Chambers T P, Fishman E K, Hruban R H
Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Clin Imaging. 1997 Jan-Feb;21(1):40-2. doi: 10.1016/0899-7071(95)00066-6.
von Hippel-Lindau (VHL) disease is an unusual inherited chromosomal dominant disease characterized by CNS hemangioblastomas and visceral neoplasms. Multiple abnormalities may involve the abdomen including pathology in the liver, pancreas, kidneys, and adrenal glands. The present case report describes a patient with renal cell carcinoma in von Hippel-Lindau disease. What was most unusual about this case was that the patient also developed pancreatic masses that were metastatic disease from renal cell carcinoma.
冯·希佩尔-林道（VHL）病是一种罕见的遗传性染色体显性疾病，其特征为中枢神经系统血管母细胞瘤和内脏肿瘤。多种异常情况可能累及腹部，包括肝脏、胰腺、肾脏和肾上腺的病变。本病例报告描述了一名患有冯·希佩尔-林道病的肾细胞癌患者。该病例最不寻常之处在于，患者还出现了胰腺肿块，这些肿块是肾细胞癌的转移病灶。
