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一例同时患有胃胃肠道间质瘤、胰腺神经内分泌肿瘤和子宫平滑肌瘤的罕见病例。

A rare case with synchronous gastric gastrointestinal stromal tumor, pancreatic neuroendocrine tumor, and uterine leiomyoma.

作者信息

Arabadzhieva Elena, Yonkov Atanas, Bonev Sasho, Bulanov Dimitar, Taneva Ivanka, Vlahova Alexandrina, Dikov Tihomir, Dimitrova Violeta

机构信息

Department of General and Hepato-pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, 1 Georgi Sofiiski Str, 1431, Sofia, Bulgaria.

Medical University-Sofia, 15 Acad. I. E. Geshov Bul, 1431, Sofia, Bulgaria.

出版信息

World J Surg Oncol. 2016 Nov 15;14(1):287. doi: 10.1186/s12957-016-1051-x.

Abstract

BACKGROUND

Although gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, they comprise less than 1% of all gastrointestinal tumors. Neuroendocrine tumors (NET) of the gastro-enteropancreatic system are also rare, representing about 2% of all gastrointestinal neoplasms. Pancreatic localization of NET is extremely uncommon-these tumors are only 1-5% of all pancreatic cancers.

CASE PRESENTATION

We describe an unusual case with triple tumor localization-a gastric tumor, a formation in the pancreas, which involves the retroperitoneal space, and a uterine leiomyoma. The exact diagnosis was confirmed with immunohistochemical study after surgical treatment of the patient. Distal pancreatic resection, splenectomy, partial gastrectomy, omentectomy, and hysterectomy were performed. The histological examination proved an epithelioid type of gastric GIST. Immunostaining showed focal positive expression of c-kit and no mitotic figures per 50 HPF. Histology of the pancreatic and retroperitoneal formation proved a well-differentiated NET with origin from the islets of Langerhans. The immunohistochemical study demonstrated co-expression of chromogranin A and synaptophysin.

CONCLUSIONS

This is the fourth case published so far of a patient with synchronous pancreatic NET and gastric GIST. The main objective of the study is to present a unique case because we have not found any reports for coexistence of the described three types of neoplasm, as in our patient, and we hope that it will be valuable in the future investigations about the genesis, diagnosis, and treatment of these types of tumors.

摘要

背景

尽管胃肠道间质瘤(GISTs)是胃肠道最常见的间叶组织肿瘤,但它们在所有胃肠道肿瘤中所占比例不到1%。胃肠胰系统的神经内分泌肿瘤(NET)也很罕见,约占所有胃肠道肿瘤的2%。NET的胰腺定位极为罕见——这些肿瘤仅占所有胰腺癌的1% - 5%。

病例报告

我们描述了一例罕见的三重肿瘤定位病例——一个胃肿瘤、一个累及腹膜后间隙的胰腺肿物以及一个子宫平滑肌瘤。在对患者进行手术治疗后,通过免疫组化研究确诊了确切诊断。实施了远端胰腺切除术、脾切除术、部分胃切除术、大网膜切除术和子宫切除术。组织学检查证实胃GIST为上皮样型。免疫染色显示c-kit局灶性阳性表达,每50个高倍视野无有丝分裂象。胰腺及腹膜后肿物的组织学检查证实为起源于胰岛的高分化NET。免疫组化研究显示嗜铬粒蛋白A和突触素共表达。

结论

这是迄今为止发表的第四例同时患有胰腺NET和胃GIST的患者。本研究的主要目的是呈现一个独特病例,因为我们尚未发现有任何关于像我们患者这样三种所述肿瘤同时存在的报道,并且我们希望这对未来关于这些类型肿瘤的发生、诊断和治疗的研究有价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a2d/5111233/98c49158aed1/12957_2016_1051_Fig1_HTML.jpg

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