Skip to main content
ENDO 2026 Main banner

Adrenal (Excluding Mineralocorticoids)

SAT-490 - Extended Survival in Metastatic Adrenocortical Carcinoma - Experience from a Tertiary Cancer Center

Saturday, June 13, 2026
9:30 AM - 4:30 PM CT
Location: ENDOExpo; Poster Floor



Background:
Advanced adrenocortical carcinoma (ACC) is associated with poor prognosis. Recent therapeutic advances and their integration into clinical practice may help to improve progression-free and overall survival. In this study, we describe the clinical and tumor characteristics of a cohort of ACC patients who achieved long-term survival (≥10 years) following a diagnosis of metastatic disease.

Method:
We conducted a retrospective, single-institution review of patients aged ≥18 years diagnosed with ACC and achieved more than 10 years of survival from the diagnosis of metastatic ACC. Patient demographics and tumor characteristics were extracted from medical records. The ENS@T staging system was used for ACC staging.

Results:
Among 496 metastatic or recurrent cases, we identified 27 patients (17 [63.0%] female) with a median age at ACC diagnosis of 42.1 years (interquartile range [IQR], 35.9–49.9). Of 20 evaluable patients, nine (33.3%) patients had non-functioning tumors, eight (29.6%) had cortisol-producing tumors (including mixed hormonal secretion), and three (11.1%) had non-cortisol-producing tumors at initial ACC diagnosis. Median tumor size was 12.0 cm (IQR, 9.3–16.5). Seven patients had Stage IV ACC at diagnosis, while 20 developed metastases later (six were Stage II at ACC diagnosis, five Stage III, and nine patients had non-metastatic disease at diagnosis with unavailable staging data). The distant metastasis among these 20 patients occurred after a median of 3.7 years (IQR 1.6-5.3) from the initial ACC diagnosis. Four (14.8%) patients had received neoadjuvant cytotoxic chemotherapy. All patients had undergone tumor resection; 13 (48.1%) with R0 resections, and 14 (51.9%) RX resections. In five available tumor samples, the median Ki-67 index was 10.0% (IQR, 6–13). The most common sites of metastases were liver (14 [52%] patients), soft tissue (10 [37%] patients), and lung (7 [26.0%] patients). Patients received a median of 4 lines of therapy (IQR, 3–8), including systemic and image-guided local treatments. The median follow-up was 13.6 years (IQR, 11.8–17.6; range, 10.8–25.6) since the diagnosis of distant metastases. At the cutoff date, 10 (37.0%) patients had died, and 17 (63.0%) were alive (9 [33.3%] with no evidence of disease, 5 [18.5%] with active disease, 3 [11.1%] with unknown disease status).

Conclusion:
Patients with ACC and either synchronous or metachronous metastases can have extended survival when treated with multimodal strategies, including curative-intent surgery. Although most patients experience disease progression, prolonged survival remains possible, underscoring the importance of individualized treatment plans. Further research into the factors that drive exceptional survival and the development of novel therapies for ACC is warranted.