Sustained remission of Lynch syndrome-associated metastatic adrenocortical carcinoma following checkpoint inhibitor therapy-associated multiorgan autoimmunity.

Abstract

Adrenocortical carcinoma (ACC) is a rare and aggressive tumour with limited treatment options for metastatic disease1 . Immunotherapy with checkpoint inhibition, in particular anti-programmed death receptor 1 (PD-1)/programmed death-ligand-1 (PD-L1) blockade has transformed the management of several malignancies. We describe prolonged remission of an ectopic-ACTH secreting refractory stage-IV ACC treated with anti-PD-1 antibody nivolumab combined with anti-cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) antibody ipilimumab.