Endocrine Abstracts

Background: While a considerable body of literature exists detailing outcomes in patients with adrenocortical carcinoma (ACC), reports specifically addressing a cohort of patients with recurrent disease remain scarce. Therefore, the objective of this study was to evaluate treatment outcomes in patients with recurrent ACC.

Methods: In our cohort of 55 patients who underwent R0/Rx tumor resection between 2011 and 2022, 18 individuals (33%) exhibited recurrent disease and were consequently included in this study. Following disease recurrence, patients were treated with various modalities including surgery, mitotane, chemotherapy, locoregional therapy and radiotherapy. The main study outcomes were overall survival (OS) and progression free survival (PFS) following ACC recurrence.

Results: The median time from the initial surgery to ACC recurrence was 29 months (IQR 18–50), with three patients experiencing recurrence within 12 months post-surgery. Seven patients (39%) manifested local recurrence, while 11 patients (61%) developed distant metastases. Among those with distant metastases, seven exhibited lesions in a single organ, whereas four presented metastatic lesions in multiple organs. The median follow-up duration after tumor recurrence was 32 months (IQR 25–53). Regarding the treatment of ACC recurrence, 10 patients underwent a second surgery: surgery alone (n=4), surgery + mitotane (n=4), surgery + mitotane and chemotherapy (n=1) and surgery + radiotherapy and mitotane (n=1). Remaining patients were treated with chemotherapy±mitotane (n=4) and locoregional therapy (microwave ablation (MWA), polyvynil alcohol (PVA) embolisation)±chemotherapy (n=3). One patient chose not to proceed with further management and follow up. The median PFS was 17 (IQR 12-22) months while the median OS was not reached. Furthermore, the one, three and five-year OS rates were 94%, 68%, and 68%, respectively. In the multivariate model, increased mortality was associated with advanced age (P=0.04) and a shorter interval to ACC recurrence (P=0.05).

Conclusion: A significant proportion of patients with ACC recurrence experience disease progression or second recurrence, despite all treatment efforts. Nevertheless, by integrating diverse treatment modalities, many patients have the potential to attain long-term survival, underscoring the pivotal role of expert centers in their management.