NEUROBLASTOMA IV-S FOLLOWED BY EXTRAADRENAL PHEOCHROMOCYTOMA 15 YEARSLATER

Common origin of sympathoblasts and pheochromoblasts from the neural c rest cells is generally accepted. Neuroblastoma and pheochromocytoma r esult from an abnormal proliferation of these committed cells. They ar e included in the group of neuroendocrine neoplasms, formerly named Ap udomas. Previous reports of mixed tumours of neuroblastoma and pheochr omocytoma, and ganglioneuroma and pheochromocytoma, support this hypot hesis. A case of extra-adrenal pheochromocytoma in an adolescent who 1 5 years before was successfully treated for a stage IV-S neuroblastoma without evidence of primitive tumour is reported. Two hypothesis are formulated: (1) the second neoplasm may have arisen from the unlocated primitive tumour, probably silent evolution could be due to its matur ation into ganglioneuroma-pheochromocytoma; (2) taken into account a c ommon origin of both neoplasms, the patient may have a genetic predisp osition to develop neuroendocrine tumours. Whatever the mechanism, sec ond neoplasms, as the case reported, late relapses, and late toxic eff ects may justify long follow-up of neuroblastoma survivors. (C) 1995 W iley-Liss, Inc.