Endocrine Abstracts

Background & Aim: Adrenocortical carcinoma (ACC) is an extremely rare malignancy usually with poor outcomes, although the prognosis varies greatly depending on the initial tumor stage. Here we present clinical and outcome diversity of the patients in a single center.

Methods: We retrospectively analyzed 16 patients with ACC diagnosed between 2000 and 2022. Demographical findings, hormonal status, radiological findings, ENSAT stage, weiss score and Ki67 levels, multidisciplinary treatment modalities and prognostic outcomes were evaluated.

Results: There were 8 (50%) women and men with a mean age of 56 (±12) years. Mean follow-up was 88 (±66) months, 11 (69%) patients were still alive without any metastasis and local recurrence. 5 patients died during follow-up, 4 of them were due to disease progression however one patient with complete remission were died due to myocardial infarction. Disease specific mortality was 25% with a mean survival of 69 months of these four patients. 25% of the patients were hormonally active predominantly hypercortisolism. The mean tumor size was 93.5±56.8 mm (range, 30-250 mm), 3 (18%) patients had local invasion and 3(18%) patients had distant metastasis including thorax, bone and liver metastasis. 16 patients (94%) underwent surgery, 5 of them had laporoscopic adrenalectomy, 2 patients had also metastasectomy and lymphnode dissection. Postoperative pathology revealed 4 (25%) oncocytic, 2 (12,5%) sarcomatoid, 2 (12,5%) mixoid and 8 (50%) clasiccal subtype ACC. 4 (25%) patients had venous and 12 (75%) had capsule invasion, mean Ki 67 score was 16 (range,2-90). According to ENSAT staging, two (12,5%) patients were stage 1, nine (56%) patients were stage 2, one was stage 3 and four (25%) were stage 4 disease. Six patients had adjuvant mitotane treatment and two patients had mitotane and EDP treatment. 2 of 6 patients on mitotane treatment had disease progression and added on EDP treatment. 2/6 patients were still on adjuvant mitotane and remaining 2/6 had remission and treatment stopped. All patients had drug toxicities in variable levels, majority of them were mild GIS symptoms and liver function test alterations, however one patient had moderate dermatological toxicity and one had neurologic toxicity necessitating dose decrement but recovered without any sequelae.

Conclusion: ACC has wide range of clinical course; therefore ACC management has to be patient specific. Mitotane is an effective treatment in properly selected patients and may be a curative treatment. Careful monitoring is mandatory to achieve disease control.