Chemotherapy and surgery are common treatments for desmoplastic small round cell tumor (DSRCT) in children. MSK Kids offers all treatments for DSRCT.

We will talk with you about the best treatment plan for your child. We will answer all your questions, so you will know what to expect during your child’s treatment.

Chemotherapy Drugs for DSRCT

We often give chemotherapy (chemo) to first shrink the tumor and make it easier to remove with surgery. These drugs also lower the chances the cancer will come back. Most chemotherapy drugs are given intravenously (by vein). Examples of chemotherapy drugs include gemcitabine and docetaxel.

Some medicines are given orally (by mouth). Your child’s doctor will talk with you about the drugs, how they are given, and for how long.

DSRCT Surgery

MSK Kids cancer surgeons in New York City have improved DSRCT treatment. They have developed new ways to remove these tumors. They work as part of a very experienced team that includes medical and radiation oncologists, nurses, and others.

Why choose MSK for DSRCT surgery?

Our surgeons do more desmoplastic small round cell tumors operations in a year than many others do in a career. Research suggests that patients at hospitals that do a high volume of a complex surgery have better treatment results.

Desmoplastic small round cell tumors can be very hard to remove all the way. They often are wrapped around blood vessels and other organs. That’s why we often give chemotherapy first to shrink the tumor, which makes surgery easier.

MSK Kids surgeons take on cases that other centers may turn away because they’re too complex. Many people bring their child to MSK Kids because of our surgeons’ reputations as experts in pediatric sarcoma care.

The operation to remove DSRCT is very long, sometimes lasting 12 hours. Experience matters with this type of surgery. MSK Kids surgeons were the first to show it’s best to remove as much of the tumor as possible. This is called debulking surgery. Taking out the tumor and nearby tissues helps children live longer.

DSRCT surgery recovery

Your child’s surgeon will talk with you about how long your child will be in the hospital. The recovery from DSRCT surgery can take several weeks. Often, there’s pain and swelling around the incision (surgical cut). We do all we can do make your child feel comfortable.

Your care team will tell you how to care for your child at home. They will talk with you about when your child can go back to their regular activities.

Improving surgeries for desmoplastic small round cell tumors

When we remove DSRCT tissue, we send it to MSK’s lab. There, our scientists try to grow and study the tumor tissue. They hope to find new molecules involved in DSRCT growth so we can make better treatments to target them.

For DSRCT in the liver, our surgeons are working with other hospitals to see if a liver transplant can help. They could remove the liver with cancer and replace it with a healthy liver. This is an area of great promise for kids with DSRCT in the liver.

Radiation Therapy for DSRCT

Some children have radiation therapy, a treatment that directs beams of high-energy rays to kill cancer cells. Your doctor will let you know if this is an option for your child.

Intensity-modulated radiation therapy for DSRCT

Intensity-modulated radiation therapy (IMRT) is a very precise form of radiation therapy used for some children with DSRCT.

A treatment machine will aim radiation beams of different strengths right at the tumor. The beams kill cancer cells without harming nearby healthy tissues. This causes fewer side effects.

Your child’s doctor will explain how many treatments your child may need and how long they last. They also will tell you what side effects to expect and how to care for them.

Proton therapy

Proton therapy is a treatment choice for some kids with DSRCT in the abdomen (belly). It uses charged particles called protons to aim high doses of radiation right at tumors.

Proton therapy can cause less harm to healthy tissues near a tumor. If your child has proton therapy treatment, it will be at the New York Proton Center. This center is in upper Manhattan.

Your Child’s Comfort During and After DSRCT Treatment

Sarcoma surgery often lasts for many hours. You may worry about how long your child will be under anesthesia. You may also be concerned about how surgery or other DSRCT treatments affect your child’s comfort.

An anesthesiologist (a-nes-THEE-zee-AH-loh-jist) uses anesthesia (medicine to make your child sleep) during surgery. MSK Kids anesthesiologists are all pediatric anesthesiologists, with special training in the care of children. As part of your child’s care team, anesthesiologists help with pain or discomfort related to DSRCT treatments. They will help control your child’s pain.