Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline

Celeste Lebbe, Jürgen C. Becker, Jean Jacques Grob, Josep Malvehy, Veronique Del Marmol, Hubert Pehamberger, Ketty Peris, Philippe Saiag, Mark R. Middleton, Lars Bastholt, Alessandro Testori, Alexander Stratigos, Claus Garbe

Research output: Contribution to journalArticle

Abstract

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 1-2 cm 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 languageEnglish
Pages (from-to)2396-2403
Number of pages8
JournalEuropean Journal of Cancer
Volume51
Issue number16
DOIs
Publication statusPublished - Jun 15 2015

Keywords

  • Diagnosis
  • Merkel cell carcinoma
  • Radiotherapy
  • Surgical management
  • Systemic treatment

ASJC Scopus subject areas

  • Cancer Research
  • Oncology
  • Medicine(all)

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  • Cite this

    Lebbe, C., Becker, J. C., Grob, J. J., Malvehy, J., Del Marmol, V., Pehamberger, H., Peris, K., Saiag, P., Middleton, M. R., Bastholt, L., Testori, A., Stratigos, A., & Garbe, C. (2015). Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline. European Journal of Cancer, 51(16), 2396-2403. https://doi.org/10.1016/j.ejca.2015.06.131