Pheochromocytoma and clear-cell renal carcinoma in a child with von Hippel-Lindau disease: a patient report.
A 6 year-old boy with von Hippel-Lindau (VHL) disease presented with hypertension due to bilateral pheochromocytomas. At age 13 he developed a renal carcinoma and bilateral paragangliomas. His mother had retinal angiomas, bilateral pheochromocytomas and a cerebellar hemangioblastoma. This unusual presentation illustrates the complexity and difficulties associated with the management of VHL disease.
Department of Pediatrics, Hospital Son Dureta, Palma de Mallorca, Spain.
J
J
Ferragut
M
M
Caimari
B
B
Rituerto
B
B
Gómez-Rivas
M
M
Herrera
F
F
Alonso
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000601:surgery; D000328:Adult; D002292:Carcinoma, Renal Cell / Q000150:complications*; D002395:Catecholamines / Q000652:urine; D002648:Child; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007680:Kidney Neoplasms / Q000150:complications*; D008025:Ligases; D008297:Male; D009154:Mutation; D010673:Pheochromocytoma / Q000150:complications* / Q000601:surgery; D011506:Proteins / Q000235:genetics; D025521:Tumor Suppressor Proteins; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis / Q000235:genetics
D002395:Catecholamines; D011506:Proteins; D025521:Tumor Suppressor Proteins; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D008025:Ligases; C497446:VHL protein, human