ESPE Abstracts

Background: Adrenocortical tumors are rare tumors in children with very limited data to reveal outcome in resource- limited settings.

Materials and Methods: A retrospective cohort study was carried out to evaluate medical records of 24 children with adrenocortical tumors, who were registered at our tertiary care hospital from Jan 2012 to date.

Results: Total 24 children were included. Their ages ranged from 10 months to 6.5 years. 14 patients had Adrenocortical adenoma (ADA) while 10 children had adrenocortical carcinoma (ACC) with Weiss score of 4 or more (n = 6 were female as compared to n = 4 males). ADA had no gender predilection. The median age of presentation was 44 months (3.8 years). The median time for diagnosis after the onset of symptoms was 5.2 months. Most common clinical feature was isolated Cushing syndrome (68%, n = 7), virilization (58%, n = 6), while 46%, n = 5 had mixed clinical presentation. Metastatic disease was found in 12.5 % of patients(n = 2) at initial presentation. The preoperative diagnosis was based on clinical manifestations, elevated dehydroepiandrosterone sulphate levels, and suprarenal masses on USG adrenal glands and computed tomography abdomen and Dehydroepiandrosterone = 0.027) and testosterone (P = 0.012) were significantly raised in ACC. Tumor volumes in ACC was significantly large (P = 0.03) than those in ACA. All patients without metastasis underwent complete resection by laparotomy (n = 04) or laparoscopic surgery (n = 6). Total 6 patients were lost to follow up. Recurrence was observed in 2 patients (16.6%) after a mean duration of 14 months. Patients with carcinoma at 3.5-year follow up had overall survival (80%) as compared to 99 % in patients with adenomas. Currently 2 patients are on chemotherapy and on regular follow up. 2 patients with ACC had postoperative distant metastases and died within 2.5 years. Complete surgical resection in non-metastasized and un spilled tumors had excellent outcome.

Conclusion: Adrenocortical tumors are rare tumors in children. Adenomas have mostly benign course. There was no disease-free survival in incompletely resected or metastatic tumors. Tumor spill may also have an adverse outcome in completely resected tumors.

Keywords: Adenoma, adrenalectomy, adrenocortical tumors, Cushing syndrome