摘要
目的探讨VHL综合征的临床及影像学特点,提高对本病的认识,以免漏诊、误诊。方法回顾性分析3例VHL综合征病例的临床和影像学资料,并复习相关文献报道。结果男2例,女1例,年龄27-49岁,均合并中枢神经系统血管母细胞瘤和肾脏多发肿瘤/囊肿。2例合并胰腺多发囊肿,2例有家族史。3例患者在病程的不同阶段曾被误诊。结论VHL综合征可累及多个系统,临床表现多样。全面的影像学检查是诊断和随访该病的重要手段。综合各个系统的影像学特点和临床表现,有助于疾病的早期确诊。
Objective： To investigate the clinical and imaging features of yon Hippel - Lindau syndrome, and make a further understanding of the disease for avoiding misdiagnosis or missing of the diagnosis. Methods： To retrospectively analyze the clinical and imaging data of 3 cases with yon Hippel - Lindau syndrome and review related literatures. Results： 3 patients （2 men and 1 woman; age range, 27 - 49 years） suffered from central nervous system hemangioblastomas, bilateral kidney neoplasms/multifocal renal cysts, of 3 patients, two was combined with muhifocal pancreatic cysts, two cases with family history. 3 patients were misdiagnosed in different phases. Conclusions： yon HippedLindau syndrome can involve multisystem, and its clinal manifestations are various, imaging examination plays a major role in both the diagnosis and the followup of patients with von Hippel - Lindau syndrome, combination with imaging featttres and clinal manifestations of many systems are helpful in making the early diagnosis of the disorder.
出处
《华西医学》
CAS
2007年第2期273-275,共3页
West China Medical Journal
