Yamasaki Ichiro, Nishimori Isao, Ashida Shingo, Kohsaki Takuhiro, Onishi Saburo, Shuin Taro
Department of Urology, Kochi Medical School, Kochi, Japan.
Pancreas. 2006 Nov;33(4):382-5. doi: 10.1097/01.mpa.0000240604.26312.e4.
The aim of this study was to elucidate the clinical characteristics of pancreatic neuroendocrine tumors (NET) in Japanese patients with von Hippel-Lindau (VHL) disease.
We sent a questionnaire to all members of the Japan Pancreas Society in 2002 asking for the number of patients with VHL and complications of NET and/or cystic lesion in the pancreas. Furthermore, we sent a second questionnaire to obtain detailed information about the clinical characteristics of pancreatic NET.
A total of 58 patients with VHL were reported. Among these, 34 (59%) patients had pancreatic lesions, including 10 with pancreatic NET, 23 with a cystic lesion, and one with both. The mean age at identification of pancreatic NET was 34.6 years (range, 22-64 years). The mean diameter of the tumors was 4.3 cm (range, 1-12.5 cm). Distant metastases were found in 2 (20%) cases. During the follow-up period (3.3 years; range, 0-8 years), 7 patients are alive, and 2 patients died of hemangioblastomas.
Pancreatic NET in the VHL disease showed a relatively lower incidence of metastasis compared with sporadic non-functioning pancreatic NET, yielding a favorable prognosis. Because they present no hormonal syndrome, periodic screening examinations are warranted to identify pancreatic NET at an early stage.
本研究旨在阐明日本冯·希佩尔-林道（VHL）病患者胰腺神经内分泌肿瘤（NET）的临床特征。
2002年，我们向日本胰腺学会的所有成员发送了一份调查问卷，询问VHL病患者的数量以及胰腺NET和/或囊性病变的并发症情况。此外，我们又发送了一份调查问卷以获取胰腺NET临床特征的详细信息。
共报告了58例VHL病患者。其中，34例（59%）患者有胰腺病变，包括10例胰腺NET、23例囊性病变患者以及1例同时患有这两种病变的患者。确诊胰腺NET时的平均年龄为34.6岁（范围22 - 64岁）。肿瘤的平均直径为4.3厘米（范围1 - 12.5厘米）。2例（20%）患者出现远处转移。在随访期（3.3年；范围0 - 8年）内，7例患者存活，2例患者死于血管母细胞瘤。
与散发性无功能性胰腺NET相比，VHL病中的胰腺NET转移发生率相对较低，预后良好。由于它们不表现出激素综合征，因此有必要进行定期筛查以早期发现胰腺NET。
