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冯·希佩尔-林道病的影像学表现。

The radiographic manifestations of von Hippel-Lindau disease.

作者信息

Fill W L, Lamiell J M, Polk N O

出版信息

Radiology. 1979 Nov;133(2):289-95. doi: 10.1148/133.2.289.

Abstract

The radiographic findings and method of investigation are presented in the retrospective and prospective study of 221 descendents of an individual affected with von Hippel-Lindau disease. Among 42 affected individuals, most of the reported manifestations were found, such as cerebellar hemangioblastoma, retinal angioma, and renal cell carcinoma. A new manifestation, pancreatic carcinoma, was also seen. Although pheochromocytoma is common in some reported families, no cases were found in this group. An organized multidisciplinary approach is necessary to effectively identify and treat individuals affected with this disease.

摘要

对一名患冯·希佩尔-林道病个体的221名后代进行了回顾性和前瞻性研究,本文展示了其影像学检查结果及研究方法。在42名患病个体中,发现了大多数已报道的表现,如小脑成血管细胞瘤、视网膜血管瘤和肾细胞癌。还发现了一种新的表现,即胰腺癌。尽管在一些已报道的家族中嗜铬细胞瘤很常见,但该组中未发现病例。需要采取有组织的多学科方法来有效识别和治疗受此病影响的个体。

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