Von Hippel-Lindau(VHL) syndrome is a rare autosomal dominant hereditary disease, and pancreas is one of the frequently involved intra-abdominal organs, including simple pancreatic cysts, pancreatic serous cystadenomas and neuroendocrine neoplasmas. Most of the VHL-related pancreatic neuroendocrine neoplasmas (VHL-pNEN)were non-functional, but they still have a tendency to be malignant. Treatment options for VHL-pNEN include regular follow-up, surgical resection, and medication therapy. When compared with sporadic pNEN, the malignant degree of VHL-pNEN is lower, with a better prognosis, so the surgical treatment should be carefully considered. The indications of surgery for VHL-pNEN include big primary lesions (≥3 cm), fast tumor doubling time (<500 days), VHL gene mutation on exon 3, malignant manifestations on imaging findings, and functional pNEN lesions. The function-preserving approach should be performed to keep the functional pancreatic parenchyma as much as possible. Even for patients with a late stage malignancy that cannot be radically resected, active medication therapy may still lead to a long-term survival.
Von Hippel-Lindau(VHL)综合征是一种罕见的常染色体显性遗传性疾病，胰腺是易被累及的腹部器官之一，包括胰腺单纯性囊肿、浆液性囊腺瘤及神经内分泌肿瘤等。VHL综合征相关胰腺神经内分泌肿瘤(VHL-pNEN)多无功能，但有恶变倾向，治疗措施包括定期随访、手术治疗及药物治疗等。VHL-pNEN的恶性程度较散发型pNEN低，预后亦较好，应慎重选择手术治疗。手术指征包括肿瘤最大径≥3 cm、肿瘤倍增时间<500 d、VHL基因发生3号外显子突变、影像学检查疑有恶性表现、功能性pNEN病灶等。手术方式应尽可能保留有功能的胰腺实质。即使无法根治切除的恶性pNEN患者，积极的内科治疗仍可能使患者获得长期生存。.
Keywords: Diagnosis; Neuroendocrine tumors; Pancreatic neoplasms; Treatment; Von Hippel-Lindau syndrome.