Impressive Response to Immunotherapy in a Patient with Metastatic Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare cancer with limited effective treatment options and poor prognosis. ACC incidence is 1 to 2 cases per million making randomized clinical trials difficult to conduct. The current standard first line treatment for metastatic ACC showing tumor response and improved progression free survival (PFS) is etoposide-doxorubicin-cisplatin (EDP) plus mitotane. Multiple efforts to find a targeted treatment for ACC have not shown impressive responses. Immunotherapy agents such as checkpoint inhibitors programmed cell death – 1 receptor (PD-1) and programmed cell deathligand 1 have shown significant responses in multiple malignancies including melanoma, non-small-cell-lung cancer, Hodgkin’s lymphoma to name a few. We present a case of a female patient with metastatic ACC involving the lungs, lymph nodes, and liver who progressed after EDP-mitotane, then had a significant decrease in tumor burden with anti-PD-1 agent pembrolizuamb.