Endocrine Abstracts

JOINT2123

Introduction: Metastatic adrenocortical carcinoma (ACC) is a highly aggressive disease with a poor prognosis and limited treatment options.

Materials and Methods: This retrospective study aims to evaluate the clinical outcomes of 29 patients with metastatic ACC: overall survival (OS), progression-free survival (PFS), and disease-specific survival (DSS).

Results: The median age of patients was 60 (IQR 53-68) years, with 76% being female. The median tumor size was 128 mm (IQR 112-160), and 22 patients had Cushing syndrome. The liver and lung were the most frequently affected organs, in 18 and 23 patients, respectively. Sixteen patients underwent an adrenalectomy with R2 resection. Postoperatively three patients received mitotane (M) monotherapy, twelve mitotane plus platinum-based chemotherapy (PE-M) and one received PE. In the non-operated group (n = 13), one patient received M monotherapy, two PE, and seven PE-M. Three patients died within one month of diagnosis and did not receive medical treatment. Among second-line therapies, four patients received gemcitabine and capecitabine plus M, while four others received sunitinib and one patient has been treated with cabozantinib. Over a median follow-up of 9 months (IQR 3–22), 24 patients died, with 21 deaths attributable to ACC. The median OS was 9 months (95% CI: 0.9–17.2), and the median DSS was also 9 months (95% CI: 0.9–17.2). The median PFS was 4 months (IQR 3–8). Patients who underwent adrenalectomy had significantly longer OS (18 months vs. 8 months, P = 0.037) and tended to have longer DSS (18 months vs. 8 months, P = 0.059) compared to non-operated patients. However, after excluding patients who died within three months of diagnosis (n = 7) the differences were no longer significant. The one-year mortality rate was 64% in patients with liver metastases, compared to 30% in those without (P = 0.036). Additionally, patients with liver metastases showed a trend toward shorter DSS (8 vs. 22 months, P = 0.056.). Similarly, there was a trend toward a lower one-year mortality in patients with only one affected organ compared to those with two or more (38% vs 64%; P = 0.083). In contrast, cortisol excess did not influence survival outcomes. In multivariate Cox regression analysis, older age was the only significant predictor of shorter OS (P = 0.013).

Conclusion: Older age is the only significant predictor of OS in patients with advanced ACC. However, liver metastases and the number of affected organs may negatively impact short-term survival within one year of diagnosis.