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十二指肠穿孔作为胃神经内分泌肿瘤的表现:一例报告

Duodenal perforation as presentation of gastric neuroendocrine tumour: A case report.

作者信息

Di Buono Giuseppe, Bonventre Giulia, Badalamenti Giuseppe, Buscemi Salvatore, Romano Giorgio, Agrusa Antonino

机构信息

Department of Surgical, Oncological and Oral Sciences, Section of General and Urgent Surgery, University of Palermo, Italy.

Department of Surgical, Oncological and Oral Sciences, Section of Oncology, University of Palermo, Italy.

出版信息

Int J Surg Case Rep. 2020;77S(Suppl):S105-S108. doi: 10.1016/j.ijscr.2020.09.120. Epub 2020 Sep 19.

Abstract

INTRODUCTION

Neuroendocrine tumors (NETs) represent uncommon neoplasms with different characteristics. They can be asymptomatic and benign or they can also proliferate and manifest themselves with neoplastic mass symptoms such as intestinal occlusion or with carcinoid syndrome. Gastric neuroendocrine neoplasms (g-NENs) are the most frequent digestive NENs while duodenal neuroendocrine neoplasms (d-NENs) may be sporadic or associated with multiple endocrine neoplasia type 1 (MEN-1) and present a functional syndrome (e.g. gastrinoma with Zollinger-Ellison syndrome).

PRESENTATION OF CASE

We report a case of duodenal perforation due to a unknown gastrinoma responsible of Zollinger-Ellison Syndrome. He underwent an emergency contrast enhanced CT abdominal scan that showed a perforation. We performed a distal gastrectomy. The histopathological examination revealed a g-NET configuring a possible picture of Zollinger-Ellison Syndrome.

DISCUSSION

The management of NETs is diffulcult and controversial because of their rarity. It is useful to know the pathologic assessment of tumor differentiation and/or grade, evaluate surgical resectability and control the carcinoid syndrome symptoms.

CONCLUSION

This case report shows that gastric NETs can be found in cases of duodenal perforation. Our future goal is to evaluate the possibilities to diagnose the Zollinger Ellison Syndrome as early as possible and to treat it with targeted therapy in order to prevent its related complications.

摘要

引言

神经内分泌肿瘤(NETs)是一类具有不同特征的罕见肿瘤。它们可能无症状且为良性,也可能增殖并表现出肿瘤性肿块症状,如肠梗阻或类癌综合征。胃神经内分泌肿瘤(g-NENs)是最常见的消化系神经内分泌肿瘤,而十二指肠神经内分泌肿瘤(d-NENs)可能是散发性的,或与1型多发性内分泌腺瘤病(MEN-1)相关,并呈现出一种功能性综合征(例如伴有卓-艾综合征的胃泌素瘤)。

病例介绍

我们报告一例因不明胃泌素瘤导致卓-艾综合征而引起十二指肠穿孔的病例。患者接受了腹部急诊增强CT扫描,结果显示有穿孔。我们实施了远端胃切除术。组织病理学检查显示为胃神经内分泌肿瘤(g-NET),呈现出可能的卓-艾综合征表现。

讨论

由于神经内分泌肿瘤罕见,其治疗具有难度且存在争议。了解肿瘤分化和/或分级的病理评估、评估手术可切除性以及控制类癌综合征症状是很有用的。

结论

本病例报告表明,十二指肠穿孔病例中可能发现胃神经内分泌肿瘤。我们未来的目标是评估尽早诊断卓-艾综合征并采用靶向治疗以预防其相关并发症的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/790b/7876995/0e9f9d4b03e1/gr1.jpg

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