1. Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline – Update 2022
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Gauci, Marie-Léa, Aristei, Cynthia, Becker, Jurgen C, Blom, Astrid, Bataille, Veronique, Dreno, Brigitte, Del Marmol, Veronique, Forsea, Ana M, Fargnoli, Maria C, Grob, Jean-Jacques, Gomes, Fabio, Hauschild, Axel, Hoeller, Christoph, Harwood, Catherine, Kelleners-Smeets, Nicole, Kaufmann, Roland, Lallas, Aimilios, Malvehy, Josep, Moreno-Ramirez, David, Peris, Ketty, Pellacani, Giovanni, Saiag, Philippe, Stratigos, Alexander J, Vieira, Ricardo, Zalaudek, Iris, van Akkooi, Alexander C J, Lorigan, Paul, Garbe, Claus, Lebbé, Céleste, European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC), Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Università degli Studi di Perugia = University of Perugia (UNIPG), German Cancer Consortium [Heidelberg] (DKTK), Biomarqueurs et essais cliniques en Cancérologie et Onco-Hématologie (BECCOH), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Saclay, Hôpital Ambroise Paré [AP-HP], Mount Vernon Cancer Centre [Northwood, UK] (MV2C), Mount Vernon Cancer Centre, Nantes Université (Nantes Univ), Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre de Recherche en Cancérologie et Immunologie Nantes-Angers (CRCINA), Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes), Université libre de Bruxelles (ULB), University Emergency Hospital [Bucharest], University of L'Aquila [Italy] (UNIVAQ), Aix Marseille Université (AMU), Assistance Publique - Hôpitaux de Marseille (APHM), University of Manchester [Manchester], University Hospital Schleswig-Holstein [Kiel, Germany], Medizinische Universität Wien = Medical University of Vienna, Queen Mary University of London (QMUL), Maastricht University [Maastricht], Frankfurt University Hospital, Aristotle University of Thessaloniki, University of Barcelona, Hospital Universitario Virgen Macarena [Séville], Università cattolica del Sacro Cuore = Catholic University of the Sacred Heart [Roma] (Unicatt), Università degli Studi di Modena e Reggio Emilia = University of Modena and Reggio Emilia (UNIMORE), National and Kapodistrian University of Athens (NKUA), Coimbra Hospital and Universitary Centre [Coimbra, Portugal] (CHUC), Università degli studi di Trieste = University of Trieste, Antoni van Leeuwenhoek Hospital, Eberhard Karls Universität Tübingen = Eberhard Karls University of Tuebingen, Roche, AbbVie, Genesis Pharma, J.C. Becker reports receiving speaker’s bureau honoraria from Amgen, Pfizer, Recordati and Sanofi, is a paid consultant/advisory board member/DSMB member for Almirall, Boehringer Ingelheim, InProTher, ICON, MerckSerono, Pfizer, 4SC, and Sanofi/Regeneron. His group receives research grants from Bristol-Myers Squibb, Merck Serono, HTG, IQVIA, and Alcedis., Speaker’s bureau honoraria from Amgen, Pfizer, Recordati and Sanofi, paid consultant/advisory/DSMB board member for Almirall, Boehringer Ingelheim, ICON, InProTher, MerckSerono, Pfizer, 4SC, and Sanofi/Regeneron. Research grants from Bristol-Myers Squibb, Merck Serono, HTG, IQVIA, and Alcedis., Gauci, Marie-Léa, Aristei, Cynthia, Becker, Jurgen C, Blom, Astrid, Bataille, Veronique, Dreno, Brigitte, Del Marmol, Veronique, Forsea, Ana M, Fargnoli, Maria C, Grob, Jean-Jacque, Gomes, Fabio, Hauschild, Axel, Hoeller, Christoph, Harwood, Catherine, Kelleners-Smeets, Nicole, Kaufmann, Roland, Lallas, Aimilio, Malvehy, Josep, Moreno-Ramirez, David, Peris, Ketty, Pellacani, Giovanni, Saiag, Philippe, Stratigos, Alexander J, Vieira, Ricardo, Zalaudek, Iri, van Akkooi, Alexander C J, Lorigan, Paul, Garbe, Clau, and Lebbé, Céleste
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Male ,Cancer Research ,Skin Neoplasms ,Consensus ,Consensu ,HIV Infections/complications ,HIV Infections ,[SDV.CAN]Life Sciences [q-bio]/Cancer ,Guideline ,Guidelines ,Sentinel Lymph Node Biopsy/adverse effects ,Merkel Cell/diagnosis ,Humans ,HIV Infection ,Skin Neoplasms/diagnosis ,Neoplasm Staging ,MCC ,Merkel cell ,Manchester Cancer Research Centre ,Sentinel Lymph Node Biopsy ,ResearchInstitutes_Networks_Beacons/mcrc ,Carcinoma ,Carcinoma, Merkel Cell ,EDF ,Oncology ,Settore MED/35 - MALATTIE CUTANEE E VENEREE ,Human - Abstract
International audience; Merkel cell carcinoma (MCC) is a rare skin cancer, accounting for less than 1% of all cutaneous malignancies. It is found predominantly in white populations and risk factors include advanced age, ultraviolet exposure, male sex, immunosuppression, such as AIDS/HIV infection, haematological malignancies or solid organ transplantation, and Merkel cell polyomavirus infection. MCC is an aggressive tumour with 26% of cases presenting lymph node involvement at diagnosis and 8% with distant metastases. Five-year overall survival rates range between 48% and 63%. Two subsets of MCC have been characterised with distinct molecular pathogenetic pathways: ultraviolet-induced MCC versus virus-positive MCC, which carries a better prognosis. In both subtypes, there are alterations in the retinoblastoma protein and p53 gene structure and function. MCC typically manifests as a red nodule or plaque with fast growth, most commonly on sun exposed areas. Histopathology (small-cell neuroendocrine appearance) and immunohistochemistry (CK20 positivity and TTF-1 negativity) confirm the diagnosis. The current staging systems are the American Joint Committee on Cancer/Union for international Cancer control 8th edition. Baseline whole body imaging is encouraged to rule out regional and distant metastasis. For localised MCC, first-line treatment is surgical excision with postoperative margin assessment followed by adjuvant radiation therapy (RT). Sentinel lymph node biopsy is recommended in all patients with MCC without clinically detectable lymph nodes or distant metastasis. Adjuvant RT alone, eventually combined with complete lymph nodes dissection is proposed in case of micrometastatic nodal involvement. In case of macroscopic nodal involvement, the standard of care is complete lymph nodes dissection potentially followed by post-operative RT. Immunotherapy with anti-PD-(L)1 antibodies should be offered as first-line systemic treatment in advanced MCC. Chemotherapy can be used when patients fail to respond or are intolerant for anti-PD-(L)1 immunotherapy or clinical trials.
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- 2022