Adrenal Tumors in Children: Spectrum of Presentation and Surgical Approach in a Tertiary Care Institute

Abstract

Abstract Context: Adrenal tumors can arise either from cortex or from medulla; both the regions being structurally and functionally different. Current knowledge on childhood adrenocortical tumors (ACTs), the management approach, and the outcome is limited due to limited number of cases and studies. Neuroblastoma is a neoplasm of the neural crest origin and 50% arise from adrenal medulla. Pheochromocytoma (PCC) is an exciting and rare neoplasm in childhood. Aims: To evaluate the clinical presentation, spectrum of pathology, and management of adrenal tumors in children. Subjects and Methods: The study was conducted in a tertiary care pediatric institute over 5 years on children with intra-abdominal solid tumors. Adrenal tumors were diagnosed on radiological studies and postoperatively confirmed by histopathology. Results: Sixty-three patients were operated for intra-abdominal solid tumors, and Wilms tumor (39) was the most common finding. Of 11 cases of adrenal tumors confirmed by histopathology, eight were adrenal neuroblastoma, one 14-month-old female baby was detected as PCC, while two female children presented with the features of Cushing syndrome and virilization at the age of 3 and 7 years. Postoperatively, one of them was found to be adrenal adenoma and one was found to be adrenocortical carcinoma. Conclusions: ACTs are rare and potentially fatal in childhood. Complete surgical resection is the mainstay of therapy in adrenal tumors and is curative for associated hypertension. Neuroblastoma was the third common intra-abdominal solid tumor after Wilms tumor and retroperitoneal teratoma. The patient with PCC contributed to “Rule of 10s” because of the absence of hypertension and childhood occurrence.