Familial occurrence of hemangioblastoma of central nervous system.
OBJECTIVE
To study the familial occurrence of hemangioblastoma of the central nervous system and to search for an effective way to diagnose and manage the disease.


METHODS
From 1974 to 1993, six patients with hemangioblastoma with family history underwent surgical treatment at our hospital. Computerized tomography (CT) or magnetic resonance imaging (MRI) technique was used for the diagnoses before operation.


RESULTS
Each patient had more than two relatives with hemangioblastoma. Multiple lesions were found in two of the six familial hemangioblastoma cases. All the patients recovered well after operation. So far, no victims have been discovered in the third generation.


CONCLUSIONS
The high occurrence rate of familial hemangioblastoma cases suggests an involvement of hereditary factors. We need a long follow-up study on the descendants of familial hemangioblastoma patients. MRI is of diagnostic value in determining the location and nature of hemangioblastoma in both brain and spinal cord. A thorough examination is necessary for hemangioblastoma patients, and microsurgery is important in removing the tumor.
Zhou
F
F
Department of Neurosurgery, Huashan Hospital, Shanghai Medical University, China.
Zhang
R
R
Ji
Y
Y
Xu
Q
Q
Zhou
L
L
Shi
Y
Y
D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D002528:Cerebellar Neoplasms / Q000235:genetics*; D005192:Family Health; D005260:Female; D018325:Hemangioblastoma / Q000235:genetics*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000235:genetics; D010375:Pedigree; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery