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Merkel Cell Carcinoma

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Neuroendocrine Tumors

Abstract

Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine malignancy. It is a rare tumor, but its incidence is increasing. MCC occurs most frequently in elderly Caucasian males on sun-exposed areas of the skin, and immunosuppression is a risk factor. There are two etiologically diverse subtypes of MCC: one due to clonal integration of the Merkel cell polyomavirus infection and the other due to the mutational effects of ultraviolet (UV) radiation. MCC has a propensity for early nodal spread, and sentinel lymph node biopsy is an important part of management. There is a paucity of evidence as to what constitutes optimal treatment of this rare malignancy. Surgery, in the form of wide excision, sentinel node lymph biopsy, and therapeutic lymphadenectomy, has been the traditional mainstay of MCC treatment. However, radiotherapy in the definitive and adjuvant settings is also an effective treatment, and emerging systemic treatments such as immune checkpoint inhibitors are showing great promise.

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Howle, J., Veness, M. (2021). Merkel Cell Carcinoma. In: Cloyd, J.M., Pawlik, T.M. (eds) Neuroendocrine Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-62241-1_16

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  • DOI: https://doi.org/10.1007/978-3-030-62241-1_16

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