Malignant pheochromocytoma with ganglioneuroblastoma 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.
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000556:secondary; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002395:Catecholamines / Q000378:metabolism; D005260:Female; D006258:Head and Neck Neoplasms / Q000473:pathology*; D006651:Histocytochemistry; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000556:secondary; D008175:Lung Neoplasms / Q000378:metabolism / Q000556:secondary; D008854:Microscopy, Electron; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009447:Neuroblastoma / Q000188:drug therapy / Q000378:metabolism / Q000556:secondary; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010673:Pheochromocytoma / Q000378:metabolism / Q000473:pathology* / Q000556:secondary
D002395:Catecholamines