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血管网状细胞瘤又称血管母细胞瘤,好发于小脑,伴有视网膜血管瘤,肝、肾、胰腺囊肿或血管瘤者称为von Hippel-Lindau(VHL)病。我们收治1例,具有家族遗传史,报告如下。 患者女,41岁。因行走不稳3个月于1997年10月2日入院。查体:双侧指鼻实验欠准稳,Romberg征阳性。MRI:双侧小脑半球及小脑蚓部共8处占位性病变,直径1.0～1.5cm,增强明显,诊断“脑囊虫病”。腹部B超:多囊胰,多囊肾。入院后行左小脑半球一处病变切除术,术后病理报告为“血管网状细胞瘤”。2000年3月17日,患者又因头痛、双眼视物不清1周入院。眼底检查:双侧视网膜血管瘤。MRI提示小脑蚓部及右侧小脑半球两处原实质性肿瘤发展成带肿瘤结节的囊性病变。入院后行两处囊性肿瘤切除术。
Vascular reticular cell tumors, also known as hemangioblastomas, occur in the cerebellum and are referred to as von Hippel-Lindau (VHL) disease with retinal hemangiomas, liver, kidney, pancreatic cysts or hemangiomas. We admitted to 1 case, with a family history, the report is as follows. Female patient, 41 years old. Three months due to unsteadiness of walking was admitted on October 2, 1997. Physical examination: bilateral nasolabial test is not stable, Romberg sign positive. MRI: Bilateral cerebellar hemispheres and cerebellar vermis in a total of 8 lesions, diameter 1.0 ~ 1.5cm, enhanced significantly, the diagnosis of “cysticercosis.” Abdomen B ultrasound: Polycystic pancreas, polycystic kidney. After admission, a lesion of the left cerebellar hemisphere was performed. The postoperative pathology was reported as “vascular reticular cell tumor.” March 17, 2000, due to headache patients, binocular vision was admitted to hospital for 1 week. Fundus examination: bilateral retinal hemangiomas. MRI prompted cerebellar vermis and the right cerebellar hemisphere two parenchymal tumors developed into cystic lesions with tumor nodules. After admission two cystic tumor resection.