Endocrine Abstracts

Background: Adrenocortical carcinoma (ACC) is a rare, often fatal endocrine cancer. Standard of care is driven by expert opinion, which advocates for: multidisciplinary evaluation, early oncologic surgical resection, complete pre-operative hormonal/imaging work-up, and avoiding adrenal mass biopsy.

Objective: We characterized factors dictating practice patterns and clinical outcomes for patients seen at a tertiary academic medical center with high referral volume for ACC.

Methods: We retrospectively collected clinical data for 75 adult patients with ACC referred to WashU from 2000-2024; complete data currently available for 68 (91%).

Results: 34/68 (50%) were female, median age 56 years (range 26-85), spanning all stages at diagnosis (34% I-II, 35% III, 31% IV). Median overall survival was 50.3 months, progression-free survival 7.8 months. 46/67 (69%) were diagnosed at community hospitals, though 41/57 (72%) received primary surgery in an academic setting (Chi-square P = 2.54x10-15). Type of hospital at diagnosis or surgery was not associated with differences in survival. Only 29/60 (48%) saw endocrinology prior to initial intervention, associated with more complete hormonal evaluation (Wilcoxon t-test P = 0.00037). 43/50 (86%) with adequate hormonal work-up had secretory tumors, with the following major secretion patterns: 30% cortisol+androgen, 28% androgen, 22% cortisol. 22/67 (33%) received adrenal biopsy despite imaging concerning for ACC; 8/22 (36%) were nondiagnostic. Adrenal biopsy was associated with higher risk of death in univariate models (HR 2.8,95%CI:1.3-6.0), but not multivariate models including stage (HR 1.2, 95%CI: 0.43-3.3). 17/65 (26%) had an antecedent adrenal nodule, with first documentation ranging from 0.32-9.63 years (median 5.09) prior to ACC diagnosis. Longer antecedent periods were associated with higher risk of death, even in multivariate models including stage (HR 1.1, 95%CI:1.01-1.3).

Discussion/Conclusion: ACC is frequently diagnosed in the community prior to academic referral, typically for surgery. Patients were not consistently referred to endocrinology prior to surgery, leading to incomplete hormonal evaluation. Adrenal biopsies were common, nondiagnostic, but not independently associated with worse survival. Many patients had antecedent adrenal nodules, indolent for years before evolving to ACC. These observations highlight a need to disseminate knowledge about appropriate work-up for adrenal tumors across practice settings, and add to literature questioning adequate duration for surveillance of incidentalomas.