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青少年胃肠道透明细胞肉瘤样肿瘤术后多发转移:一例报告

Postoperative multiple metastasis of clear cell sarcoma-like tumor of the gastrointestinal tract in adolescent: A case report.

作者信息

Huang Wen-Peng, Li Li-Ming, Gao Jian-Bo

机构信息

Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Henan Province, China.

出版信息

World J Clin Cases. 2022 Jun 26;10(18):6175-6183. doi: 10.12998/wjcc.v10.i18.6175.

Abstract

BACKGROUND

Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT) is a rare malignant gastrointestinal mesenchymal soft tissue tumor. Its genetic feature is 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 the lymphatic system and bloodstream after surgical resection.

摘要

背景

胃肠道透明细胞肉瘤样肿瘤(CCSLGT)是一种罕见的恶性胃肠道间叶性软组织肿瘤。其基因特征是基因重排。组织学上,它常伴有数量不等的CD68阳性破骨细胞样巨细胞。CCSLGT多发生于年轻人和儿童的小肠壁。在临床表现方面,它与其他胃肠道肿瘤无明显差异,诊断依赖于免疫组织化学和基因检测。

病例摘要

一名16岁男性2个月前出现头晕、乏力,10天前阵发性上腹部疼痛进行性加重,停止排气排便。计算机断层扫描显示回肠末端有一软组织肿块。病变完整切除后,经免疫组织化学和基因联合检查诊断为CCSLGT。术后患者逐渐出现淋巴结、肝、肺、骨、左大腿、胸膜及肾上腺转移。生存时间为4年8个月。

结论

对于有胃肠道症状的患者,建议行全腹计算机断层扫描增强检查。手术切除后发生淋巴系统和血行多处转移的CCSLGT尚无有效治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bab/9254186/2791b19c64da/WJCC-10-6175-g001.jpg

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