Rapid disease progression in a patient with mismatch repair-deficient and cortisol secreting adrenocortical carcinoma treated with pembrolizumab

: Metastatic adrenocortical carcinoma (ACC) is an aggressive malignancy with a poor prognosis and limited therapeutic options. A subset of ACC is due to Lynch syndrome, an inherited tumor syndrome resulting from germline mutations in mismatch repair (MMR) genes. It has been demonstrated that several cancers characterized by MMR-deficiency are sensitive to immune checkpoint inhibitors that target PD-1. Here, we provide the first report of PD-1 blockade with pembrolizumab in a patient with Lynch syndrome and progressive cortisol-secreting metastatic ACC.

Source: Seminars in Oncology - June 21, 2018 Category: Cancer & Oncology Authors: R.T. Casey, O. Giger, I. Seetho, A. Marker, D. Pitfield, L.H. Boyle, M. Gurnell, A. Shaw, M. Tischowitz, E.R. Maher, V.K. Chatterjee, T. Janowitz, G. Mells, P. Corrie, B.G. Challis Source Type: research