Ⅰ型Von Hippel-Lindau综合征的影像学特点  

Imaging features of Von Hippel-Lindau syndrome type Ⅰ

作　　者：颜立群[1] 侯亚平[2] 王文燕[1] 闫乐卡[1] 汪国石[1] 黄渤源[1] 韩娟[1] 康建丽[1] 耿左军[1] 

机构地区：[1]河北医科大学第二医院医学影像科,河北石家庄050000 [2]河北省血液中心机采科

出　　处：《实用放射学杂志》2015年第3期353-356,共4页Journal of Practical Radiology

摘　　要：目的：分析总结Ⅰ型 Von Hippel-Lindau（VHL）综合征患者的影像学特点。方法收集本院经临床确诊的 VHL 病例12例,患者均行3.0T磁共振及64排螺旋CT平扫及增强检查,影像资料由2位资深影像学医生分析并归纳总结其影像学表现。结果本组病例中患者均可见小脑、脊髓或者脑干的多发血管母细胞瘤,病变内可见不同程度的血管流空征象,6例患者病变周围可见异常扩张的供血及引流血管,全部病例均可见肾脏、胰腺多发囊肿,4例患者伴发脊髓空洞,7例患者发现眼部视网膜血管瘤,5例患者合并肾细胞癌,2例患者肺内出现磨玻璃样及微结节样病变,患者有痰中带血的症状。结论Ⅰ型 VHL具有复杂的影像学特点,影像学检查对早期发现 VHL病变具有重要意义。Objective To analyze the imaging characteristics of Von Hippel-Lindau Syndrome （VHL）typeⅠ.Methods 12 patients diag-nosed as VHL type Ⅰ were collected and all the patients had underwent 3.0T MRI and 64-slice spiral CT plain and enhanced scan. Imaging data was analyzed by two senior radiologists to summarize its imaging featueres.Results Multiple hemangioblastomas （HGBLs）in cerebellum,spinal cord or brainstem were seen in all the patients.Flow void effect with varied degree was seen in the lesions.Dilated feeding and drainage blood vessels around lesions was seen in 6 cases.Multiple kidney and pancreas cysts were seen in all the patients.There were 4 cases with syringomyelia,7 cases with ocular retinal angioma,5 cases with renal cell carcinoma,2 cases with ground-glass-like lesions and micronodulars in lung.Conclusion VHL type Ⅰ has complex imaging performance.Ima-ging examination is important for early detection of VHL disease.

关 键 词：VHL综合征 血管母细胞瘤 肾细胞癌 磁共振成像 计算机体层成像 

分 类 号：R739.4[医药卫生—肿瘤] R445.2[医药卫生—临床医学]