[Hemangioblastomas of the central nervous system with von Hippel-Lindau's disease].
OBJECTIVE
To study the clinical features of hemangioblastomas of the central nervous system (CNS) when they occucr as a manifestation of von Hippel-Lindau's disease (HLD).


METHODS
174 patients with hemanioblastomas of CNS were reviewed from 1982 to 1995. All patients were verified pathologically.


RESULTS
Ten of the patients definitely had HLD. Four patients reported a positive family history. Other 5 patients had retinal angiomas. The following lesions were detected: renal cysts(2 patients), renal tumor (1). Five patients were suspicous.


CONCLUSIONS
Hemangio-blastoma may arise in isolation or as a manifestation of von Hippel-Lindau's disease, and is often multiple in the latter. Enhanced MRI can decrease chances of negative diagnosis for tumor nodules. Microsurgery may be helpful to discover unclear lesions. Patients, especially those with HLD, should be subjected to follow-up and regular MRI examinations for a long time.
Department of Neurosurgery, Beijing Tiantan Hospital.
Z
Z
Ma
J
J
Xie
Y
Y
Zhao
D000293:Adolescent; D000328:Adult; D000368:Aged; D016543:Central Nervous System Neoplasms / Q000150:complications / Q000175:diagnosis*; D002528:Cerebellar Neoplasms / Q000150:complications / Q000175:diagnosis; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018325:Hemangioblastoma / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D006623:von Hippel-Lindau Disease / Q000150:complications*