozniak, FNP
Sabina Shamayeva, BS
Juliana Basko-Plluska, MD
Amy J. Derick, MD

The Diagnosis: Merkel Cell Carcinoma

A partial biopsy was performed during the dermatology examination. Histopathology demonstrated a dense dermal infiltrate of small, dark blue, pleomorphic cells (Figure 1). On high power, the individual cells were noted to have vesicular nuclei with finely granular and dusty chromatin (Figure 2). Numerous mitotic figures were present. Immunohistochemical stains were performed and revealed positive staining for cytokeratin 20 (with a perinuclear dot pattern), synaptophysin, and chromogranin.

Figure 1. A biopsy of the lesion showed a small blue cell tumor located primarily in the dermis (H&E, original magnification ×4).

Figure 2. On high power, the cells were noted to have vesicular nuclei containing small nucleoli as well as granular and dusty chromatin (H&E, original magnification ×20).

Merkel cell carcinoma (MCC) is an uncommon carcinoma of the epidermal neuroendocrine cells with approximately 1500 cases a year in the United States.1 Merkel cell carcinoma has a poor prognosis with approximately one-third of cases resulting in death within 5 years and with a survival rate strongly dependent on the stage of disease at presentation.2 A complete surgical excision with histologically verified clear margins is the main form of treatment of the primary cancer.3 Although the effectiveness of adjuvant therapy for MCC has been debated,4 retrospective analysis has shown that the high local recurrence rate of the primary tumor can be reduced by combining surgical excision with a form of radiation therapy.5

A systematic cohort study of 195 patients diagnosed with MCC summarized its most clinical factors with the acronym AEIOU: asymptomatic, expanding rapidly, immunosuppression, older than 50 years of age, and UV-exposed site on a fair-skinned individual.6 The role of immune function in MCC was highlighted by a 16-fold overrepresentation of immunosuppressed patients in the studied cohort as compared to the general US population. The immunosuppressed patients included individuals with human immunodeficiency virus, chronic lymphocytic leukemia, and iatrogenic suppression secondary to solid organ transplantation.6

In 2008, Merkel cell polyomavirus (MCPyV) was found in 80% (8/10) of MCC tumors tested.7 Since then, many different studies have suggested that MCPyV is an etiologic agent of MCC.8-10 A natural component of skin flora, MCPyV only becomes tumorigenic after integration into the host DNA and with mutations to the viral genome.11 Although there currently is no difference in treatment of MCPyV-positive and MCPyV-negative MCC,12 research is being done to determine how the discovery of the MCPyV could impact the treatment of MCC.

References
  1. Albores-Saavedra J, Batich K, Chable-Montero F, et al. Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study. J Cutan Pathol. 2009;37:20-27.
  2. Allen PJ, Bowne WB, Jaques DP, et al. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005;23:2300-2309.
  3. Eng TY, Boersma MG, Fuller CD, et al. A comprehensive review of the treatment of Merkel cell carcinoma. Am J Clin Oncol. 2007;30:624-636.
  4. Beenken SW, Urist MM. Treatment options for Merkel cell carcinoma. J Natl Compr Canc Netw. 2004;2:89-92.
  5. Decker RH, Wilson LD. Role of radiotherapy in the management of Merkel cell carcinoma of the skin. J Natl Compr Canc Netw. 2006;4:713-718.
  6. Heath M, Jaimes N, Lemos B, et al. Clinical characteristics of Merkel cell  carcinoma at diagnosis in 195 patients: the "AEIOU" features. J Am Acad Dermatol. 2008;58:375-381.
  7. Feng H, Shuda M, Chang Y, et al. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008;319:1096-1100.
  8. Duncavage EJ, Zehnbauer BA, Pfeifer JD. Prevalence of Merkel cell polyomavirus in Merkel cell carcinoma. Mod Pathol. 2009;22:516-521.
  9. Sastre-Garau X, Peter M, Avril MF, et al. Merkel cell carcinoma of the skin: pathological and molecular evidence for a causative role of MCV in oncogenesis. J Pathol. 2009;218:48-56.
  10. Varga E, Kiss M, Szabó K, et al. Detection of Merkel cell polyomavirus DNA in Merkel cell carcinomas. Br J Dermatol. 2009;161:930-932.
  11. Wendzicki JA, Moore PS, Chang Y. Large T and small T antigens of Merkel cell carcinoma. Curr Opin Virol. 2015;11:38-43.
  12. Duprat JP, Landman G, Salvajoli JV, et al. A review of the epidemiology and treatment of Merkel cell carcinoma. Clinics (Sao Paulo). 2011;66:1817-1823.
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From Derick Dermatology, Barrington, Illinois.

The authors report no conflict of interest.

Correspondence: Kristen Wozniak, FNP, 1531 S Grove Ave #101, Barrington, IL 60010 (Kwozniak@derickdermatology.com).

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Author(s)
Kristen Wozniak, FNP
Sabina Shamayeva, BS
Juliana Basko-Plluska, MD
Amy J. Derick, MD
Author(s)
Kristen Wozniak, FNP
Sabina Shamayeva, BS
Juliana Basko-Plluska, MD
Amy J. Derick, MD
Author and Disclosure Information

From Derick Dermatology, Barrington, Illinois.

