Desmoplastic small round cell tumors

Diagnosis

The following tests may be performed to diagnose desmoplastic small round cell tumors:

• Tissue biopsy. A tissue sample is taken by surgery or by inserting a needle into the skin. The sample is sent to the laboratory for testing under a microscope to check for cancer cells and for determining the DNA changes present. The results will form a diagnosis and treatment plan.
• Imaging tests. Ultrasound, Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and Positron Emission Tomography (PET) may be performed in order to determine the size and location of cancer.

Treatment

Treatment will be decided based on the patient’s condition, location of cancer and the staging.

Surgery

Surgery aims to remove cancer if it is located in an area that it can be removed without impacting other organs. Chemotherapy may be recommended to reduce the size of the tumor primarily prior to surgery. If surgical removal is impossible, the surgeon will remove as much tumor as possible. Then chemotherapy and radiation can be done post-surgery to destroy the remaining cancer cells.

Chemotherapy

Chemotherapy uses medications to kills cancer cells. The options of chemotherapy include:

• Chemotherapy that affects the whole body. This chemotherapy is often taken intravenously. It can be used to treat desmoplastic small round cell tumor in any parts of the body. The medication flows all over the body and kills cancer cells.
• Hyperthermic Intraperitoneal Chemotherapy (HIPEC). In this procedure, chemotherapy is administered directly into the abdomen (peritoneal cavity) by heating up the medication and infusing it into the abdomen for a certain period of time and removed thereafter. It can be done after the surgical procedure.

Radiation therapy

Radiation utilizes a strong beam of energy through X-rays or protons in order to destroy cancer cells. The patient will lie down on the table and a machines maneuvers around the body at specific points to target cancer. In patients who have desmoplastic small round cell tumors, radiation may be done after surgery to destroy the remaining cancer cells or to relieve symptoms if the cancer has already metastasized.

Targeted drug therapy

Targeted therapy uses medication to destroy specific chemicals in the cancer cells. The doctor will test your cancer cells in the laboratory to determine the targeted therapy drug that will provide the maximum effect. This therapy can be used if the cancer has recurred after treatment. It may be utilized as a treatment alone or in combination with chemotherapy.