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Merkel cell carcinoma (MCC) is a rare tumour of the skin of neuro-endocrine origin probably developing from neuronal mechanoreceptors. A collaborative group of multidisciplinary experts form the European Dermatology Forum (EDF), The European Association of Dermato-Oncology (EADO) and the European Organization of Research and Treatment of Cancer (EORTC) was formed to make recommendations on MCC diagnosis and management, based on a critical review of the literature, existing guidelines and expert's experience. Clinical features of the cutaneous/subcutaneous nodules hardly contribute to the diagnosis of MCC. The diagnosis is made by histopathology, and an incisional or excisional biopsy is mandatory. Immunohistochemical staining contributes to clarification of the diagnosis. Initial work-up comprises ultrasound of the loco-regional lymph nodes and total body scanning examinations. The primary tumour should be excised with 1-2cm margins. In patients without clinical evidence of regional lymph node involvement, sentinel node biopsy is recommended, if possible, and will be taken into account in a new version of the AJCC classification. In patients with regional lymph node involvement radical lymphadenectomy is recommended. Adjuvant radiotherapy might be considered in patients with multiple affected lymph nodes of extracapsular extension. In unresectable metastatic MCC mono- or poly-chemotherapy achieve high remission rates. However, responses are usually short lived. Treatment within clinical trials is regarded as a standard of care in disseminated MCC.

Original publication

DOI

10.1016/j.ejca.2015.06.131

Type

Journal article

Journal

Eur J Cancer

Publication Date

11/2015

Volume

51

Pages

2396 - 2403

Keywords

Diagnosis, Merkel cell carcinoma, Radiotherapy, Surgical management, Systemic treatment, Antineoplastic Combined Chemotherapy Protocols, Biomarkers, Tumor, Biopsy, Carcinoma, Merkel Cell, Consensus, Cooperative Behavior, Dermatology, Diagnostic Imaging, Europe, Humans, Immunohistochemistry, Interdisciplinary Communication, Lymph Node Excision, Lymphatic Metastasis, Medical Oncology, Neoplasm Staging, Predictive Value of Tests, Radiotherapy, Adjuvant, Skin Neoplasms, Treatment Outcome