1 型多发性内分泌肿瘤综合征中的胆道树胃泌素瘤。
Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome.
机构信息
Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy.
出版信息
World J Gastroenterol. 2013 Dec 7;19(45):8312-20. doi: 10.3748/wjg.v19.i45.8312.
AIM
To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.
METHODS
Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree.
RESULTS
Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES.
CONCLUSION
These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.
目的
描述患有异位胆管树胃泌素瘤的患者，并复习该主题的文献。
方法
1992 年 1 月至 2012 年 6 月期间，在我院接受治疗的多发性内分泌肿瘤 1 型（MEN1）综合征合并胰十二指肠内分泌肿瘤的 28 例患者中，17 例患有 Zollinger-Ellison 综合征（ZES）的患者接受了胰十二指肠手术。本文回顾性分析了这 17 例患者的临床资料，这些患者接受了胰十二指肠切除术（DP）或全胰切除术（TP）治疗 ZES。手术治疗包括胰十二指肠切除术（DP）或全胰切除术（TP）。区域淋巴结清扫术始终是常规进行的。术中发现任何肝肿瘤性病变均予以切除。在 MEN1 患者中，十二指肠病变的切除有时可导致高胃泌素血症的持续或复发。这种不良结果的一个可能解释可能是未识别的胃泌素分泌肿瘤的异位定位。本研究在 17 例患者中描述了 3 例患者存在位于胆管树中的异位胃泌素瘤。
结果
对 17 例患有 ZES 的 MEN1 患者进行了分析。患者的平均年龄为 40 岁。15 例行 DP，2 例行 TP。17 例患者的病理检查均发现胰十二指肠内分泌肿瘤。在十二指肠的前三部分共发现 81 个胃泌素瘤。仅在胰腺中发现 1 个胃泌素瘤。每位患者的胃泌素瘤平均数量为 5 个（范围 1-16 个）。发现淋巴结、肝和网膜转移后，12 例（70.5%）患者被诊断为恶性肿瘤。3 例患者除了十二指肠胃泌素瘤外，还存在胆管树胃泌素瘤。在 2 例中，异位胃泌素瘤在胰腺手术时同时切除，而在第 3 例中，由于 ZES 复发，在 DP 一年后切除了胆管树胃泌素瘤。
结论
这些发现表明，在 MEN1 患者行 ZES 手术时，检查胆管树中是否存在异位胃泌素瘤非常重要。
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