Abstract
Merkel cell carcinoma (MCC) is a rare but highly aggressive and rapidly expanding
malignant skin tumor. It affects the periocular region in approximately 10% of cases.
The current treatment
recommendation for resectable non-metastatic MCC comprises total surgical excision;
however, lymph node or distant metastases are often already present by the time of
the diagnosis. Since an
immune checkpoint inhibitor therapy with avelumab was first approved for MCC
in 2016, there has been considerable improvement in mean survival compared to cytostatic
therapy; at the same
time, there has been a reduction in serious treatment-associated adverse events.
Other immune checkpoint inhibitors are currently still in clinical trials, with very
promising initial
results. Because of the complexity of the diagnosis, treatment, and prognosis,
it is essential that MCC patients receive interdisciplinary care in a specialized
center including consultation
with a tumor review board.
Key words
immune checkpoint inhibitor - Merkel cell polyomavirus - PD-1 - PD-L1 - carcinoma
of the eyelid
