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回肠恶性胃肠道神经外胚层肿瘤:一例报告及影像学特征综述

Malignant gastrointestinal neuroectodermal tumor of the ileum: A case report and review of imaging characteristics.

作者信息

Miwa Tatsuki, Tokuda Bunta, Sato Osamu, Honda Mizuki, Imura Tetsuya, Katada Kazuhiro, Ochiai Toshiya, Yamada Kei

机构信息

Department of Radiology, North Medical Center of Kyoto Prefectural University of Medicine, 481 Otokoyama, Yosanocho, Yosagun, Kyoto 629-2261, Japan.

Department of Radiology, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto 602-8566, Japan.

出版信息

Radiol Case Rep. 2025 Mar 8;20(5):2597-2603. doi: 10.1016/j.radcr.2025.02.029. eCollection 2025 May.

Abstract

Malignant gastrointestinal neuroectodermal tumors (MGNETs) are extremely rare malignant mesenchymal tumors derived from ectodermal neural cells of the gastrointestinal tract that most commonly arise in the small intestine. Preoperative diagnosis is challenging owing to the lack of well-established imaging characteristics. Here, we report the case of a 50-year-old female patient with MGNET of the ileum. Computed tomography revealed a highly lobulated hypervascular mass and circumferential wall thickening in the distal ileum, whereas magnetic resonance imaging revealed marked diffusion restriction. A laparoscopic ileal resection was performed. The patient remained recurrence-free for 1 year without additional treatment. Specific imaging characteristics observed in our case, including the combined pattern of wall thickening and submucosal mass formation, marked diffusion restriction, and highly lobulated appearance, may aid in differentiating MGNET from other tumors.

摘要

恶性胃肠道神经外胚层肿瘤(MGNETs)是极其罕见的恶性间充质肿瘤,起源于胃肠道的外胚层神经细胞,最常见于小肠。由于缺乏成熟的影像学特征,术前诊断具有挑战性。在此,我们报告一例50岁女性回肠MGNET患者。计算机断层扫描显示回肠远端有一个高度分叶状的高血运肿块及肠壁环形增厚,而磁共振成像显示明显的弥散受限。进行了腹腔镜回肠切除术。患者未经额外治疗,1年内无复发。我们病例中观察到的特定影像学特征,包括肠壁增厚和黏膜下肿块形成的联合模式、明显的弥散受限以及高度分叶状外观,可能有助于将MGNET与其他肿瘤区分开来。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a4b/11930665/6c63667af35a/gr1.jpg

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