家族性Von Hipple-Lindau病的影像学诊断(附2例报告)

IMAGING DIAGNOSIS OF FAMILIAL VON HIPPLE-LINDAU(WITH A REPORT OF 2 CASES)

目的评价家族性Von Hipple-Lindau病的影像学特点,增加对本病的认识。方法回顾性分析家族中两例患者的临床及影像学资料,并复习相关文献。结果一个家系两例患者,分别是兄弟,年龄45～49岁,两兄弟都患有小脑血管母细胞瘤,一例有肾脏囊肿,另一例患者合并胰腺囊肿、肾囊肿和肾细胞癌等。结论影像学检查在Von Hipple-Lindau病的诊断、随访以及长期检测中起着极其重要的作用。

Objective To remark the imaging characteristics of familial Von Hipple-Lindau and to in- crease the understanding of the disease. Methods Retrospective analysis of the clinical and imaging charac- teristics in the two patients with familial Von Hipple-Lindau and the review of related literature were con- ducted. Results The two patients with were brothers, aged 45 and 49, suffering from Von Hippel-Lindan syndromes. One case suffered from renal cyst, another case suffered from pancreatic cyst, cyst of kidney and renal cell carcinoma. Conclusion Imaging examination plays an important role in the diagnosis of Von Hipple-Lindau, the follow-up surveys and the long-term detection.