Familial occurrence of cerebellar hemangioblastomas--analysis of five families.
Characteristic features of familial cases of cerebellar hemangioblastoma were analyzed on the basis of 54 patients, 32 male and 22 female. In this group there were 7 cases from five affected families. The overall recurrence rate was 33.3% among patients who had been followed for more than 4 years after total removal of the tumor. Recurrence was more common in the familial cases (50%). The average age at the onset of symptoms was younger (23.4 years) in familial cases than in nonfamilial cases (36.2 years) and tended to be even younger (18.9 years) in the second generation of familial cases. These findings suggest that the integration of primitive vasoformative tissues is disturbed more severely in familial cases of cerebellar hemangioblastoma.
D000293:Adolescent; D000328:Adult; D002528:Cerebellar Neoplasms / Q000235:genetics*; D002648:Child; D005260:Female; D006394:Hemangiosarcoma / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000235:genetics; D010375:Pedigree; D012189:Retrospective Studies