Jakhere Sandeep G, Yeragi Bhakti, Jain Darshan G
Department of Radiology, B Y L Nair Charitable Hospital and T N Medical College, Dr A R Nair Road, Mumbai Central, Mumbai, Maharashtra, India-400008.
Acta Med Indones. 2012 Oct;44(4):324-6.
In this case, we describe the wide magnetic resonance imaging (MRI) spectrum in a single patient who had multi organ involvement. Von Hippel Lindau Disease is a rare inherited autosomal dominant disorder characterized by development of benign and malignant lesions involving multiple organs. Imaging plays a vital role in the diagnosis and surveillance of VHL as it can differentiate benign from malignant lesions. The median life expectancy of VHL is 49 years and the commonest cause of death in patients with VHL is renal cell carcinoma and neurologic complication from cerebellar hemangioblastomas.
在本病例中,我们描述了一名多器官受累患者广泛的磁共振成像(MRI)表现。冯·希佩尔-林道病是一种罕见的常染色体显性遗传性疾病,其特征是多个器官出现良性和恶性病变。影像学检查在VHL的诊断和监测中起着至关重要的作用,因为它可以区分良性和恶性病变。VHL患者的中位预期寿命为49岁,VHL患者最常见的死亡原因是肾细胞癌和小脑成血管细胞瘤引起的神经系统并发症。