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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2020; 41(03): 351-357
DOI: 10.4103/ijmpo.ijmpo_176_18
Original Article

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

Prasanta Kumar Tripathy
Department of Pediatric Surgery, SVP PG Institute of Pediatrics, SCB Medical College, Cuttack, Odisha
,
Kaumudee Pattnaik
Department of Pathology, SCB Medical College, Cuttack, Odisha
,
Pradeep Kumar Jena
Department of Pediatric Surgery, SVP PG Institute of Pediatrics, SCB Medical College, Cuttack, Odisha
,
Hiranya Kishor Mohanty
Department of Pediatric Surgery, SVP PG Institute of Pediatrics, SCB Medical College, Cuttack, Odisha
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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.

Keywords

Adrenal adenoma - adrenocortical carcinoma - neuroblastoma - pheochromocytoma


Publication History

Received: 12 August 2018

Accepted: 06 February 2020

Article published online:
28 June 2021

© 2020. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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