Abstract

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 1-2 million cases per year.( 1 Fassnacht M. Terzolo M. Allolio B. Baudin E. Haak H. Berruti A. et al. Combination Chemotherapy in Advanced Adrenocortical Carcinoma. New England Journal of Medicine. 2012; 366: 2189-2197 Crossref PubMed Scopus (0) Google Scholar ) For patients with limited recurrent local or distant metastatic disease, resection is the most effective therapy and is associated with 5-year overall survival (OS) rates of up to 41%.( 2 Datrice N.M. Langan R.C. Ripley R.T. Kemp C.D. Steinberg S.M. Wood B.J. et al. Operative management for recurrent and metastatic adrenocortical carcinoma. Journal of Surgical Oncology. 2012; 105: 709-713 Crossref PubMed Scopus (0) Google Scholar ) However, for patients with advanced ACC not amenable to resection, outcome is typically poor, with low objective response rates (ORR; 14-23%) and median OS (14-25 months) to available chemotherapy regimens including immunotherapy or mitotane with multi-agent cytotoxic chemotherapy.( 1 Fassnacht M. Terzolo M. Allolio B. Baudin E. Haak H. Berruti A. et al. Combination Chemotherapy in Advanced Adrenocortical Carcinoma. New England Journal of Medicine. 2012; 366: 2189-2197 Crossref PubMed Scopus (0) Google Scholar , 3 Raj N. Zheng Y. Kelly V. Katz S.S. Chou J. Do R.K.G. et al. PD-1 Blockade in Advanced Adrenocortical Carcinoma. Journal of Clinical Oncology. 2020; 38: 71-80 Crossref PubMed Scopus (0) Google Scholar ).

Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call