Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and aggressive cancer that affects mainly children and young adults. It is a type of soft tissue sarcoma that usually arises in the abdomen, but can also occur in other parts of the body. Due to its rarity and aggressiveness, there is no standard treatment protocol for DSRCT, but various treatment options are available, which are discussed below.
Surgery: Surgery is the primary treatment for DSRCT, and the goal of surgery is to remove as much of the tumor as possible. However, due to the location and size of the tumor, complete removal may not be possible. If the tumor has spread to other organs, surgery may not be an option. In some cases, surgery may be followed by radiation therapy and/or chemotherapy to eliminate any remaining cancer cells.
Radiation therapy: Radiation therapy involves using high-energy radiation to kill cancer cells. It may be used before or after surgery, or as a standalone treatment. Radiation therapy can be delivered externally (external beam radiation) or internally (brachytherapy).
Chemotherapy: Chemotherapy involves using drugs to kill cancer cells. It is usually given intravenously, but in some cases, it may be given directly into the abdomen (intraperitoneal chemotherapy). Chemotherapy is often used in combination with surgery and/or radiation therapy. There is no standard chemotherapy regimen for DSRCT, but a combination of drugs such as ifosfamide, etoposide, vincristine, and doxorubicin may be used.
Targeted therapy: Targeted therapy is a newer type of cancer treatment that targets specific genes, proteins, or other molecules that contribute to the growth and spread of cancer cells. It may be used in combination with chemotherapy, and examples of targeted therapy drugs include trabectedin and pazopanib.
Immunotherapy: Immunotherapy involves using the body’s own immune system to fight cancer. It may be used in combination with chemotherapy and/or radiation therapy. Examples of immunotherapy drugs used for DSRCT include pembrolizumab and nivolumab.
Clinical trials: Clinical trials are research studies that test new treatments or combinations of treatments for cancer. Patients with DSRCT may be eligible to participate in clinical trials to receive experimental treatments.
In summary, treatment for DSRCT often involves a combination of surgery, radiation therapy, chemotherapy, targeted therapy, immunotherapy, and/or participation in clinical trials. The treatment plan depends on various factors such as the location and size of the tumor, the stage of the cancer, and the overall health of the patient. A multidisciplinary team of doctors, including oncologists, surgeons, and radiation oncologists, will work together to develop an individualized treatment plan for each patient.