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Merkel cell carcinoma: Epidemiology, pathogenesis, diagnosis and therapy

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Abstract

Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer with a neuroendocrine phenotype. Incidence varies according to the geographic regions but is overall increasing. Different risk factors have been identified namely advanced age, immunosuppression, and ultraviolet light exposure. An association between MCC and polyomavirus infection is known. However, the exact mechanism that leads to carcinogenesis is yet to be fully understood. Surgery when feasible is the recommended treatment for localized disease, followed by adjuvant radiation or chemoradiation. In the metastatic setting, chemotherapy has been the standard treatment. However, two recently published trials with immune checkpoint inhibitors in first and second line showed promising results with a tolerable safety profile and these might become the standard therapy shortly. Somatostatin receptors are expressed in many MCC but such expression is not associated with disease severity. Presently there are no biomarkers predictive of response that could help to better select patients to these new therapies, and additional research is essential.

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Acknowledgements

We would like to thank Ingrid de Léon for the figures.

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  1. Center for Dermatooncology, Department of Dermatology, University Hospital Tuebingen, Liebermeisterstr. 25, 72076, Tuebingen, Germany

    Teresa Amaral, Ulrike Leiter & Claus Garbe

  2. Portuguese Air Force Health Direction, Paço do Lumiar, 1649-020, Lisbon, Portugal

    Teresa Amaral

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Dr. Amaral reports non-financial support from GSK, non-financial support from P3solution, personal fees and non-financial support from Roche, personal fees and non-financial support from BMS, outside the submitted work.

Dr. Leiter reports personal fees from Roche, personal fees from Novartis, outside the submitted work.

Dr. Garbe reports personal fees from Amgen, grants and personal fees from BMS, grants and personal fees from Novartis, grants and personal fees from Roche, personal fees from MSD, personal fees from Philogen, outside the submitted work.

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Amaral, T., Leiter, U. & Garbe, C. Merkel cell carcinoma: Epidemiology, pathogenesis, diagnosis and therapy. Rev Endocr Metab Disord 18, 517–532 (2017). https://doi.org/10.1007/s11154-017-9433-0

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