Abstract: |
Desmoplastic small cell tumor (DSCT) is a distinctive, poorly understood malignant tumor with a complex phenotype. The main features of the 46 cases presented here include a mean age of 20.8 years (range 7-48), male predominance (37 M:9 F), primary intraabdominal location (except for 2 cases), characteristic histologic pattern consisting of tumor cell nests surrounded by a cellular desmoplastic stroma, immunohistochemical reactivity for markers of epithelial, muscle and neural differentiation, and association with a unique cytogenetic abnormality. These findings suggest that this tumor is a distinct neoplastic process and that it probably belongs to the family of primitive small round cell developmental tumors. It is proposed that DSCT arises during development from a progenitor cell with potential for multiphenotypic differentiation. |
Keywords: |
immunohistochemistry; adolescent; adult; child; middle aged; gene translocation; genetics; comparative study; phenotype; tumor markers, biological; age factors; cell differentiation; pathology; tumor marker; age; translocation, genetic; sex difference; sex factors; chromosome 11; chromosomes, human, pair 11; ultrastructure; small cell carcinoma; carcinoma, small cell; chromosome 22; chromosomes, human, pair 22; humans; human; male; female; article
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