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目的:初步探讨VHL病的发病机制、诊断以及治疗。方法:回顾分析既往收治的3例VHL病人并结合文献复习。结果:3例患者中1例有家族史,另2例为多发病变,均未能全切。第1例患者鞍上无症状病变动态观察,第2例患者颈椎HB过小,动态观察,第3例患者一处病变位于颈椎腹侧,无法切除,其余病变全部切除。结论:本病诊断主要依靠MRI,治疗主要依靠手术,因累及脏器多,很难全部切除,易复发,愈后较差。
Objective: To investigate the pathogenesis, diagnosis and treatment of VHL. Methods: Retrospective analysis of 3 cases of previously treated VHL patients and literature review. Results: One of 3 patients had a family history and the other 2 had multiple lesions. The first patient with asymptomatic lesions on the saddle dynamic observation, the second case of patients with cervical spine HB too small, dynamic observation, the third case of a lesion in the cervical ventral, can not be removed, the remaining lesions were removed. Conclusion: The diagnosis of this disease depends mainly on MRI, the treatment depends mainly on surgery, due to the involvement of organs and more difficult to remove all, easy to relapse, worse after.