Endocrine Abstracts

Introduction: Despite advancements in the treatment of adrenocortical carcinoma (ACC), the prognosis for metastatic disease remains poor. This study aims to identify prognostic factors for survival in patients with metastatic ACC.

Materials and Methods: This retrospective study included 23 patients with metastatic ACC treated between 2012 and 2023. The main outcomes were overall survival (OS), progression free survival (PFS) and disease specific survival (DSS).

Results: The median age of patients was 60 (IQR 48-67) years, with 80% being female. The median tumor size was 123 mm (IQR102-160), and 18 patients had Cushing syndrome. The liver and lung were the most frequently affected organs (14 and 18 patients, respectively). Fourteen patients underwent adrenalectomy (R2 resection). As the first line medical treatment, among the operated patients, three received mitotane monotherapy, and 11 received mitotane plus platinum-based chemotherapy. In the non-operated group, one patient received mitotane monotherapy, while five others were treated with a combination of mitotane and platinum-based chemotherapy. Three non-operated patients, who received no medical treatment, died within one month of diagnosis. Over a median follow-up of 9 months, (IQR 5-22), 21 patients died, 18 of whom from ACC. The median PFS was 3 months 95% CI (2-4), median OS was 9 months 95% CI (4-14), and median DSS was 9 months 95% CI (6-10). Patients who underwent adrenalectomy had longer OS compared to non-operated patients (18 months vs. 2 months; P = 0.0132). In contrast, patients with liver metastases had shorter OS (8 months vs. 22 months, P = 0.24) and tended to have shorter DSS (8 months vs. 17 months, P = 0.063). The presence of cortisol excess did not influence OS or DSS.

Conclusion: In patients with advanced ACC, the presence of liver metastases was associated with shorter OS. Conversely, surgical removal of the primary tumour was associated with longer OS.