Desmoplastic small cell tumor with multi-phenotypic differentiation Journal Article


Authors: Gerald, W. L.; Rosai, J.
Article Title: Desmoplastic small cell tumor with multi-phenotypic differentiation
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
Journal Title: Zentralblatt für Pathologie
Volume: 139
Issue: 2
ISSN: 0863-4106
Publisher: Gustav Fischer Verlag  
Date Published: 1993-06-01
Start Page: 141
End Page: 151
Language: English
PUBMED: 8396418
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 1 March 2019 -- Source: Scopus
Citation Impact
MSK Authors
  1. Juan Rosai
    181 Rosai
  2. William L Gerald
    375 Gerald