DSRCT

Rare and Aggressive Cancer Strikes Young People

Desmoplastic small round cell tumors (DSRCT) are soft tissue sarcomas that primarily occur as masses in the abdomen, but other areas may be affected. Patients are adolescents and young adults, predominantly male. Even rarer (but occurring) in females, DSRCT is often mistaken for ovarian cancer.

DSRCT is a relatively “new” cancer (first described in 1989). It is frequently misdiagnosed, because most patients are young and otherwise healthy and because the disease has few early warning signals. Tumors often grow and spread uninhibited within the abdominal cavity before detection. There is no known point of origin. The prognosis for those affected is poor, with a five-year survival of about 15 percent.

Treatment

No standard treatment protocol exists. All current modalities (surgery, radiation, chemotherapy) are highly toxic, have numerous side effects, and are not particularly effective. Although DSRCTs have been shown to be sensitive to chemotherapy, treated patients invariably relapse. One theory behind the relapse is that unidentified stem cells among the DSRCT cells develop resistance and regenerate the tumor after it has been reduced by treatment. Identification of such stem cells should stimulate new molecularly-targeted therapies. Other directions being investigated are immunotherapy (e.g., using monoclonal antibodies to deliver chemical agents) and hyperthermic perfusion, or the destruction of the tumor’s cells through a combination of heat and chemotherapy.

Patrick Scranton Inspires Research

The Foundation’s interest in DSRCT resulted from meeting and working with Patrick Scranton, a remarkable DSRCT patient who succumbed to the disease in 2005. With the support of Patrick’s family, the Foundation initiated research into DSRCT, and its progress has been documented on a website initially created by Patrick to educate and provide referrals to other DSRCT patients – www.DSRCT.org.

Tumor Collection and Study

CHRISTUS Stehlin Foundation has been collecting DSRCT specimens for several years, and work continues to build a broader library of tumor cell lines. Currently, the Foundation has 17 DSRC tumors in storage, and nine distinct tumor xenografts are growing in its research mice. Dr. Constantine Markides is leading the Foundation’s research investigating treatments of DSRCT using hyperthermia and chemotherapy.

DSRCT research may be aided by the use of green fluorescent proteins to mark the metastatic spread of the disease. When a gene encoded with the protein in inserted into the cancer cells, the cells fluoresce (glow) when exposed to a particular spectrum of light. This provides a visual record the tumor’s growth and metastasis, and allows the effects of experimental therapies to be documented more accurately.

If you, a family member, or patient are interested in participating in the Foundation’s DSRCT research or have questions about the tumor collection program, please contact Doug Coil, Deputy Laboratory Supervisor, 713-659-1336.