PHEOCHROMOCYTOMA AND PARAGANGLIOMA IN CHILDREN - A REPORT OF 24 CASESOF THE FRENCH-SOCIETY-OF-PEDIATRIC-ONCOLOGY

Pheochromocytoma and paraganglioma of childhood are rare neuroendocrin e tumors. Urinary catecholamine measurements, metaiobenzylguanidine (M IBG) scanning, computed tomographic scanning, and magnetic resonance i maging have greatly facilitated diagnosis. Prognosis after surgical re section is excellent. In this retrospective series collected from Fren ch oncology centers, the risk of tumor progression was studied in orde r to assess prognostic factors and the optimal diagnostic and therapeu tic management. Medical records of 24 children with paraganglioma were reviewed This tumor occurred at a median age of 12.5 years and in mos t cases was revealed by arterial hypertension. The diagnosis was made by the demonstration of urinary excretion of catecholamines and their metabolites. Six patients had bilateral adrenal pheochomocytomas; two patients had extra-adrenal paragangliomas. In eight patients, the para ganglioma occurred as a familial disease. Surgical excision war the on ly therapeutic procedure With a follow-up of 5.2 years, 14 of the pati ents are still in first complete remission and 6 have developed metast ases or shown tumor progression. Despite a high long-term survival rat e, the risk of malignancy and of multifocal involvement is of concern and is associated with a significant rate of late events. The outcome depends on adequacy of tumor resection and must be serially assessed.