Congenital sarcoma in the terminal ileum histologically resembling clear cell sarcoma of the kidney: a case report with an immunohistochemical study.

We report a case of a newborn female with a rare tumor, a congenital sarcoma, presenting as an abdominal mass. Laparotomy demonstrated the tumor arising from the wall of the terminal ileum. Histologically, the tumor tissue was indistinguishable from clear cell sarcoma of the kidney and was composed of proliferating cells with poorly stained cytoplasm divided into nests or cords by arborizing vasculature. Immunohistochemical staining revealed that the neoplastic cells had a phenotype similar to metanephric blastemal cells of fetal kidney, ie, positive for vimentin and CD24 but negative for cytokeratin and CD9. The results suggest that this congenital tumor may originate from primitive mesenchymal cells phenotypically related to cells present in the fetal kidney.