What is DSRCT?

Understanding Desmoplastic Small Round Cell Tumor (DSRCT)

A Rare and Aggressive Cancer

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and aggressive soft tissue sarcoma that primarily develops in the abdomen and pelvic region. First identified in 1989, this cancer mostly affects young males between the ages of 10 and 30. Due to its rarity, fewer than 200 cases have been recorded worldwide, and there is no standard treatment protocol currently in place.

Disclaimer: The information provided here is based on research from MD Anderson Cancer Center and the National Cancer Institute but is not a substitute for professional medical advice. For official medical guidance, please consult a healthcare professional or visit trusted medical sources.

Because DSRCT often grows undetected until it reaches an advanced stage, symptoms may not appear until the tumor has spread. Common symptoms include:

  • Persistent abdominal pain or swelling
  • Unexplained weight loss
  • Loss of appetite or digestive issues
  • Fatigue and weakness
  • Bowel or urinary difficulties due to tumor growth

Since these symptoms are non-specific, early detection is challenging, making awareness and research critical in the fight against this disease.

DSRCT is difficult to diagnose due to its rarity and non-specific symptoms. The diagnosis typically involves:

  1. Imaging TestsCT scans, MRIs, and PET scans help detect tumors and determine their spread.
  2. Biopsy – A tissue sample is taken and analyzed to confirm the presence of small, round blue cells, a characteristic of DSRCT.
  3. Genetic Testing – DSRCT is associated with a specific chromosomal abnormality (EWS-WT1 fusion gene), which can help confirm the diagnosis.

 

There is no single effective treatment for DSRCT, making research and clinical trials crucial. Common treatment methods include:

  • Chemotherapy – Used to shrink tumors before surgery.
  • Aggressive Surgery – Aims to remove as much of the tumor as possible.
  • Radiation Therapy – Helps target remaining cancer cells after surgery.
  • Hyperthermic Intraperitoneal Chemotherapy (HIPEC) – A heated chemotherapy treatment applied directly inside the abdomen after surgery.

Despite these approaches, DSRCT often recurs, and survival rates remain low—around 15% at five years, highlighting the urgent need for more research and better treatment options.

The Kadeem Carson Foundation is committed to advancing research on DSRCT and other rare cancers by funding scientific studies and clinical trials aimed at improving treatment outcomes.

Ongoing and Past Research Initiatives

🔬 Investigating targeted therapies that focus on the genetic markers of DSRCT.
🔬 Exploring immunotherapy options to enhance the body’s natural ability to fight cancer.
🔬 Supporting clinical trials for experimental treatments that show promise.

Spotlight on Breakthroughs & Researchers

📢 Dr. Mary Louise Keohan, a leading researcher at Memorial Sloan Kettering Cancer Center, is pioneering new approaches in sarcoma treatment.

📢 Dr. Wyllie Hosmer of the Hartford HealthCare Cancer Institute continues to provide critical care and treatment for patients battling rare cancers.

(Stay tuned for updates on new research, studies, and success stories in the fight against DSRCT.)

A DSRCT diagnosis can be overwhelming, but support is available. Below are valuable resources for patients, caregivers, and loved ones.

Guides & Support Networks

📖 [Understanding DSRCT: A Patient & Family Guide] (Downloadable PDF coming soon!)
💙 [Find a Support Group] – Connect with others facing similar challenges.
🎗️ [Cancer Care Counseling Services] – Mental health resources for families coping with cancer.

Practical Tips for Managing Treatment

Navigating Treatment – What to expect and how to prepare.
Financial & Insurance Assistance – Programs that help cover treatment costs.
Emotional Well-being – Coping strategies and self-care for both patients and caregivers.