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Birth and evolution of the desmoplastic small round-cell tumor
Enrique de Alava1, David Marcilla1
1Department of Pathology, Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital, CSIC, University of Sevilla, Seville 41013, Spain; Department of Pathology, La Merced Hospital, AGS Osuna, Osuna, Spain.
Abstract:
The first large series of desmoplastic small round cell tumor was reported twenty-five years ago. This article reviews the original characterization of this neoplasm, and the eventual expansion of its clinical and pathological spectrum. Relevant data on its molecular features are summarized, in order to understand the search for therapeutic targets. The challenge ahead is to better know and cure this disease through the finding and validation of actionable therapeutic targets.
Insights
Desmoplastic small round cell tumor (DSRCT) has evolved over 25 years. This review covers its clinical, pathological, and molecular features, highlighting the need for new therapeutic targets to improve patient outcomes.
Area of Science:
- Oncology
- Pathology
- Molecular Biology
Background:
- Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive sarcoma.
- First described 25 years ago, its clinical and pathological spectrum has expanded significantly.
Purpose of the Study:
- To review the evolution of DSRCT characterization over the past 25 years.
- To summarize molecular features and identify potential therapeutic targets for DSRCT.
Main Methods:
- Literature review of original characterization and subsequent studies on DSRCT.
- Synthesis of data on clinical presentation, pathology, and molecular alterations.
Main Results:
- The clinical and pathological spectrum of DSRCT is broader than initially recognized.
- Molecular data provides insights into potential therapeutic vulnerabilities.
Conclusions:
- Understanding the expanded spectrum and molecular landscape of DSRCT is crucial.
- Further research is needed to validate actionable therapeutic targets for improved DSRCT treatment.
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