Prognosis of children with malignant pheochromocytoma - Report of 2 cases and review of the literature

Malignant pheochromocytomas are rare in childhood and the prognosis of chil dren with this tumor is not well known. We present 2 pediatric observations of malignant pelvic pheochromocytoma. Symptoms in both cases were headache and hypertension. The tumor invaded the sacral bone, Angiogram helped to l ocalize the tumor and metastases, and allowed preoperative embolization of the tumor in 1 case. The first child underwent incomplete surgical resectio n, I-131-MIBG therapy and radiotherapy, and is still alive 2 years after di agnosis. The second child died from metastatic invasion a few weeks after d iscovery of the tumor. We reviewed previous reports of children with malign ant pheochromocytomas (30 cases). Primary tumor was extraadrenal in 50% of cases. The 3-year survival rate was 73 +/- 9% (mean +/- SD). Apart from sur gical resection, no particular treatment appeared to be more effective than others in reducing mortality.