Clear cell sarcoma of the ileum: A case report

Abstract

We report a case of clear cell sarcoma of the ileum in an adult. A 41-year-old woman presented with fever, hematochezia and anemia. A tumor in the pelvic cavity was visualized by abdominal CT and MRI. A segmental small bowel resection and sigmoidectomy were performed. Histological examination of the specimen revealed a tumor with epithelioid neoplasm which was infiltrating the bowel wall in sheets. The tumor cells had high nuclearcytoplasmic ratio, and clear cytoplasm. The immunohistochemical profile of the tumor was focally positive for S-100, and diffusely positive for SOX10. EWRS1 gene rearrangement was observed during cytogenetic analysis. We diagnosed the tumor as a clear cell sarcoma of the ileum. The patient had postoperative adjuvant chemotherapy using doxorubicin and ifosfamide for five courses. No recurrences had observed for 28 months.