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Merkel cell carcinoma
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Merkel cell carcinoma

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Contributors: Jeffrey M. Cohen MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Merkel cell carcinomas (MCCs, also known as cutaneous neuroendocrine carcinomas) are rare skin malignancies that demonstrate both neuroendocrine and epithelial differentiation. They are so named for their histological similarity to normal Merkel cells, which reside in the basal layer of the epidermis in the skin. They most frequently occur on the head and neck but can also be seen elsewhere on the body.

MCC occurred in 0.6 per 100 000 individuals in the United States in 2009. MCC favors older adults with a median age of 75-80 years old at the time of diagnosis. It is more common among individuals of Northern European descent than those of African, Asian, or Hispanic descent. MCC is also more common in the immunocompromised population. Two important etiologic factors for MCC are the Merkel cell polyomavirus and ultraviolet (UV) exposure. Patients with MCC who are polyomavirus negative have more aggressive tumors.

Affected individuals will typically present with an asymptomatic solitary nodule. Patients with metastatic disease may demonstrate weight loss, fatigue, and lymphadenopathy. Despite aggressive treatment, recurrence rates are high, metastases are common, and 5-year relative survival is approximately 60% in the United States based on data from a national database from 1973-1999.

Codes

ICD10CM:
C4A.9 – Merkel cell carcinoma, unspecified

SNOMEDCT:
253001006 – Merkel cell carcinoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 12/18/2017
Last Updated: 01/23/2018
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Merkel cell carcinoma
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Merkel cell carcinoma : Smooth papule, Tumor, Sun-exposed distribution, Firm nodule
Clinical image of Merkel cell carcinoma
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