小脳血管芽腫（Hemangioblastoma）家族発生の1家系

Familial Hemangioblastoma of the Cerebellum:Report of three cases in a pedigree

I．はじめに

　頭蓋内に発生する血管芽腫（hemangioblastoma）^2）の領度は比較的低く，全脳腫瘍の1-2％^19），後頭蓋窩腫瘍の7-10％^13,17,19）とされる．本腫瘍には，文献報告例からも明らかなように，注目すべきいくつかの生物学的特徴があるが，その1つに家族発生がある，6,18-21）．今回，われわれは小脳血管芽腫の家族発生（1家系3症例）を経験したので，本疾患における家族発生例の特徴などを中心に若干の文献的考察を加え報告する．

Familial hemangioblastoma of the cerebellum isreported here. The cerebellar hemangioblastomahas many interesting characteristics, for example,heredity, erythrocytosis, angioma of the retina andcystic tumor in the other organs. Several pedigreesof hemangioblastoma have been reported in Japan.Recently, the authors experienced one pedigree ofhemangioblastomas and the cases will be reported andalso it will be considered bibliographically.

Case 1 was 27-year-old female complaining ofheadache, vomiting and unsteadness. Her niece diedof hemangioblastoma (case 3). Neurologically, theright cerebellar signs were observed.