The entity was first described by pathologists William L. Gerald and Juan Rosai in 1989. Pathology reveals well circumscribed solid tumor nodules within a dense<unk>  stroma. Often areas of central necrosis are present. Tumor cells have hyperchromatic nuclei with increased nuclear/cytoplasmic ratio. On immunohistochemistry, these cells have trilinear coexpression including the epithelial marker cytokeratin, the mesenchymal markers desmin and vimentin, and the neuronal marker neuron-specific enolase. Thus, although initially thought to be of mesothelial origin due to sites of presentation, it is now hypothesized to arise from a progenitor cell with multiphenotypic differentiation.
Desmoplastic small-round-cell tumor
Plexiform angiomyxoid myofibroblastic tumor
Perivascular epithelioid cell tumour
Adenomatoid tumor
Solid pseudopapillary tumour
Sclerosing epithelioid fibrosarcoma
Small-blue-round-cell tumor
Multinucleate cell angiohistiocytoma