TY - JOUR
T1 - Diagnosis and treatment of Merkel cell carcinoma
T2 - European consensus-based interdisciplinary guideline - Update 2022
AU - Gauci, Marie-Léa
AU - Aristei, Cynthia
AU - Becker, Jurgen C
AU - Blom, Astrid
AU - Bataille, Veronique
AU - Dreno, Brigitte
AU - Del Marmol, Veronique
AU - Forsea, Ana M
AU - Fargnoli, Maria C
AU - Grob, Jean-Jacques
AU - Gomes, Fabio
AU - Hauschild, Axel
AU - Hoeller, Christoph
AU - Harwood, Catherine
AU - Kelleners-Smeets, Nicole
AU - Kaufmann, Roland
AU - Lallas, Aimilios
AU - Malvehy, Josep
AU - Moreno-Ramirez, David
AU - Peris, Ketty
AU - Pellacani, Giovanni
AU - Saiag, Philippe
AU - Stratigos, Alexander J
AU - Vieira, Ricardo
AU - Zalaudek, Iris
AU - van Akkooi, Alexander C J
AU - Lorigan, Paul
AU - Garbe, Claus
AU - Lebbé, Céleste
AU - European Dermatology Forum (EDF)
AU - European Association of Dermato-Oncology (EADO)
AU - European Organization for Research and Treatment of Cancer (EORTC)
N1 - Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.
PY - 2022/8
Y1 - 2022/8
N2 - 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.
AB - 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.
KW - Carcinoma, Merkel Cell/diagnosis
KW - Consensus
KW - HIV Infections/complications
KW - Humans
KW - Male
KW - Neoplasm Staging
KW - Sentinel Lymph Node Biopsy/adverse effects
KW - Skin Neoplasms/diagnosis
U2 - 10.1016/j.ejca.2022.03.043
DO - 10.1016/j.ejca.2022.03.043
M3 - (Systematic) Review article
C2 - 35732101
SN - 0959-8049
VL - 171
SP - 203
EP - 231
JO - European Journal of Cancer
JF - European Journal of Cancer
ER -