Malignant pheochromocytoma with ganglioneuroblastomatous elements in a patient with von recklinghausen's disease

A 14-year-old girl with numerous café-au-lait spots in her skin was hospitalized because of fever, weight loss, and a mass of the right upper quadrant of the abdomen. Despite intensive chemotherapy, she died 6 months after admission. The autopsy revealed a right adrenal tumor with metastases to liver, lungs, vertebrae, and lymph nodes. Histologically the tumor was a pheochromocytoma with small foci of ganglioneuroblastoma. The catecholamine contents of the tumor were markedly elevated, as confirmed by the catecholamine fluorescence technique. Electron microscopically, the tumor cells contained intracytoplasmic membrane-bound chromaffin granules of varying sizes and shapes. This may be the first report of the concomitant occurrence of malignant catecholamine-secreting pheochromocytoma with ganglioneuroblastoma elements in a patient with von Recklinghausen's disease.