Pediatric malignant pheochromocytoma

Abstract

Pediatric malignant pheochromocytomas are very rare tumors, and no institution has more than one or two of these problem cases. The authors report on two children with such tumors, over a 9-year period, from two hospitals. In 1984, D.B. (14 years of age) presented with symptoms and signs of extradural metastasis from a right adrenal primary; he also had lung and bone metastases. After spinal decompression almost 4 years. He has remained well and is in remission 6 years later. In 1987, G.R. (13 years of age) presented with a larger right adrenal malignant pheochromocytoma invading surrounding structures; he also had liver metastasis. Preoperative chemotherapy did not shrink the tumor much; it was grossly resected, and there were many postoperative problems. In 1990, bone metastases developed, for which radiotherapy and chemotherapy were used. Three years later, the metastases have not disappeared; he remains on chemotherapy, and his liver function is borderline. From our small experience as well as a literature review, it appears that surgical excision remains the treatment of choice for the pediatric malignant phoechromocytoma. Unresectable tumors may be rendered resectable by intensive chemotherapy (similar to that used for neuroblastoma); adjuvant chemotherapy should be used for residual disease after surgery and for metastatic disease.