摘要
目的探讨中枢神经系统（ＣＮＳ）血管网织细胞瘤作为希林病（ｖｏｎｈｉｐｐｅｌｌｉｎｄａｕｄｉｓｅａｓｅ，ＨＬＤ）的临床特点。方法回顾性研究了我院１９８２～１９９５年收治的１７４例ＣＮＳ血管网织细胞瘤，所有病例均经手术病理证实。结果符合ＨＬＤ者１０例，其中４例有明确家族史，５例患有视网膜血管瘤，２例病人患肾囊肿，１例病人有肾肿瘤。另外，可疑者５例。结论血管网织细胞瘤可单独发生在中枢神经系统，也可做为ＨＬＤ的一种表现，与非ＨＬＤ者相比，肿瘤的多发性是其主要特点。
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. Conclusion 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.
出处
《中华医学杂志》
CAS
CSCD
北大核心
1998年第6期460-461,共2页
National Medical Journal of China
