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 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.