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Genetic and Rare Diseases Information Center (GARD)

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Desmoplastic small round cell tumor


Other Names for this Disease

  • Desmoplastic small round-cell tumor
  • DSRCT
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Overview

Desmoplastic small round cell tumors (DSRCT), a rare malignant cancer, is a soft tissue sarcoma that usually affects young boys and men and is found most often in the abdomen. Its name means that it is formed by small, round cancer cells surrounded by scarlike tissue. The most common symptoms include abdominal pain, abdominal mass and symptoms of gastrointestinal obstruction.[1] DSRCTs are treated first with chemotherapy, then with surgery to remove the tumor, if possible. Radiation therapy is sometimes given, depending on the tumor. In addition, some people with DSRCT are candidates for a bone marrow transplant.[2]
Last updated: 6/19/2009

References

  1. Desmoplastic tumors. Mayo Clinic. http://www.mayoclinic.org/desmoplastic-tumors/. Accessed 6/19/2009.
  2. Desmoplastic tumors - treatment. Mayo Clinic. http://www.mayoclinic.org/desmoplastic-tumors/treatment.html. Accessed 6/19/2009.
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In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Desmoplastic small round cell tumor. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Desmoplastic small round-cell tumor
  • DSRCT
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.