Exploring the role of systemic therapy in adult adrenocortical carcinoma: A single-center experience

Background: Adrenocortical carcinoma (ACC) is a rare malignancy with poor outcomes. Objectives: To analyze the clinicopathologic features, treatment patterns and outcomes of patients with ACC who received systemic therapy at our center. Patients and Methods: This was a retrospective study conducted in a tertiary cancer center in India. Patients aged 15 years and older who were diagnosed with ACC between January 2011 and December 2018 and received systemic therapy were included in this study. For tumor staging, the European Network for the Study of Adrenal Tumors (ENSAT) system was used. The outcomes were reported as progression-free survival (PFS) and overall survival (OS). All statistical calculations were performed using the SPSS statistical software for Windows version 20.0. Results: Out of the 106 patients with ACC, 54 who received systemic therapy were included in this study. The median age of the cohort was 43 years (range, 15–72); 32 (59.3%) were men. Five (9.2%) patients had ENSAT Stage II, 31 (57.4%) had Stage III, and 18 (33.3%) had Stage IV (metastatic) disease at baseline. The chemotherapy drugs used in the palliative setting included etoposide (E), doxorubicin (D), and cisplatin (P), with or without mitotane. The median OS was 140 months (95% confidence interval [CI], 38.2–241.8) for ENSAT Stage II patients; 43 months for Stage III patients (95% CI, 27.2–58.7); and 22 months (95% CI, 9.4–34.6) for Stage IV patients, P = 0.012. The median PFS for patients treated with etoposide and platin (EP) and etoposide, doxorubicin, and platin (EDP) regimens was similar at 7 months (95% CI, 0–14.9) and 6 months (95% CI: 0–14.6) (P = 0.633), respectively. The corresponding median OS was 20.9 months (95% CI, 11.7–30.2) and 13.0 months (95% CI, 2.1–23.8) (P = 0.454), respectively. The patients who received palliative intent mitotane had a median PFS of 13 months (95% CI, 0–26.3) and those who did not had a median PFS of 6 months (95% CI, 1.2–10.7) (P = 0.492). The corresponding median OS was 22.6 months (95% CI, 17.8–27.5) and 15.5 months (95% CI, 5.8–25.2) (P = 0.351), respectively. Grade 3 or higher toxicities were observed in 25% of the patients receiving EP chemotherapy and 76.9% receiving EDP chemotherapy (P = 0.013). Conclusions: The use of mitotane is limited in the real-world setting in view of the financial constraints. The results with palliative chemotherapy in patients with ACC continue to remain poor. Patients with ACC treated with EDP and EP protocols had similar survival, but the three-drug protocol was associated with higher toxicities.