Choyke P L, Filling-Katz M R, Shawker T H, Gorin M B, Travis W D, Chang R, Seizinger B R, Dwyer A J, Linehan W M
Diagnostic Radiology Department, Warren G. Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892.
Radiology. 1990 Mar;174(3 Pt 1):815-20. doi: 10.1148/radiology.174.3.2305064.
The visceral manifestations of von Hippel-Lindau (VHL) disease can cause significant morbidity and mortality. The authors prospectively screened 37 persons from a single kindred. Twenty-five subjects underwent abdominal ultrasound (US), contrast material-enhanced abdominal computed tomography (CT), and nonenhanced abdominal magnetic resonance (MR) imaging. Eight subjects younger than 16 years of age underwent abdominal US and MR imaging only. Scrotal US was employed in 25 male patients. Eleven subjects had renal cysts or tumors. Contrast-enhanced CT depicted renal abnormalities in 10 of these subjects, US in seven, and MR imaging in nine. Among 12 subjects with pancreatic cysts or tumors, CT showed pancreatic abnormalities in all 12, US in nine, and MR imaging in nine. Three subjects (mean age, 34.5 years) had renal tumors, and three had pancreatic masses. Scrotal US revealed epididymal cystadenomas in seven subjects; two of these tumors were surgically verified. A combination of contrast-enhanced CT and scrotal US in male patients appears to be the best way to screen for visceral manifestations of VHL disease.
冯·希佩尔-林道（VHL）病的内脏表现可导致严重的发病和死亡。作者对来自一个家族的37人进行了前瞻性筛查。25名受试者接受了腹部超声（US）、对比剂增强腹部计算机断层扫描（CT）和非增强腹部磁共振（MR）成像。8名16岁以下的受试者仅接受了腹部超声和磁共振成像。25名男性患者接受了阴囊超声检查。11名受试者有肾囊肿或肿瘤。对比增强CT显示其中10名受试者有肾脏异常，超声显示7名，磁共振成像显示9名。在12名有胰腺囊肿或肿瘤的受试者中，CT显示所有12名受试者有胰腺异常，超声显示9名，磁共振成像显示9名。3名受试者（平均年龄34.5岁）有肾肿瘤，3名有胰腺肿块。阴囊超声显示7名受试者有附睾囊腺瘤；其中2个肿瘤经手术证实。男性患者联合使用对比增强CT和阴囊超声似乎是筛查VHL病内脏表现的最佳方法。
