Twenty-five-year surgical experience with pheochromocytoma in children

Our objective was to analyze the presentation, diagnostic localization, ope rative management, histology, and long-term out-come of a single center's e xperience with pheochromocytomas in children. A chart review was done to id entify all operatively managed pheochromocytomas in patients age 18 years o r younger. Open and laparoscopic cases were included. We reviewed the prese ntation, diagnostic imaging, localization, operative management, pathology, and postoperative outcome of these patients. Clinic visits, contact with t he tumor registry, and telephone interviews were used for follow-up. From 1 973 through 1999, there were 11 children (four males and seven females) wit h 14 pheochromocytomas. Two (18.2%) patients had bilateral adrenal lesions and one patient had both adrenal and extra-adrenal tumors. Six (54.5%) pati ents had extra-adrenal lesions. The average age at operation was 14.7 years (range 9-18 years). Nine (82%) patients had significant hypertension at pr esentation. CT was used to localize the tumor in eight patients and urine c atecholamine levels were used to confirm the diagnosis. Two of the cases we re associated with inherited syndromes (multiple endocrine neoplasia 2A and von Hippel-Lindau). Ten patients underwent an open operation and one patie nt had a laparoscopic resection. The average patient follow-up was 9.2 year s (range 9 months to 25 years). There were no operative complications and a ll patients were alive and well at the time of last follow-up. Three patien ts (27.2%) had tumors with microscopic malignant features. No tumors recurr ed or had evidence for metastatic spread. We conclude that peak incidence o f pheochromocytomas in children is in early adolescence, Resection can be c arried out safely with minimal morbidity and mortality. Current best manage ment of this entity includes establishment of a biochemical diagnosis, adeq uate preoperative blockade, appropriate imaging, and an individualized oper ative approach based on tumor location and size.