7805 Adrenocortical Carcinoma - A Tertiary Center's Recent ∼10-Year Experience

Abstract Disclosure: E. Osher: None. K.M. Tordjman: None. Y. Sofer: None. N. Stern: None. J. Klausner: None. G. Lahat: None. N. Lubezky: None. Y. Goykhman: None. B. Sagie: None. G. Ravit: None. A. Asaf: None. K. Rivka: None. W. Ido: None. Y. Greenman: None. Background: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor prognosis. Prospective randomized controlled studies to evaluate the optimal treatment modalities are limited. Therefore, retrospective studies are needed. The aim of the study is to describe and summarize additional data and knowledge on treatment modalities in a tertiary medical center treating patients with ACC by a multidisciplinary expert team. Methods: A retrospective study. Results: The study cohort included 42 subjects (57% females), followed for a median of 26 (range 0.3-237) months. The mean age at diagnosis was 54±17 years; 60% of tumors were functioning, 57 % were cortisol-secreting, 41% were androgen-secreting, 12% aldosterone-secreting, and 45% were co-secreting. ENSAT stage at diagnosis was stage 1 in 19%, stage 2 in 26%, stage 3 in 24% and stage 4 in 29%. Eighty-eight % of patients underwent surgery; residual disease was in 21% post surgery. Treatment with mitotane was initiated in 62% of patients, reaching a mean maximal dose of 3.1 ±2 grams/day. Oncologic pharmacological treatment (Chemotherapy, TKI, Immunotherapy) and/or radiation were given in 50 % and 21%, respectively. In 14 /42 patients, genetic evaluation was done, in most cases, no target for treatment was found. 52% of patients died during follow-up; all cohort median time to death was 31(range 0.3-237) months. Time to death longer than 31 months was also seen in advanced disease stage 3- 3/9(33%) and stage 4-4/11(36%). Except association between mitotane and prolonged time to death (p=0.013), no other factors (age, gender, surgery, radiation, residual disease functional status), were associated with prolonged survival. Conclusions: ACC remains a rare disease with a poor prognosis. However, it is a heterogeneous disease, with some patients with advanced disease achieving more prolonged survival. Further characterization of these patients may improve our understanding of the biology and treatment of this rare disease. Presentation: 6/2/2024