PDF(Pubmed)
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（GIST）是罕见的肿瘤，发病率越来越高。GIST是胃肠道最常见的间质瘤，涉及老年人群，进展缓慢。它起源于Cajal的间质细胞。在胃肠道外发展并且与胃肠管状器官的肠壁或浆膜表面没有连接的GIST被称为肠外胃肠道间质瘤(EGIST)。它们具有与GIST相似的形态学和免疫组织学特征。这里,我们描述了一个在44岁的非裔美国男性中发生的极具侵袭性的肠系膜GIST的独特病例.患者出现在医院，抱怨与50磅体重减轻有关的全身性腹痛，食欲下降,还有便秘.他接受了腹部和骨盆的计算机断层扫描（CT），显示沿中央肠系膜有一个大肿块，约15×11cm，并伴有相邻的转移性淋巴结疾病。他接受了腹部肿块的CT引导活检，组织病理学结果为c-kit（CD117）阳性，并在GIST-1（DOG-1）上发现与GIST一致。根据TNM分期，他的肿瘤为T4级，N1级，考虑到淋巴结受累，他处于IV期。他被转诊给肿瘤科医生，并开始接受伊马替尼的新辅助治疗。肠系膜EGIST,虽然罕见，已知与其他EGIST相比，预后较差；因此，必须迅速采取行动积极治疗这些肿瘤。有丝分裂指数和肿瘤大小等因素影响肠系膜GIST的预后。
