Familial occurrence of hemangioblastoma
Markowska-Woyciechowska, A.; Wroński, J.; Czapiga, B.; Druszcz, A.; Bronowicz, A.
Neurologia i Neurochirurgia Polska 33(4): 971-980
1999
ISSN/ISBN: 0028-3843 PMID: 10612111 Document Number: 501837
Haemangioblastoma is a benign vascular neoplasm accounting for approximately 1-2% of all intracranial tumours. Patients with haemangioblastoma are aged usually about 40 years, the tumour is situated mostly in the cerebellum, more frequently in males. The tumour is a component of the Hippel-Lindau syndrome with familial-hereditary dominant aetiology, often associated with retinal angiomatosis, cysts of the pancreas and kidneys, renal carcinoma and phaeochromocytoma. A familial haemangioblastoma is reported in father and son. The father had the tumour in cerebellar vermis but the son had multiple tumours in cerebellum and medulla.