Kuhlman J K, Fishman E K, Marshall F F, Siegelman S S
Russell H. Morgan Department of Radiology, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Urology. 1987 Nov;30(5):505-8. doi: 10.1016/0090-4295(87)90396-7.
Von Hippel-Lindau disease is a hereditary disorder with complex, multi-organ involvement including retinal, central nervous system, and abdominal manifestations. We report a case of clinically unsuspected von Hippel-Lindau disease identified during computed tomography (CT) evaluation of a renal mass. The CT demonstration of a coexisting pancreatic tumor and renal cell carcinoma suggested the correct diagnosis of von Hippel-Lindau disease, which was subsequently confirmed. This case reemphasizes the value of preoperative assessment of renal tumors by CT. The finding of coexisting renal and pancreatic tumors should stimulate the search for further evidence of von Hippel-Lindau disease.
冯·希佩尔-林道病是一种遗传性疾病,会累及多个器官,情况复杂,包括视网膜、中枢神经系统及腹部表现。我们报告一例在对肾肿块进行计算机断层扫描(CT)评估时意外发现的冯·希佩尔-林道病病例。CT显示同时存在胰腺肿瘤和肾细胞癌,提示正确诊断为冯·希佩尔-林道病,随后得以证实。该病例再次强调了CT对肾肿瘤进行术前评估的价值。发现同时存在肾和胰腺肿瘤应促使医生进一步寻找冯·希佩尔-林道病的其他证据。