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    Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline - Update 2022

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    Authors
    Gauci, M. L.
    Aristei, C.
    Becker, J. C.
    Blom, A.
    Bataille, V.
    Dreno, B.
    Del Marmol, V.
    Forsea, A. M.
    Fargnoli, M. C.
    Grob, J. J.
    Gomes, Fabio
    Hauschild, A.
    Hoeller, C.
    Harwood, C.
    Kelleners-Smeets, N.
    Kaufmann, R.
    Lallas, A.
    Malvehy, J.
    Moreno-Ramirez, D.
    Peris, K.
    Pellacani, G.
    Saiag, P.
    Stratigos, A. J.
    Vieira, R.
    Zalaudek, I.
    van Akkooi, A. C. J.
    Lorigan, Paul C
    Garbe, C.
    Lebbé, C.
    Show allShow less
    Affiliation
    Universite' de Paris, INSERM U976, AP-HP, Dermatology Department, Saint Louis Hospital, Paris, France
    Issue Date
    2022
    
    Metadata
    Show full item record
    Abstract
    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.
    Citation
    Gauci ML, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, et al. Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline – Update 2022. Vol. 171, European Journal of Cancer. Elsevier BV; 2022. p. 203–31.
    Journal
    European Journal of Cancer
    URI
    http://hdl.handle.net/10541/625385
    DOI
    10.1016/j.ejca.2022.03.043
    PubMed ID
    35732101
    Additional Links
    https://dx.doi.org/10.1016/j.ejca.2022.03.043
    Type
    Article
    Language
    en
    ae974a485f413a2113503eed53cd6c53
    10.1016/j.ejca.2022.03.043
    Scopus Count
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