The authors report no conflict of interest.

Correspondence: Kristen Wozniak, FNP, 1531 S Grove Ave #101, Barrington, IL 60010 (Kwozniak@derickdermatology.com).

Author and Disclosure Information

From Derick Dermatology, Barrington, Illinois.

The authors report no conflict of interest.

Correspondence: Kristen Wozniak, FNP, 1531 S Grove Ave #101, Barrington, IL 60010 (Kwozniak@derickdermatology.com).

Article PDF
CT100004007_e.PDF
Article PDF
CT100004007_e.PDF

The Diagnosis: Merkel Cell Carcinoma

A partial biopsy was performed during the dermatology examination. Histopathology demonstrated a dense dermal infiltrate of small, dark blue, pleomorphic cells (Figure 1). On high power, the individual cells were noted to have vesicular nuclei with finely granular and dusty chromatin (Figure 2). Numerous mitotic figures were present. Immunohistochemical stains were performed and revealed positive staining for cytokeratin 20 (with a perinuclear dot pattern), synaptophysin, and chromogranin.

Figure 1. A biopsy of the lesion showed a small blue cell tumor located primarily in the dermis (H&E, original magnification ×4).

Figure 2. On high power, the cells were noted to have vesicular nuclei containing small nucleoli as well as granular and dusty chromatin (H&E, original magnification ×20).

Merkel cell carcinoma (MCC) is an uncommon carcinoma of the epidermal neuroendocrine cells with approximately 1500 cases a year in the United States.1 Merkel cell carcinoma has a poor prognosis with approximately one-third of cases resulting in death within 5 years and with a survival rate strongly dependent on the stage of disease at presentation.2 A complete surgical excision with histologically verified clear margins is the main form of treatment of the primary cancer.3 Although the effectiveness of adjuvant therapy for MCC has been debated,4 retrospective analysis has shown that the high local recurrence rate of the primary tumor can be reduced by combining surgical excision with a form of radiation therapy.5

A systematic cohort study of 195 patients diagnosed with MCC summarized its most clinical factors with the acronym AEIOU: asymptomatic, expanding rapidly, immunosuppression, older than 50 years of age, and UV-exposed site on a fair-skinned individual.6 The role of immune function in MCC was highlighted by a 16-fold overrepresentation of immunosuppressed patients in the studied cohort as compared to the general US population. The immunosuppressed patients included individuals with human immunodeficiency virus, chronic lymphocytic leukemia, and iatrogenic suppression secondary to solid organ transplantation.6

In 2008, Merkel cell polyomavirus (MCPyV) was found in 80% (8/10) of MCC tumors tested.7 Since then, many different studies have suggested that MCPyV is an etiologic agent of MCC.8-10 A natural component of skin flora, MCPyV only becomes tumorigenic after integration into the host DNA and with mutations to the viral genome.11 Although there currently is no difference in treatment of MCPyV-positive and MCPyV-negative MCC,12 research is being done to determine how the discovery of the MCPyV could impact the treatment of MCC.

The Diagnosis: Merkel Cell Carcinoma

A partial biopsy was performed during the dermatology examination. Histopathology demonstrated a dense dermal infiltrate of small, dark blue, pleomorphic cells (Figure 1). On high power, the individual cells were noted to have vesicular nuclei with finely granular and dusty chromatin (Figure 2). Numerous mitotic figures were present. Immunohistochemical stains were performed and revealed positive staining for cytokeratin 20 (with a perinuclear dot pattern), synaptophysin, and chromogranin.

Figure 1. A biopsy of the lesion showed a small blue cell tumor located primarily in the dermis (H&E, original magnification ×4).

Figure 2. On high power, the cells were noted to have vesicular nuclei containing small nucleoli as well as granular and dusty chromatin (H&E, original magnification ×20).

Merkel cell carcinoma (MCC) is an uncommon carcinoma of the epidermal neuroendocrine cells with approximately 1500 cases a year in the United States.1 Merkel cell carcinoma has a poor prognosis with approximately one-third of cases resulting in death within 5 years and with a survival rate strongly dependent on the stage of disease at presentation.2 A complete surgical excision with histologically verified clear margins is the main form of treatment of the primary cancer.3 Although the effectiveness of adjuvant therapy for MCC has been debated,4 retrospective analysis has shown that the high local recurrence rate of the primary tumor can be reduced by combining surgical excision with a form of radiation therapy.5

A systematic cohort study of 195 patients diagnosed with MCC summarized its most clinical factors with the acronym AEIOU: asymptomatic, expanding rapidly, immunosuppression, older than 50 years of age, and UV-exposed site on a fair-skinned individual.6 The role of immune function in MCC was highlighted by a 16-fold overrepresentation of immunosuppressed patients in the studied cohort as compared to the general US population. The immunosuppressed patients included individuals with human immunodeficiency virus, chronic lymphocytic leukemia, and iatrogenic suppression secondary to solid organ transplantation.6

In 2008, Merkel cell polyomavirus (MCPyV) was found in 80% (8/10) of MCC tumors tested.7 Since then, many different studies have suggested that MCPyV is an etiologic agent of MCC.8-10 A natural component of skin flora, MCPyV only becomes tumorigenic after integration into the host DNA and with mutations to the viral genome.11 Although there currently is no difference in treatment of MCPyV-positive and MCPyV-negative MCC,12 research is being done to determine how the discovery of the MCPyV could impact the treatment of MCC.

