[von Hippel-Lindau disease--hemangioblastoma associated with pheochromocytoma].
The association of a pheochromocytoma with von Hippel-Lindau disease is uncommon. We had a family with eight patients affected by von Hippel-Lindau disease, of whom five had hemangioblastoma of the central nervous system and seven had pheochromocytoma. As other lesions, retinal angiomatosis, spinal A-V malformation and spinal hemangioma were included in this family. In this paper five hemangioblastomas, four of whom had the association of hemangioblastoma and pheochromocytoma, are presented and clinical features of hemangioblastoma associated with pheochromocytoma are discussed, comparing to the 16 reported cases that had the association of hemangioblastoma and pheochromocytoma. The mean age of our own five cases at the onset of clinical symptoms was 32.2 years (ranging from 24 to 41 years) and that of reported 16 cases was 33.9 years (ranging from 18 to 55 years). These ages are slightly younger than that of sporadic hemangioblastoma. On the other hand, the mean age at the onset of pheochromocytoma was 28.3 years in our cases and 31.0 years in reported cases. This may suggest that hemangioblastoma when it is associated with pheochromocytoma presents its symptoms several years after the signs and symptoms of pheochromocytoma are manifested. In our cases a male to female ratio was 4:1 and in reported ones it was 9:7, showing that hemangioblastoma associated with pheochromocytoma as well as sporadic hemangioblastoma is likely to occur more in male.(ABSTRACT TRUNCATED AT 250 WORDS)
D000310:Adrenal Gland Neoplasms / Q000235:genetics*; D000328:Adult; D000798:Angiomatosis / Q000235:genetics*; D001932:Brain Neoplasms / Q000235:genetics*; D002551:Cerebral Ventricle Neoplasms / Q000235:genetics; D006394:Hemangiosarcoma / Q000235:genetics*; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000150:complications; D008297:Male; D008526:Medulla Oblongata; D009378:Neoplasms, Multiple Primary / Q000235:genetics*; D010375:Pedigree; D010673:Pheochromocytoma / Q000235:genetics*; D006623:von Hippel-Lindau Disease / Q000235:genetics*