Nature Reviews Disease Primers
October 26, 2017
Becker J, Stang A, DeCaprio J, Cerroni L, Lebbé C, Veness M, Nghiem PDownload PDF
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.
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The authoritative source on Merkel cell carcinoma.
September 22, 2020
- What is a Merkel cell?
- What is Merkel cell carcinoma?
- Symptoms & appearance of Merkel cell carcinoma
- Causes of Merkel cell carcinoma
- Surgical excision
- Mohs micrographic surgery
- Radiation therapy
- Complementary & alternative therapies
- Clinical trials
- Adjuvant Avelumab in Merkel Cell Carcinoma Trial