BACKGROUND Clear cell sarcoma-like tumor of the gastrointestinal tract(CCSLGT)is a rare malignant gastrointestinal mesenchymal soft tissue tumor.Its genetic feature is EWSR1 gene rearrangement.Histologically,it is oft...BACKGROUND Clear cell sarcoma-like tumor of the gastrointestinal tract(CCSLGT)is a rare malignant gastrointestinal mesenchymal soft tissue tumor.Its genetic feature is EWSR1 gene rearrangement.Histologically,it is often accompanied by a varying number of CD68-positive osteoclast-like giant cells.CCSLGT mostly occurs in the small intestinal wall of young people and children.In terms of clinical manifestations,there is no significant difference between it and other gastrointestinal tumors,and the diagnosis depends on immunohistochemistry and gene detection.CASE SUMMARY A 16-year-old man developed dizziness and fatigue 2 mo ago,and 10 d ago showed progressive exacerbation of paroxysmal epigastric pain and stopped flatulence and defecation.Computed tomography showed a soft tissue mass in the distal ileum.After complete resection of the lesion,it was diagnosed by combined immunohistochemical and genetic examination as CCSLGT.After surgery,the patient gradually developed lymph node,liver,lung,bone,left thigh,pleura and adrenal metastasis.The survival time was 4 years and 8 mo.CONCLUSION Whole abdominal computed tomography enhancement is recommended for patients with gastrointestinal symptoms.There is no effective treatment for CCSLGT with multiple metastases via the lymphatic system and bloodstream after surgical resection.展开更多
目的探讨胃肠道透明细胞肉瘤(clear cell sarcoma of the gastrointestinal tract,CCS-GI)的临床病理特征及遗传学特点。方法对1例CCS-GI进行组织学观察、免疫组化染色、荧光原位杂交(fluorescence in situ hybridization,FISH)检测,并...目的探讨胃肠道透明细胞肉瘤(clear cell sarcoma of the gastrointestinal tract,CCS-GI)的临床病理特征及遗传学特点。方法对1例CCS-GI进行组织学观察、免疫组化染色、荧光原位杂交(fluorescence in situ hybridization,FISH)检测,并复习相关文献。结果患者女性,因"腹痛1周"入院,CT检查示右半结肠肿瘤,肉眼观察结肠黏膜面可见一溃疡型肿块,切面灰白、实性、质嫩,侵及全层。镜下见中等大小的圆形或卵圆形肿瘤细胞呈片状排列,肿瘤细胞间可见散在分布的破骨细胞样多核巨细胞。肿瘤细胞S-100蛋白弥漫阳性,HMB-45、Melan-A、CD117、CD1a及PCK均阴性。FISH检测结果示74%的肿瘤细胞存在EWSR1基因易位。结论 CCS-GI是一种特殊类型的胃肠道肿瘤,具有独特的组织学、免疫表型、超微结构及遗传学特征,该类肿瘤中的胃肠道透明细胞肉瘤样肿瘤亚型是否为一个独立的病变实体,尚需增加病例量进一步研究,包括细胞遗传学和分子生物学的相关研究。展开更多
文摘BACKGROUND Clear cell sarcoma-like tumor of the gastrointestinal tract(CCSLGT)is a rare malignant gastrointestinal mesenchymal soft tissue tumor.Its genetic feature is EWSR1 gene rearrangement.Histologically,it is often accompanied by a varying number of CD68-positive osteoclast-like giant cells.CCSLGT mostly occurs in the small intestinal wall of young people and children.In terms of clinical manifestations,there is no significant difference between it and other gastrointestinal tumors,and the diagnosis depends on immunohistochemistry and gene detection.CASE SUMMARY A 16-year-old man developed dizziness and fatigue 2 mo ago,and 10 d ago showed progressive exacerbation of paroxysmal epigastric pain and stopped flatulence and defecation.Computed tomography showed a soft tissue mass in the distal ileum.After complete resection of the lesion,it was diagnosed by combined immunohistochemical and genetic examination as CCSLGT.After surgery,the patient gradually developed lymph node,liver,lung,bone,left thigh,pleura and adrenal metastasis.The survival time was 4 years and 8 mo.CONCLUSION Whole abdominal computed tomography enhancement is recommended for patients with gastrointestinal symptoms.There is no effective treatment for CCSLGT with multiple metastases via the lymphatic system and bloodstream after surgical resection.
文摘目的探讨胃肠道透明细胞肉瘤(clear cell sarcoma of the gastrointestinal tract,CCS-GI)的临床病理特征及遗传学特点。方法对1例CCS-GI进行组织学观察、免疫组化染色、荧光原位杂交(fluorescence in situ hybridization,FISH)检测,并复习相关文献。结果患者女性,因"腹痛1周"入院,CT检查示右半结肠肿瘤,肉眼观察结肠黏膜面可见一溃疡型肿块,切面灰白、实性、质嫩,侵及全层。镜下见中等大小的圆形或卵圆形肿瘤细胞呈片状排列,肿瘤细胞间可见散在分布的破骨细胞样多核巨细胞。肿瘤细胞S-100蛋白弥漫阳性,HMB-45、Melan-A、CD117、CD1a及PCK均阴性。FISH检测结果示74%的肿瘤细胞存在EWSR1基因易位。结论 CCS-GI是一种特殊类型的胃肠道肿瘤,具有独特的组织学、免疫表型、超微结构及遗传学特征,该类肿瘤中的胃肠道透明细胞肉瘤样肿瘤亚型是否为一个独立的病变实体,尚需增加病例量进一步研究,包括细胞遗传学和分子生物学的相关研究。
