A surgical approach to adrenocortical tumors in children: The mainstay of treatment

Background: Adrenocortical tumors (ACTs) are rare in the pediatric population. The pathogenesis, prognostic indicators, and management of these tumors are still unclear because of its infrequent occurrence. This case series presents the surgical experience of the authors' center over 29 years. Methods: The medical records of children treated for ACTs between 1974 and 2003 were reviewed. Information on age, sex, presenting symptoms, hormonal levels, pathology, stage, treatment, and outcome was obtained. Results: Nine children (5 girls, 4 boys) were treated for ACTs. The median age at presentation was 29 months (range, 5 months to 11 years). Endocrine dysfunction was found in 8 patients. Four presented with virilizing symptoms, 4 presented with both virilizing and Cushing's symptoms, and 1 patient with Beckwith-Wiedemann syndrome was identified during routine screening. One was an adenoma, and 8 were carcinomas. Of the carcinomas, 3 were stage I, and 5 were stage II. The mean tumor weight was 125 g (range, 42 g to 336 g) with a mean volume of 139 cm(3) (range, 30 cm(3) to 626 cm(3)). All patients had complete excision of the tumor with spillage occurring in 2 cases. Lymph node biopsies were done in all but 2 patients. Two patients were treated with chemotherapy because of large tumor size and nodal involvement. All patients are doing well including those with tumor spillage. Conclusions: This study shows that surgical excision continues to be the mainstay of treatment for ACTs. Extensive lymph node biopsy in small ACTs can probably be avoided given the generally good outcome with surgery alone. The role of adjuvant chemotherapy remains unclear because most of the children in our series were effectively treated with surgical resection only. Patients should be enrolled in multicenter trials to assess the added value of chemotherapy.