Abstract:

(DSRCT) is an aggressive neoplasia that occurs mainly in childhood and involves abdominal or peritoneal sites, coexpressing ectodermal and mesenchimal immunophenotypic markers. Since it is a rare tumor, we aimed to report an additional case and discuss the differential diagnosis. Case: In this study, a 3- year- old boy with a large irregular abdominal and retroperitoneal infiltrating mass is presented. A large biopsy from the abdominal mass and a nodule from the liver were excised during explorative surgery. The surgical specimens are irregular solid fragile tissues with a grey-white cut surface. Uniform malignant round cells are observed in sharply demarcated nests and sheets in a desmoplastic stroma in microscopic examination. The nuclei are hyperchromatic, nucleoli are inconspicuous and cytoplasm is scant and eosinophilic. Immunohistochemistry showed perinucleer positivity for desmin, diffuse positivity for vimentin, cytokeratin, chromogranin, plasental alkaline phosphatase and negativity for alpha fetoprotein and CD99 in tumor cells. Conclusion: DSRCT demonstrates histopathologic and phenotypic overlap with some other malignancies. Differential diagnosis includes problems especially on tru-cut biopsies. Its aggressive course and necessity of complicated therapy requires a definite differential diagnosis

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