title: Diagnosis and treatment of Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline.
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 1-2cm 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.
authors.affiliation: APHP University Department of Dermatology, Saint-Louis Hospital, INSERM U976, Paris, France. Electronic address: celeste.lebbe@sls.aphp.fr.
authors.forename: Celeste
authors.initials: C
authors.lastname: Lebbe
authors.affiliation: University Department of Dermatology, Graz, Austria.
authors.forename: Jürgen C
authors.initials: JC
authors.lastname: Becker
authors.affiliation: University Department of Dermatology, Marseille, France.
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Grob
authors.affiliation: Melanoma Unit, Department of Dermatology, Hospital Clinic, Barcelona, Spain.
authors.forename: Josep
authors.initials: J
authors.lastname: Malvehy
authors.affiliation: University Department of Dermatology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium.
authors.forename: Veronique
authors.initials: V
authors.lastname: Del Marmol
authors.affiliation: University Department of Dermatology, Vienna, Austria.
authors.forename: Hubert
authors.initials: H
authors.lastname: Pehamberger
authors.affiliation: University Department of Dermatology, L'Aquila, Italy.
authors.forename: Ketty
authors.initials: K
authors.lastname: Peris
authors.affiliation: University Department of Dermatology, Université de Versailles-Saint Quentin en Yvelines, APHP, Boulogne, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Saiag
authors.affiliation: Department of Oncology, Oxford National Institute for Health Research Biomedical Research Centre, United Kingdom.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Middleton
authors.affiliation: Department of Oncology, Odense University Hospital, Denmark.
authors.forename: Lars
authors.initials: L
authors.lastname: Bastholt
authors.affiliation: Istituto Europeo di Oncologia, Divisione dermato-oncologica, Milan, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Testori
authors.affiliation: 1st Department of Dermatology, University of Athens, A. Sygros Hospital, Athens, Greece.
authors.forename: Alexander
authors.initials: A
authors.lastname: Stratigos
authors.affiliation: University Department of Dermatology, Tuebingen, Germany.
authors.forename: Claus
authors.initials: C
authors.lastname: Garbe
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000592:standards / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy / Q000592:standards; D015266:Carcinoma, Merkel Cell / Q000737:chemistry / Q000175:diagnosis* / Q000628:therapy*; D032921:Consensus; D003299:Cooperative Behavior; D003880:Dermatology / Q000592:standards*; D003952:Diagnostic Imaging / Q000592:standards; D005060:Europe; D006801:Humans; D007150:Immunohistochemistry / Q000592:standards; D033183:Interdisciplinary Communication; D008197:Lymph Node Excision / Q000592:standards; D008207:Lymphatic Metastasis; D008495:Medical Oncology / Q000592:standards*; D009367:Neoplasm Staging; D011237:Predictive Value of Tests; D018714:Radiotherapy, Adjuvant / Q000592:standards; D012878:Skin Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Diagnosis; Merkel cell carcinoma; Radiotherapy; Surgical management; Systemic treatment