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高度侵袭性肠系膜胃肠道外间质瘤:一例报告及文献复习

Extremely Aggressive Mesenteric Extragastrointestinal Stromal Tumor: A Case Report and Literature Review.

作者信息

Abidoye Oluseyi, Johnson Andrew

机构信息

Internal Medicine, Northeast Georgia Medical Center Gainsville, Gainesville, USA.

Oncology, Longstreet Clinic Cancer Center, Gainesville, USA.

出版信息

Cureus. 2022 Mar 12;14(3):e23108. doi: 10.7759/cureus.23108. eCollection 2022 Mar.

Abstract

Gastrointestinal stromal tumors (GISTs) are rare tumors with increasing incidence. GIST is the most common mesenchymal tumor of the gastrointestinal tract involving the elderly population with a slow progression. It originates from the interstitial cells of Cajal. GISTs that develop outside the gastrointestinal tract and have no connections with the intestinal walls or serosal surfaces of the gastrointestinal tubular organs are referred to as extraintestinal gastrointestinal stromal tumors (EGISTs). They have similar morphological and immunohistological characteristics as GISTs. Here, we describe a unique case of an extremely aggressive mesenteric GIST in a 44-year-old African American male. The patient presented to the hospital with complaints of generalized abdominal pain associated with 50-pound weight loss, decreased appetite, and constipation. He underwent computed tomography (CT) of the abdomen and pelvis which showed a large mass along the central mesentery measuring about 15 × 11 cm with adjacent metastatic nodal disease. He underwent a CT-guided biopsy of his abdominal mass with histopathology findings positive for c-kit (CD117) and discovered on GIST-1 (DOG-1) consistent with GIST. Based on TNM staging, his tumor was graded T4 with N1 given nodal involvement placing him as a stage IV. He was referred to an oncologist and was started on neoadjuvant therapy with imatinib. Mesenteric EGISTs, while rare, are known to have a worse prognosis compared to other EGISTs; hence, prompt action must be taken in aggressively treating these tumors. Factors such as mitotic index and tumor size affect the prognosis of mesenteric GISTs.

摘要

胃肠道间质瘤(GISTs)是一种发病率呈上升趋势的罕见肿瘤。GIST是胃肠道最常见的间充质肿瘤,多见于老年人群,进展缓慢。它起源于Cajal间质细胞。发生在胃肠道外且与胃肠道管状器官的肠壁或浆膜面无连接的GISTs被称为肠外胃肠道间质瘤(EGISTs)。它们具有与GISTs相似的形态学和免疫组织学特征。在此,我们描述了一例44岁非裔美国男性中极为侵袭性的肠系膜GIST的独特病例。该患者因全身腹痛、体重减轻50磅、食欲减退和便秘而入院。他接受了腹部和盆腔的计算机断层扫描(CT),结果显示沿肠系膜中部有一个约15×11 cm的大肿块,并伴有相邻的转移性淋巴结疾病。他接受了腹部肿块的CT引导下活检,组织病理学结果显示c-kit(CD117)阳性,且在GIST-1(DOG-1)检测中确诊为GIST。根据TNM分期,他的肿瘤分级为T4,伴有N1(淋巴结受累),属于IV期。他被转诊给肿瘤学家,并开始接受伊马替尼新辅助治疗。肠系膜EGISTs虽然罕见,但已知与其他EGISTs相比预后更差;因此,必须迅速采取积极措施治疗这些肿瘤。有丝分裂指数和肿瘤大小等因素会影响肠系膜GISTs的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e2c2/9002020/1a88b0e9ccda/cureus-0014-00000023108-i01.jpg

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