Published online by Cambridge University Press: 01 March 2011
A Merkel cell carcinoma is a primary cutaneous neuroendocrine carcinoma that often involves soft tissue, especially the subcutis. Metastatic carcinomas are common diagnostic problems, especially in determining their primary origin. When sarcomatoid, carcinomas often simulate primary soft tissue sarcomas. The discussion of carcinomas from specific sources is therefore twofold in this chapter: (1) histologic features and specific markers to evaluate the site of origin, and (2) problems related to sarcomatoid carcinomas of a particular organ site or tumor type. This chapter includes the most common carcinomas, and selected practical, well-established, and important markers are emphasized. Clinical or radiologic correlation is always necessary to confirm the primary site for a metastasis from an unknown source. Carcinosarcoma of the gynecological tract is discussed in Chapter 18.
MERKEL CELL CARCINOMA (PRIMARY NEUROENDOCRINE CARCINOMA OF THE SKIN, TRABECULAR CARCINOMA OF THE SKIN)
Merkel cell carcinoma is a distinctive, relatively rare primary cutaneous or subcutaneous high-grade neuroendocrine carcinoma, originally reported as trabecular carcinoma of the skin by Toker in 1972. Its ultrastructural resemblance to Merkel cells, the cutaneous neuroendocrine cells that are scattered in the basal epidermis and hair shafts, led to its being named Merkel cell carcinoma during the early 1980s. Positivity for keratin 20 also mirrors Merkel cells, but in neurofilament positivity it differs from normal Merkel cells. Therefore, the histogenetic origin of Merkel cell carcinoma from Merkel cells is not uniformly agreed on, and some authors prefer the designation of primary neuroendocrine carcinoma of the skin.
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