Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with poor prognosis. Objective: The aim of this study is to characterize patients with ACC followed at a tertiary center. Material and Methods: Retrospective analysis of clinical records of patients with histopathological diagnosis of ACC followed in our clinic. Results and conclusions: We reviewed 11 patients. The average age at diagnosis was 57.3±15.2 years and 63.6% were females. Seven patients had a functioning adrenal carcinoma. At diagnosis, 72.7% of the patients presented weight loss, 36.4% back pain and 27.3% referred asthenia. Two patients presented Cushing’s syndrome, one hirsutism and three arterial hypertension and hypokalaemia. Regarding imaging data, the tumour was detected almost exclusively through abdominal computed tomography (CT); dimensions ranged from 3.8 to 20 cm with a median size of 10 cm; 54.5% were located on the left adrenal gland; 28.6% had signs of necrosis; 27.3% presented local invasion and 36.4% distant metastasis. Prognostic stratification based on ENSAT tumour stage was used; two (18.2%) patients were in stage I, four (36.4%) in stage II, one (9.1%) in stage III and four (36.4%) in stage IV. Ten patients (90.9%) underwent adrenalectomy, 70% through laparotomy. Presence of residual tumour could not be assessed(Rx) in 50% of the patients; total resection was reported in 10%; microscopic (R1) or macroscopic (R2) res ection margins were observed in 30% and 10%, respectively. The Weiss Score ranged from 3 to 6 (median of 3) and the Ki67 proliferative index ranged from 2 to 20 (median of 7). Postoperatively, 4 (36.3%) patients received only mitotane treatment and 3 (27.3%) patients were given systemic chemotherapy combined treatment with mitotane. Adjuvant radiotherapy was applied in two patients; and other two required palliative radiotherapy. One patient who received mitotane did not report any drug side effects. The symptoms reported were mostly gastrointestinal disturbances (81.4%, n=5); one patient presented neurotoxicity and other reported skin lesions and mucositis. Two patients even had to discontinue treatment du e to intolerance. Disease specific mortality was 85.7%. The mean overall survival was 62.1±14.7 months for stages I+II (ranging from 33.3 to 90.9 months) and 8.8±2.9 months for stages III+IV (ranging from 3.1 to 14.5 months), P=0.005. ENSAT staging at diagnosis was the major prognostic factor in our series.