Desmoplastic Small Round Cell Tumor (DSRCT)

Most parents would agree—a cancer diagnosis for your son or daughter is much more difficult to bear than a cancer diagnosis for yourself. So if you are reading this informational guide as a result of a Desmoplastic Small Round Cell Tumor (DSRCT) diagnosis for your child, we offer our condolences.

While we recommend that you speak to your doctor or team of medical professionals about all aspects of your child’s diagnosis, you can consider what follows to be an introduction to the disease, designed to help you ask more informed questions.

In addition, the bottom of this guide includes a list of a few reasons why you might want to pursue a medical malpractice and/or tort claim if you believe that your child’s DSRCT diagnosis has been mishandled. You can also use this information to ensure that you fully discuss any potential complications with your doctor.

About Desmoplastic Small Round Cell Tumor (DSRCT)

DSRACT is a particularly aggressive cancer that happens almost exclusively in young people. Its manifestations are typically related to wider abdominal disease, and it is often accompanied by epithelial, neuronal and mesenchymal markers.

Oftentimes distant metastases are found at the time of diagnosis (meaning, that when the cancer is found, it has already spread to other areas of the body). This occurs in around 40 percent of patients.

Even with intensive surgery and chemotherapy and/or radiation, the prognosis for this disease is poor, with five year survival rates less than fifteen percent.

Manifestations of DSRCT

Typically DSRCT is first identified as an abdominal mass with peritoneal (the lining of the abdominal cavity) and omental (a fold of peritoneum that hangs from the stomach) implants.

Patients with DSRCT might experience the following symptoms:

• Abdominal pain and cramping
• Weight loss
• Constipation
• Scoliosis
• Chronic sinusitis
• Erectile dysfunction
• Pain

Occasionally DSRCT is an incidental finding of Caesarian sections.

Treatment of DSRCT

DSRCT happens to be a particularly aggressive form of cancer. As mentioned, its five year survival rate is less than fifteen percent. There is no standard therapeutic course recommended for DSRCT because no one method has shown a clear superiority to others.

Typically DSRCT is treated through surgery, chemotherapy, radiotherapy and sometimes stem cell transplantation. Recently molecular targeted therapy has been attempted as well.

Surgery is invasive, and involves excision of the omentum, a splenectomy and lymph node resections. Because the tumor is so invasive, it is rarely possible to achieve the ideal – complete excision with wide surgical margins.

Chemotherapy more often than not is ineffective in treating DSRCT. Although these tumors are sensitive to the chemotherapy, almost all patients relapse. This is probably because there are certain cells (sometimes called cancer stem cells) that are particularly resistant to chemotherapy and/or radiation, and these are the cells that tend to metastasize.

There are also some local control options for DSRCT which include:

• Radiofrequency ablation
• Gammaknife
• Cryoablation
• Embolization
• Chemoembolization