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Abstract：
Gastrointestinal clear cell sarcoma (GICCS)/malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare form of cancer with aggressive clinical behavior. It has distinct pathological, immunohistochemical, ultrastructural, and molecular features. Herein, we present the case of a 20-year-old woman with no notable medical history who presented to the outpatient department with complaints of abdominal pain and vomiting. Symptoms had been evolving for 3 months. The physical examination revealed slight abdominal tenderness and melena. Biological investigations revealed iron-deficiency anemia. The upper and lower endoscopies showed no abnormalities. Magnetic resonance enterography revealed small bowel wall thickening of 15 mm × 2 mm. Exploratory laparotomy revealed an ileal mass with mesenteric lymphadenopathy. A wide resection of the mass was then performed. The final pathological report confirmed the diagnosis of small bowel GICCS/GNET. After 11 months of follow-up, the patient presented with mesenteric lymph node metastases.
摘要：
胃肠道透明细胞肉瘤（GICCS）/恶性胃肠道神经外胚层肿瘤（GNET）是一种极为罕见的癌症，具有侵袭性临床行为。它有明显的病理，免疫组织化学，超微结构，和分子特征。在这里，我们介绍了一例20岁无明显病史的女性，她到门诊部就诊，主诉腹痛和呕吐。症状已经演变了3个月。体格检查显示轻微的腹部压痛和黑便。生物学研究显示缺铁性贫血。上部和下部内窥镜检查未显示异常。磁共振小肠造影显示小肠壁增厚15mm×2mm。剖腹探查术显示回肠肿块伴肠系膜淋巴结肿大。然后进行肿块的广泛切除。最终病理报告证实诊断为小肠GICCS/GNET。经过11个月的随访，患者出现肠系膜淋巴结转移。
