Pediatric and adolescent pheochromocytoma: clinical presentation and outcome of surgery.

OBJECTIVES To describe the clinical presentation and outcome of surgery in children with pheochromocytoma in a tertiary care hospital in India. METHODS Clinical records of 24 children who were operated between January 1990 and January 2011 were reviewed. The diagnosis of familial disease was established based on clinical examination and follow-up events. RESULTS Familial, bilateral, extra-adrenal and malignant pheochromocytoma were observed in 20.8%, 20.8%, 12.5% and 4.2% children, respectively. Median follow-up duration was 36 months. Persistent hypertension was noted in 12.5% patients and similar proportion died in follow-up. CONCLUSIONS In the absence of routine genetic screening, good history and long- term follow up are essential to rule out familial pheochromocytoma.