摘要:Von Hippel-Lindau(VHL)综合征是较罕见的常染色体显性遗传病,呈现多器官累及与年龄依赖性.VHL综合征胰腺病变类型包括单纯囊肿,浆液性囊腺瘤及神经内分泌肿瘤.胰腺囊性病变恶变率低,无症状则无需处理;神经内分泌肿瘤多数无功能但有恶变倾向,需要谨慎处理,措施包括定期随访,手术治疗及药物治疗.VHL综合征胰腺病变表现多样且缺乏诊治指南,应根据全身情况进行个体化治疗,制定全过程诊疗及随访计划.
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