ABSTRACT

Systemic therapy for Merkel cell carcinoma and dermal sarcomas is frequently utilized in the setting of metastatic or locally advanced disease with the goal of disease control, but likely not cure. Merkel cell carcinoma has previously been treated with chemotherapy with modest response rates; however, benefits from such treatment are short-lived, with virtually all patients progressing within several months. Elucidation of the complex interplay between Merkel cell carcinoma and the immune system has led to the approval of immune checkpoint inhibitors as a mainstay of systemic therapy. Early data have shown improved outcomes in overall survival and progression-free survival using such agents, and perhaps most promising, the potential for durable responses in a subset of patients.

Dermal sarcomas are a highly varied group of malignancies, with treatment regimens often extrapolated from the data of diverse study populations. While many systemic treatments are broadly applied to tumors within this category, distinct chemotherapeutic and targeted agents have shown success in managing specific subtypes. Given the rarity of each of these cancers, enrollment in ongoing clinical trials is critical in clarifying the optimal treatment of each.