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Desmoplastic small cell tumor with multi-phenotypic differentiation

W L Gerald¹, J Rosai

¹Department of Pathology, Memorial Sloan-Kettering Cancer Center New York, New York.

Zentralblatt Fur Pathologie

|June 1, 1993

Summary

Desmoplastic small cell tumors (DSCT) are rare cancers primarily affecting young males, often located in the abdomen. These tumors exhibit a unique histology and genetic abnormality, suggesting a developmental origin.

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Area of Science:

Oncology
Pathology
Genetics

Background:

Desmoplastic small cell tumor (DSCT) is a rare and poorly understood malignant neoplasm.
DSCT presents a complex phenotype with distinct histological and immunohistochemical features.

Purpose of the Study:

To characterize the main features of 46 cases of desmoplastic small cell tumor.
To investigate the potential origin and classification of DSCT within neoplastic processes.

Main Methods:

Retrospective analysis of 46 histologically confirmed DSCT cases.
Evaluation of clinical data, histological patterns, immunohistochemical reactivity, and cytogenetic abnormalities.

Main Results:

The study included 46 patients (mean age 20.8 years, predominantly male).
Primary intra-abdominal location was observed in most cases.
Characteristic findings included nests of tumor cells within a desmoplastic stroma, multipotent differentiation markers, and a unique cytogenetic abnormality.

Conclusions:

DSCT represents a distinct neoplastic entity.
The findings suggest DSCT originates from a primitive progenitor cell with multipotent differentiation potential.
DSCT likely belongs to the family of primitive small round cell developmental tumors.

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