Functioning adrenal tumours in children and adolescents: An institutional experience

Background: The purpose of the present paper was to carry out an audit of c linicopathological profile and treatment outcome in 13 children with functi oning adrenal tumours. Methods: The medical records of 13 children with functioning adrenal tumour s who were managed between June 1990 and January 1999 were reviewed. Demogr aphic data, clinical features, biochemical and localization studies, operat ive details and follow-up records were studied. Children with neuroblastoma were excluded. Results: The mean age was 7.4 +/- 5.3 years. Seven patients had Cushing's s yndrome (CS), two patients had virilizing rumours, three patients had phaeo chromocytoma (PCC) and one patient had Conn's syndrome. All patients (excep t one child with CS) were treated surgically. Two children with adrenocorti cal carcinoma (ACCa) died during the perioperative period. Histopathologica l diagnosis was adrenal cortical adenoma (ACAd) in four patients, ACCa in f ive patients and PCC in three patients. Two ACCa patients died of metastase s at 12 and 14 months, respectively, while the third is alive and well at 3 0 months. Children with ACAd are alive and well at 91, 56, 32 and 27 months postoperatively. Children with PCC are free of disease (normal urinary met anephrines) at 63, 18 and 8 months after surgery but require antihypertensi ve drugs in low doses. Conclusion: The outcome of surgery is good in cases of ACAd and PCC. Althou gh outcome is poor in ACCa, surgery remains the mainstay of treatment and o ffers good palliation.