References
  1. Albores-Saavedra J, Batich K, Chable-Montero F, et al. Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study. J Cutan Pathol. 2009;37:20-27.
  2. Allen PJ, Bowne WB, Jaques DP, et al. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005;23:2300-2309.
  3. Eng TY, Boersma MG, Fuller CD, et al. A comprehensive review of the treatment of Merkel cell carcinoma. Am J Clin Oncol. 2007;30:624-636.
  4. Beenken SW, Urist MM. Treatment options for Merkel cell carcinoma. J Natl Compr Canc Netw. 2004;2:89-92.
  5. Decker RH, Wilson LD. Role of radiotherapy in the management of Merkel cell carcinoma of the skin. J Natl Compr Canc Netw. 2006;4:713-718.
  6. Heath M, Jaimes N, Lemos B, et al. Clinical characteristics of Merkel cell  carcinoma at diagnosis in 195 patients: the "AEIOU" features. J Am Acad Dermatol. 2008;58:375-381.
  7. Feng H, Shuda M, Chang Y, et al. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008;319:1096-1100.
  8. Duncavage EJ, Zehnbauer BA, Pfeifer JD. Prevalence of Merkel cell polyomavirus in Merkel cell carcinoma. Mod Pathol. 2009;22:516-521.
  9. Sastre-Garau X, Peter M, Avril MF, et al. Merkel cell carcinoma of the skin: pathological and molecular evidence for a causative role of MCV in oncogenesis. J Pathol. 2009;218:48-56.
  10. Varga E, Kiss M, Szabó K, et al. Detection of Merkel cell polyomavirus DNA in Merkel cell carcinomas. Br J Dermatol. 2009;161:930-932.
  11. Wendzicki JA, Moore PS, Chang Y. Large T and small T antigens of Merkel cell carcinoma. Curr Opin Virol. 2015;11:38-43.
  12. Duprat JP, Landman G, Salvajoli JV, et al. A review of the epidemiology and treatment of Merkel cell carcinoma. Clinics (Sao Paulo). 2011;66:1817-1823.
References
  1. Albores-Saavedra J, Batich K, Chable-Montero F, et al. Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study. J Cutan Pathol. 2009;37:20-27.
  2. Allen PJ, Bowne WB, Jaques DP, et al. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol. 2005;23:2300-2309.
  3. Eng TY, Boersma MG, Fuller CD, et al. A comprehensive review of the treatment of Merkel cell carcinoma. Am J Clin Oncol. 2007;30:624-636.
  4. Beenken SW, Urist MM. Treatment options for Merkel cell carcinoma. J Natl Compr Canc Netw. 2004;2:89-92.
  5. Decker RH, Wilson LD. Role of radiotherapy in the management of Merkel cell carcinoma of the skin. J Natl Compr Canc Netw. 2006;4:713-718.
  6. Heath M, Jaimes N, Lemos B, et al. Clinical characteristics of Merkel cell  carcinoma at diagnosis in 195 patients: the "AEIOU" features. J Am Acad Dermatol. 2008;58:375-381.
  7. Feng H, Shuda M, Chang Y, et al. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008;319:1096-1100.
  8. Duncavage EJ, Zehnbauer BA, Pfeifer JD. Prevalence of Merkel cell polyomavirus in Merkel cell carcinoma. Mod Pathol. 2009;22:516-521.
  9. Sastre-Garau X, Peter M, Avril MF, et al. Merkel cell carcinoma of the skin: pathological and molecular evidence for a causative role of MCV in oncogenesis. J Pathol. 2009;218:48-56.
  10. Varga E, Kiss M, Szabó K, et al. Detection of Merkel cell polyomavirus DNA in Merkel cell carcinomas. Br J Dermatol. 2009;161:930-932.
  11. Wendzicki JA, Moore PS, Chang Y. Large T and small T antigens of Merkel cell carcinoma. Curr Opin Virol. 2015;11:38-43.
  12. Duprat JP, Landman G, Salvajoli JV, et al. A review of the epidemiology and treatment of Merkel cell carcinoma. Clinics (Sao Paulo). 2011;66:1817-1823.
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Painless Telangiectatic Lesion on the Wrist
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A 91-year-old white man with a history of atrial fibrillation, benign prostatic hyperplasia, dysphagia, gastroesophageal reflux disease, hypertension, hypothyroidism, osteoarthritis, and laryngeal cancer presented with an 8-mm firm, painless, pink lesion with telangiectasia on the left wrist. The lesion had been present for an unknown period of time and was asymptomatic at presentation.

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