Our team based in Seattle conducted a comprehensive review including evolving trends in the management of Merkel cell carcinoma (MCC). This summary covers key decision points, including recommended work-up during initial diagnosis, treatment options for MCC when it’s in one place or has spread, management of recurrent MCC, and new treatments that are showing promise with fewer side effects and good results. This review gives valuable information on how to handle MCC overall and emphasizes new methods that are effective and less toxic on patients.
Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer that occurs in elderly patients. The risk of MCC is increased by immunosuppression due to conditions such as human immunodeficiency virus infection, hematological malignancies, and organ transplantation. These risk factors can also be associated with a poor prognosis in MCC. Chronic active Epstein-Barr virus (CAEBV) infection is a rare Epstein-Barr virus (EBV) infectious disease characterized by persistent infectious mononucleosis-like symptoms. Recently, programmed cell death ligand 1 (PD-L1) blockade using avelumab was approved as the first-line therapy for patients with metastatic MCC. Here, we report a case of extremely hyperprogressive MCC occurring in a patient with CAEBV.