Surgical aspects of adrenal tumors in children

Abstract

Background: Adrenal tumors are uncommon in children. They can be divided into 2 categories, namely: adrenomedullary tumor and adrenocortical tumor. Surgical aspects of these conditions receive little attention.

Objective: The objective of this study was to describe clinical data, operative findings, and operative complications in children with adrenal tumors.

Methods: Patients, aged 0 - 15 years, undergoing adrenalectomy between January 2007 and December 2016 were retrospectively reviewed.

Results: Thirty-seven patients underwent adrenalectomies. Male to female ratio was 1.31 to 1. Median age of the patients was 44 months (range, 2 months-14 years). Pathological examination revealed 27 adrenomedullary tumors (19 neuroblastomas, 5 ganglioneuroblastomas, 2 ganglioneuromas, and 1 pheochromocytoma) and 10 adrenocortical tumors (8 adrenocortical carcinomas, 1 adrenocortical adenoma, and 1 micronodular adrenal hyperplasia). Most patients (35/37) underwent open adrenalectomies. As for neuroblastomas, most patients were in stage IV. Two ganglioneuromas and one ganglioneuroblastoma were diagnosed and treated as neuroblastoma before adrenalectomy. Pre-operative median tumor-size was 6.3 cm. In neuroblastic cases, complete resection could be achieved in 54.0% (14/26). Post-operative complications were reported in 19.0% (5/26) including chylous ascites, pancreatitis, and sepsis. As for adrenocortical tumors, all were functioning. Cushing syndrome (70.0%) is the most common presentation followed by precocious puberty (60.0%). Only one adrenocortical carcinoma patient presented with adrenal mass and lung metastasis. Most adrenocortical tumors exhibited hypervascularity. Median tumor-size was 6.3 cm (range 0.4 to 23 cm). Tumor with local invasion was found in 2 cases. No peri-operative complication was reported.

Conclusions: As for adrenomedullary tumors, neuroblastoma stage IV is the main indication for surgery in our series. This implies either delayed diagnosis or delayed medical attention. For adrenocortical tumors, surgical resection could be safely performed without significant morbidity.