Desmoplastic small cell tumor with multi-phenotypic differentiation.
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.
Department of Pathology, Memorial Sloan-Kettering Cancer Center New York, New York.
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WL
Gerald
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Rosai
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D014408:Biomarkers, Tumor / Q000032:analysis; D018288:Carcinoma, Small Cell / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D002454:Cell Differentiation; D002648:Child; D002880:Chromosomes, Human, Pair 11; D002892:Chromosomes, Human, Pair 22; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012737:Sex Factors; D014178:Translocation, Genetic
D014408:Biomarkers, Tumor