Department of Nephrology and General Medicine, University Medical Center, Albert Ludwigs University, Hugstetter Strasse 55, 79106 Freiburg, Germany.
World J Surg. 2011 Mar;35(3):563-7. doi: 10.1007/s00268-010-0878-5.
Pancreatic neuroendocrine tumors (PNETs) are a characteristic feature of the tumor syndromes multiple endocrine neoplasia type 1 (MEN-1) and von Hippel-Lindau disease (VHL). With VHL, about 10% of the patients exhibit PNETs by age 40 years. Metastatic potential is high if the tumors have grown to >3 cm in diameter. Optimal surgical treatment is still a challenge.
We report three cases, all women, ages 22, 30, and 39 years, respectively, who had known VHL, confirmed by classic organ manifestations and germline mutations of the VHL gene. All were diagnosed, in an asymptomatic stage, with solid tumors of the pancreatic tail or tail/corpus area measuring 2.9-5.6 cm diameter. All accepted the offer of laparoscopic organ-sparing removal of the tumors.
In all three cases, the tumor was entirely removed. In two cases, resection of the spleen was also necessary as dissection of the tumor from the major splenic vessels was impossible. Operating time was 215-365 min, and blood loss was 200-700 ml. Histolopathology revealed benign PNETs in two cases, but the third patient had regional lymph node metastases. There were no complications, and the hospital stay was 4-7 days.
Organ-sparing laparoscopic surgery is an important option for treating VHL-associated PNETs of the pancreatic tail.
胰腺神经内分泌肿瘤（PNETs）是多发性内分泌肿瘤 1 型（MEN-1）和 von Hippel-Lindau 病（VHL）等肿瘤综合征的特征。在 VHL 中，约 10%的患者在 40 岁之前出现 PNETs。如果肿瘤直径>3cm，则转移潜能较高。最佳手术治疗仍然是一个挑战。
我们报告了 3 例女性患者，年龄分别为 22 岁、30 岁和 39 岁，均患有已知的 VHL，通过经典的器官表现和 VHL 基因突变证实。所有患者均在无症状阶段被诊断为胰腺尾部或尾部/体部直径为 2.9-5.6cm 的实性肿瘤。所有患者均接受了腹腔镜保留器官切除肿瘤的治疗方案。
在所有 3 例患者中，肿瘤均被完全切除。在 2 例患者中，由于无法从主脾血管上分离肿瘤，因此还需要切除脾脏。手术时间为 215-365 分钟，失血量为 200-700ml。组织病理学显示 2 例为良性 PNETs，但第 3 例患者有局部淋巴结转移。无并发症，住院时间为 4-7 天。
保留器官的腹腔镜手术是治疗 VHL 相关胰腺尾部 PNETs 的重要选择。
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