Desmoplastic small cell tumor with multi-phenotypic differentiation - PubMed (original) (raw)

Comparative Study

. 1993 Jun;139(2):141-51.

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Comparative Study

Desmoplastic small cell tumor with multi-phenotypic differentiation

W L Gerald et al. Zentralbl Pathol. 1993 Jun.

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.

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