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Merkel Cell Carcinoma

24

 

Stephanie A. Diamantis and Victor J. Marks

 

Abstract

Merkel cell carcinoma (MCC) is a rare, aggressive tumor of neuroendocrine derivation mostly seen in elderly and immunosuppressed individuals. MCC has a propensity for local recurrence and regional and distant metastases. Because of the rarity of MCC, no evidenced-based unified treatment algorithm exists. Surgery is the mainstay of treatment for localized MCC. Mohs micrographic surgery (MMS) may be a tissue-sparing alternative to wide local excision. Radiation of the primary tumor site and/or regional lymph node basin may favorably improve recurrence rates. Chemotherapy is an option for inoperable or widespread disease. Prognosis is generally poor, especially if regional and distant metastases are present.

Keywords

Merkel cell carcinoma • Mohs micrographic surgery • Merkel cell polyomavirus

• Trabecular cell carcinoma • Wide local excision

Summary: Overview of Merkel Cell Carcinoma

Merkel cell polyomavirus may play a role in the development of Merkel cell carcinoma.

Merkel cell carcinoma is more common in the elderly, white, and immunosuppressed populations.

The most common location for Merkel cell carcinoma is the head and neck.

S.A. Diamantis (*)

Department of Dermatology, Geisinger Medical Center, Danville, PA, USA

e-mail: sadiamantis@geisinger.edu

V.J. Marks

Department of Dermatologic Surgery, Geisinger Health

System, Danville, PA, USA

24.1Overview of Merkel Cell Carcinoma

Merkel cell carcinoma (also called trabecular cell carcinoma, primary cutaneous neuroendocrine carcinoma, and primary small cell carcinoma of the skin) was initially described by Toker in 1972 [1–3]. The cell of origin is the Merkel cell, a mechanoreceptor usually found in the basal layer of the epidermis [4, 5].

Recent work by Feng et al. describes a virus, the Merkel cell polyomavirus (MCV), integrated into Merkel cell carcinoma (MCC) tumor cell DNA. In 10 patients with MCC, 8 (80%) were found to have evidence of genomic sequences containing MCV in their tumors. The majority of these tumors showed a clonal pattern of integrated viral DNA in the MCC tumor genome, arguing for an oncogenic role of MCV. Only

K. Nouri (ed.), Mohs Micrographic Surgery,

287

DOI 10.1007/978-1-4471-2152-7_24, © Springer-Verlag London Limited 2012