Treatment Outcomes in Patients with Recurrent Adrenocortical Carcinoma.

Background: While numerous studies have explored treatment outcomes for the overall ACC patient cohort, data on the subpopulation of patients with recurrent disease are limited. Therefore, the aim of this study was to assess treatment outcomes in patients with recurrent ACC.
Methods: In this retrospective study, we included 18 patients median age 49 years (42-62); 67% female) diagnosed with recurrent ENSAT stage I-III ACC who underwent either R0 ( n  = 16) or Rx ( n  = 2) surgical resection of the tumor.
Results: The median time from the initial surgery to ACC recurrence was 29 months (IQR 18-50). Seven patients (39%) manifested local recurrence, while 11 patients (61%) developed distant metastases. 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 either as an alone procedure ( n  = 4), or in combination with mitotane ( n  = 4), mitotane and chemotherapy ( n  = 1), or mitotane combined with radiotherapy ( n  = 1). The remaining patients received treatment involving chemotherapy±mitotane ( n  = 4) and locoregional therapy ±chemotherapy ( n  = 3). One patient chose not to proceed with further management and follow-up. The median PFS was 17 (95% CI 8-26) months while the median OS was not reached. In the multivariate model, increased mortality was associated with advanced age ( p  = 0.04) and a shorter interval to ACC recurrence ( p  = 0.03).
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.