Department of Urology, Kochi Medical School, Nankoku, Kochi 783-8505, Japan.
World J Gastroenterol. 2010 Sep 28;16(36):4515-8. doi: 10.3748/wjg.v16.i36.4515.
The pancreatic manifestations seen in patients with von Hippel-Lindau (VHL) disease are subdivided into 2 categories: pancreatic neuroendocrine tumors (NET), and cystic lesions, including simple cyst and serous cystadenoma. The VHL-associated cystic lesions are generally asymptomatic and do not require any treatment, unless they are indistinguishable from other cystic tumor types with malignant potential. Because pancreatic NET in VHL disease are non-functioning and have malignant potential, it is of clinical importance to find and diagnose these as early as possible. It will be recommended that comprehensive surveillance using dynamic computed tomography for abdominal manifestations, including pancreatic NET, should start from the age of 15 years in VHL patients. Unlike sporadic non-functioning NET without VHL disease, in which surgical resection is generally recommended, VHL patients at lower metastatic risk of pancreatic NET should be spared the risks of operative resection.
在患有 von Hippel-Lindau (VHL) 病的患者中，胰腺表现可分为两类：胰腺神经内分泌肿瘤 (NET) 和囊性病变，包括单纯性囊肿和浆液性囊腺瘤。与 VHL 相关的囊性病变通常无症状，不需要任何治疗，除非它们与其他具有恶性潜能的囊性肿瘤类型无法区分。由于 VHL 病中的胰腺 NET 是无功能性的且具有恶性潜能，因此尽早发现和诊断这些肿瘤具有重要的临床意义。建议从 15 岁开始，对 VHL 患者进行包括胰腺 NET 在内的腹部表现的综合监测。与没有 VHL 病的散发性无功能性 NET 不同，后者一般建议手术切除，而胰腺 NET 转移风险较低的 VHL 患者应避免手术切除的风险。
