Merkel cell carcinoma.
Identifieur interne : 000813 ( Main/Exploration ); précédent : 000812; suivant : 000814Merkel cell carcinoma.
Auteurs : Jürgen C. Becker [Allemagne] ; Andreas Stang [Allemagne] ; James A. Decaprio [États-Unis] ; Lorenzo Cerroni [Autriche] ; Celeste Lebbé [France] ; Michael Veness [Australie] ; Paul Nghiem [États-Unis]Source :
- Nature reviews. Disease primers [ 2056-676X ] ; 2017.
Abstract
Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer with neuroendocrine features. MCC pathogenesis is associated with either the presence of Merkel cell polyomavirus or chronic exposure to ultraviolet light (UV), which can cause a characteristic pattern of multiple DNA mutations. Notably, in the Northern hemisphere, the majority of MCC cases are of viral aetiology; by contrast, in areas with high UV exposure, UV-mediated carcinogenesis is predominant. The two aetiologies share similar clinical, histopathological and prognostic characteristics. MCC presents with a solitary cutaneous or subcutaneous nodule, most frequently in sun-exposed areas. In fact, UV exposure is probably involved in both viral-mediated and non-viral-mediated carcinogenesis, by contributing to immunosuppression or DNA damage, respectively. Confirmation of diagnosis relies on analyses of histological features and immunological marker expression profiles of the lesion. At primary diagnosis, loco-regional metastases are already present in ∼30% of patients. Excision of the tumour is the first-line therapy; if not feasible, radiotherapy can often effectively control the disease. Chemotherapy was the only alternative in advanced-stage or refractory MCC until several clinical trials demonstrated the efficacy of immune-checkpoint inhibitors.
DOI: 10.1038/nrdp.2017.77
PubMed: 29072302
Affiliations:
- Allemagne, Australie, Autriche, France, États-Unis
- Bade-Wurtemberg, District de Karlsruhe, Massachusetts, Nouvelle-Galles du Sud, Washington (État), Île-de-France
- Heidelberg, Paris, Seattle, Sydney
- Université de Sydney, Université de Washington
Links toward previous steps (curation, corpus...)
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- to stream PubMed, to step Checkpoint: 000350
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- to stream Ncbi, to step Checkpoint: 005103
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Le document en format XML
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<front><div type="abstract" xml:lang="en">Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer with neuroendocrine features. MCC pathogenesis is associated with either the presence of Merkel cell polyomavirus or chronic exposure to ultraviolet light (UV), which can cause a characteristic pattern of multiple DNA mutations. Notably, in the Northern hemisphere, the majority of MCC cases are of viral aetiology; by contrast, in areas with high UV exposure, UV-mediated carcinogenesis is predominant. The two aetiologies share similar clinical, histopathological and prognostic characteristics. MCC presents with a solitary cutaneous or subcutaneous nodule, most frequently in sun-exposed areas. In fact, UV exposure is probably involved in both viral-mediated and non-viral-mediated carcinogenesis, by contributing to immunosuppression or DNA damage, respectively. Confirmation of diagnosis relies on analyses of histological features and immunological marker expression profiles of the lesion. At primary diagnosis, loco-regional metastases are already present in ∼30% of patients. Excision of the tumour is the first-line therapy; if not feasible, radiotherapy can often effectively control the disease. Chemotherapy was the only alternative in advanced-stage or refractory MCC until several clinical trials demonstrated the efficacy of immune-checkpoint inhibitors.</div>
</front>
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<country name="France"><region name="Île-de-France"><name sortKey="Lebbe, Celeste" sort="Lebbe, Celeste" uniqKey="Lebbe C" first="Celeste" last="Lebbé">Celeste Lebbé</name>
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<country name="Australie"><region name="Nouvelle-Galles du Sud"><name sortKey="Veness, Michael" sort="Veness, Michael" uniqKey="Veness M" first="Michael" last="Veness">Michael Veness</name>
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