S2k Guideline - Merkel cell carcinoma (MCC, neuroendocrine carcinoma of the skin) - Update 2022. 2023

Jürgen C Becker, and Ambros J Beer, and Viola K DeTemple, and Thomas Eigentler, and Michael Flaig, and Thilo Gambichler, and Stephan Grabbe, and Ulrike Höller, and Bernhard Klumpp, and Stephan Lang, and Claudia Pföhler, and Christian Posch, and Vikas Prasad, and Peter Schlattmann, and Sylke Schneider-Burrus, and Jan Ter-Nedden, and Patrick Terheyden, and Kai Thoms, and Dirk Vordermark, and Selma Ugurel
Translational Skin Cancer Research (TSCR), Deutsches Konsortium für translationale Krebsforschung (DKTK), Partnerstandort Essen, Department of Dermatology, University Hospital Essen, German Cancer Research Center (DKFZ), Heidelberg.

Merkel cell carcinoma (MCC, ICD-O M8247/3) is a rare, malignant, primary skin tumor with epithelial and neuroendocrine differentiation. The tumor cells share many morphologic, immunohistochemical, and ultrastructural features with cutaneous Merkel cells. Nevertheless, the cell of origin of MCC is unclear. MCC appears clinically as a reddish to purple spherical tumor with a smooth, shiny surface and a soft to turgid, elastic consistency, usually showing rapid growth. Spontaneous and often complete regressions of the tumor are observed. These likely immunologically-mediated regressions explain the cases in which only lymph node or distant metastases are found at the time of initial diagnosis and why the tumor responds very well to immunomodulatory therapies even at advanced stages. Due to its aggressiveness, the usually given indication for sentinel lymph node biopsy, the indication of adjuvant therapies to be evaluated, as well as the complexity of the necessary diagnostics, clinical management should already be determined by an interdisciplinary tumor board at the time of initial diagnosis.

UI MeSH Term Description Entries
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D015266 Carcinoma, Merkel Cell A carcinoma arising from MERKEL CELLS located in the basal layer of the epidermis and occurring most commonly as a primary neuroendocrine carcinoma of the skin. Merkel cells are tactile cells of neuroectodermal origin and histologically show neurosecretory granules. The skin of the head and neck are a common site of Merkel cell carcinoma, occurring generally in elderly patients. (Holland et al., Cancer Medicine, 3d ed, p1245) Merkel Cell Tumor,Merkel Cell Cancer,Merkel Cell Carcinoma,Merkle Tumors,Cancer, Merkel Cell,Cell Cancer, Merkel,Tumor, Merkel Cell,Tumors, Merkle
D018278 Carcinoma, Neuroendocrine A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992) Carcinomas, Neuroendocrine,Neuroendocrine Carcinoma,Neuroendocrine Carcinomas
D021701 Sentinel Lymph Node Biopsy A diagnostic procedure used to determine whether LYMPHATIC METASTASIS has occurred. The SENTINEL LYMPH NODE is the first lymph node to receive drainage from a neoplasm. Biopsy, Sentinel Lymph Node,Lymph Node Biopsy, Sentinel

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