| 刘影,何文,张红霞,王立淑,张强,邬冬芳.von Hippel-Lindau综合征腹部超声及中枢神经系统MRI特征[J].中国医学影像技术,2016,32(4):560~564 |
| von Hippel-Lindau综合征腹部超声及中枢神经系统MRI特征 |
| Abdominal ultrasound and central nervous system MRI characteristics of von Hippel-Lindau syndrome |
| 投稿时间：2015-08-13 修订日期：2016-01-26 |
| DOI：10.13929/j.1003-3289.2016.04.021 |
| 中文关键词: 超声检查 磁共振成像 VHL综合征 中枢神经系统 |
| 英文关键词:Ultrasonography Magnetic resonance imaging von Hippel-Lindau syndrome Central nervous system |
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| 中文摘要: |
| 目的 观察伴有中枢神经系统(CNS)血管母细胞瘤的von Hippel-Lindau(VHL)综合征患者的腹部超声及CNS的MRI特征.方法 回顾性分析26例经手术病理证实伴有CNS血管母细胞瘤的VHL综合征患者的腹部超声及MRI资料.结果 对26例VHL患者行腹部超声检查,发现胰腺病变23例,占88.46%,其中19例为胰腺多发囊肿,2例胰腺单发囊肿,1例胰腺多发囊肿合并胰腺囊腺瘤,1例胰腺多发实性结节;肾脏病变8例,占30.77%,其中2例肾透明细胞癌,1例双肾多发囊肿,2例双肾多发囊肿伴一侧肾透明性细胞癌,3例双肾多发囊肿伴双肾错构瘤;发现肾上腺病变1例,占3.85%;肝血管瘤3例,占11.54%.26例VHL患者MR检查均发现CNS血管母细胞瘤,14例肿瘤表现为囊性+囊"壁"结节,11例为实性,1例两种表现形式同时存在.结论 VHL综合征的腹部超声表现具有一定的特征性.腹部超声联合CNS的MR检查对诊断及随访VHL综合征具有重要价值. |
| 英文摘要: |
| Objective To observe the abdominal ultrasound and central nervous system (CNS) MRI characteristics of von Hippel-Lindau (VHL) syndrome. Methods The images of abdominal ultrasound and MRI of CNS in 26 VHL patients with CNS hemangioblastoma were reviewed. Results A total of 26 cases with VHL disease underwent abdominal ultrasonud examination, the pancreatic lesion was found in 23 cases, accounting for 88.46%, 19 cases with pancreatic multiple cysts, 2 cases with pancreatic single cyst, 1 case with pancreatic multiple cysts and cystadenoma, 1 case with pancreatic multiple solid nodules. Renal lesion was found in 8 cases, accounting for 30.77%, 2 cases with renal cell carcinoma, 1 case with bilateral renal multiple cysts, 2 cases with bilateral renal multiple cysts and unilateral renal clear cell carcinoma, 3 cases with bilateral renal multiple cysts and bilateral renal hamartoma. Adrenal lesion was found in 1 case, accounting for 3.85%. Hepatic hemangioma was found in 3 cases, accounting for 11.54%. CNS hemangioblastoma was found in 26 cases by MRI, the imaging showed cyst and "wall" nodule in 14 cases, solid mass in 11 cases, two forms coexisted in 1 case. Conclusion The abdominal ultrasonogram of VHL syndrome have some characteristics. Abdominal ultrasound combined with MR examination of CNS will play great significance on diagnosis and follow-up for VHL syndrome. |
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