Desmoplastic Small Round Cell Tumor (DSRCT) Clinical Trials

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignant neoplasm characterized by pathognomonic EWSR1-WT1 fusion gene resulting from t(11;22)(p13;q12) translocation. The tumor arises in the peritoneum (~80% of cases), pleura, biliary tract, and rarely other sites. Histologically characterized by clusters of small round cells surrounded by prominent desmoplastic stromal reaction.

The most common symptoms of Desmoplastic Small Round Cell Tumor include Abdominal mass and abdominal distension, Abdominal pain, Nausea and vomiting, Constipation or bowel obstruction, Weight loss and fatigue, among others. Fewer than 200 cases per year in the U.S.; occurs at any age but most common age 15-35. Symptoms can range from mild to severe and may vary between individuals.

Trial Friend lists all active clinical trials for Desmoplastic Small Round Cell Tumor from ClinicalTrials.gov, with eligibility criteria translated into plain English. Patients can filter by trial phase and recruitment status, search by location, and use the Match Me tool to score trials against their profile. Clinical trials are investigating novel chemotherapy combinations, targeted therapies against EWS-WT1 fusion, and immunotherapy approaches for DSRCT. Early diagnosis and aggressive multimodal therapy including induction chemotherapy (typically doxorubicin and cyclophosphamide-based), complete surgical debulking when feasible, and high-dose chemotherapy with stem cell rescue improve survival outcomes.

As of October 14, 2026, no current FDA drug label names Desmoplastic Small Round Cell Tumor as a condition the drug treats.

Desmoplastic Small Round Cell Tumor arises from acquired (somatic) genetic changes in the affected cells, commonly involving EWSR1-WT1 fusion, rather than from an inherited variant. The t(11;22) EWSR1::WT1 fusion in tumor tissue confirms the diagnosis; it is acquired, not inherited. Fewer than 200 cases per year in the U.S.; occurs at any age but most common age 15-35.

For Desmoplastic Small Round Cell Tumor the genetic testing that matters is usually of the tumor or affected blood cells rather than inherited DNA, looking for EWSR1-WT1 fusion. The t(11;22) EWSR1::WT1 fusion in tumor tissue confirms the diagnosis; it is acquired, not inherited.

Those results guide treatment choice and are often the entry requirement for targeted-therapy trials, so ask whether the biopsy was sent for molecular testing and request the report. The Genetic Testing section of this page lists laboratories in the NIH Genetic Testing Registry.

Eligibility for Desmoplastic Small Round Cell Tumor clinical trials depends on factors like age, diagnosis, treatment history, and overall health. Each trial sets its own inclusion and exclusion criteria. Trial Friend translates these requirements from medical language into plain English so patients can review them more easily. The Match Me tool can score your profile against active trials to help identify studies that may be a fit.

Participating in a Desmoplastic Small Round Cell Tumor clinical trial typically involves scheduled visits, medical tests, and close monitoring by a research team. Depending on the study phase, you may receive an investigational treatment, a placebo, or the current standard of care. Trials may last from a few weeks to several years. Study participation is voluntary, and you can withdraw at any time.