Malignant Small-Cell Epithelial Tumor of the Peritoneum Coexpressing Mesenchymal-type Intermediate Filaments

A Case of Desmoplastic Small Round Cell Tumor Diagnosed in a Young Female Patient

“…Because the tumor can have various differentiations such as epithelial, mesenchymal, or neuronal differentiation, it is difficult to diagnose DSRCT even when the immunohistochemistry results are available (3)(4)(5).…”

PDGF-A, PDGF-Rβ, TGFβ3 and bone morphogenic protein-4 in desmoplastic small round cell tumors with EWS-WT1 gene fusion product and their role in stromal desmoplasia: an immunohistochemical study

“…[1][2][3] Histologically, it is composed of neoplastic small round cells with divergent differentiation in a characteristic prominent stromal proliferation called desmoplasia. [1][2][3][4][5] Genetically, the tumor shows a characteristic t(11:22)(p13;q12) involving the EWS on chromosome 22 and the WT1 on chromosome 11, resulting in an EWS-WT1 fusion gene. [6][7][8] The EWS-WT1 fusion gene generates a chimeric protein containing the potential transactivation domain of the EWS protein fused to zinc-finger DNA-binding domain of the WT1 protein.…”

Book Review

“…It was originally described as a tumor primarily involving the abdominal peritoneum, with characteristic small round cell morphology (1,(3)(4)(5). Although the histogenesis of desmoplastic small round cell tumor has yet to be elucidated, immunophenotypical analysis shows a polyphenotypic differentiation overlapping with other round cell tumors such as Ewing's sarcoma/peripheral neuroectodermal tumors, rhabdomyosarcoma, Wilms' tumor, small cell mesothelioma, and carcinoma (1)(2)(3)(4)(5). Genetic studies revealed a characteristic translocation between the EWS gene on chromosome 22 and the WT1 gene on chromosome 11, resulting in an EWS-WT1 fusion gene (6 -8).…”