Cytoreductive Surgery for DSRCT

Cytoreductive (sigh-toe-ree-DUCK-tiv) surgery (CRS) is also called debulking surgery. It is a cancer surgery to reduce the number and size of cancerous tumors in the belly.

Most children who undergo this surgery have many small tumors inside the tissues lining the belly and pelvis area. This area is very close to vital organs.

The goal of CRS is to remove as much of the visible cancer as possible. CRS can be paired later with another type of treatment (usually chemotherapy) to finish killing off the remaining cancer cells.

After cancer has been debulked, it can become more vulnerable to other treatments. That means chemotherapy has a better chance of being successful and completely removing the cancer.

CRS aims to increase life expectancy by removing cancerous tumors in children with certain cancers, including:

• Desmoplastic small round cell tumors (DSRCT).
• Other cancers that have metastasized (meh-TASS-ta-sized) or spread to the tissues lining the belly.

On its own, cytoreductive surgery can:

• Increase life expectancy.
• Increase quality of life.
• Reduce cancer cells.
• Reduce the rate of tumor growth.
• Reduce symptoms.

In rare instances, this surgery alone can cure children of their cancer. But it’s usually combined with another therapy to remove microscopic cancer cells left behind after CRS.

Cytoreductive surgery for desmoplastic small round cell tumors

Desmoplastic (dez-moe-PLAZZ-tick) small round cell tumor (DSRCT) is a rare but aggressive form of childhood and young adult cancer that shows up as multiple small tumors in the belly.

Its name comes from what the cancer cells look like when studied under a microscope:

• Desmoplastic – The tumor is surrounded by fibrous tissue.
• Small round cell – The size and shape of the cancer cells are small and round.

DSRCT is caused by two specific genes – EWS and WT1 – that fuse together abnormally. This incorrect fusion creates abnormal proteins that produce cancerous cells. DSRCT usually starts in the peritoneum (pare-it-tun-NEE-um), which is the tissue that lines the belly beneath the skin.

It is common for these tiny tumors to have multiplied by the dozens or hundreds by the time a DSRCT diagnosis is made. They spread easily throughout the belly and to other vital organs, including the liver and lungs.

DSRCT has a low long-term survival rate. About 15% to 25% of children will live five years after initial diagnosis. Still, CRS can help reduce the number of tumors in the body. And, when paired with other therapies, it can often remove cancer completely.

How common are desmoplastic small round cell tumors?

DSRCT is so rare that there are currently no accurate studies on how many children are diagnosed each year.

Is cytoreductive surgery right for my child?

Cytoreductive surgery may be right for your child if they have been diagnosed with DSRCT or a similar cancer that has spread into the cavity of the belly and needs to be removed.

Your child’s care team will decide if CRS is a good option for your child. They may put together a treatment plan that includes surgery and other therapies to treat your child’s cancer.

Before surgery

Depending on your child’s overall health and diagnosis, they may receive some chemotherapy to shrink the tumors before surgery.

• Chemo before surgery is called neoadjuvant (nee-oh-ADD-joo-vunt) therapy.
• The goal of neoadjuvant chemotherapy is to shrink the tumors to make them easier to remove during surgery and to lower the chance of the cancer coming back.

For CRS, your child will be given general anesthesia and will be asleep for the entire operation. Cytoreductive surgery is complicated and involved. It can last up to 12 hours, depending on the severity and location of tumors.

Because your child is asleep for this surgery, they will wake up with a catheter tube in place connecting their bladder to a bag that will collect their urine.

During surgery

Once your child is asleep, the surgeon will make the incision(s) in their abdomen.

The size of the incision(s) will depend on how much of the abdomen is involved with tumor deposits.

Your child’s surgeon will explain the exact method they plan to use before you consent to the surgery.

• Using various methods, the surgeon will destroy, burn off, and remove as many small tumors and cancer cells as possible. Depending on the cancer stage and organs affected, they may also remove other tissues or organs.
• The goal of cytoreduction surgery is to remove all visible cancerous tumors in the belly cavity.
• Sometimes, surgeons use hyperthermic (hi-purr-THUR-mick) intraperitoneal (in-tra-pare-it-tun-NEE-ull) chemotherapy (HIPEC) after CRS. HIPEC is a type of advanced regional perfusion cancer therapy.
• During HIPEC, surgeons soak the belly cavity in very high doses of heated chemo to kill any invisible cancer cells that might be left behind after CRS.
• If your child’s care team has recommended HIPEC, your child will be given heated chemotherapy drugs directly into the abdomen during the surgery, after the cytoreduction is complete. The chemo is drained from the body after a certain amount of time.
• Then, the surgeon will close the incision(s) and cover the wound(s) with bandages.

After cytoreductive surgery

Your child will likely have belly soreness for the next few days. You can expect your child to be in the hospital for one or two weeks, depending on their cancer status and recovery from surgery.

It usually takes a few months for children to recover completely from CRS and return to normal activities.

Complications of cytoreductive surgery

In follow-up appointments with your child’s surgeon, mention any issues, changes, symptoms, or difficulties your child is experiencing. You can expect your child to be monitored and tested after surgery to determine the success of the operation and the state of your child's cancer.

Complications from CRS can include:

• Blood clots.
• Breathing problems.
• Excessive bleeding.
• Fever.
• Heart problems.
• Infection.
• Kidney injury, especially if HIPEC is also performed.
• Sepsis.
• Shock.

Get emergency treatment immediately if your child has any of these changes or severe symptoms of any kind.

When general anesthesia is needed, there are important rules for eating and drinking that must be followed in the hours before the surgery.

One business day before your child’s surgery, you will receive a phone call from a nurse between the hours of 1 and 9 p.m. (Nurses do not make these calls on weekends or holidays.) Please have paper and a pen ready to write down these important instructions.

The nurse will give you specific eating and drinking instructions for your child based on your child’s age.

The following are the usual instructions given for eating and drinking. No matter what age your child is, you should follow the specific instructions given to you on the phone by the nurse.

For children older than 12 months

• After midnight the night before the surgery, do not give any solid food or non-clear liquids. That includes milk, formula, juices with pulp, coffee, and chewing gum or candy.

For infants under 12 months

• Up to six hours before the scheduled arrival time, formula-fed babies may be given formula.
• Up to four hours before the scheduled arrival time, breastfed babies may nurse.

For all children

• Up to two hours before the scheduled arrival time, give only clear liquids. Clear liquids include water, Pedialyte®, Kool-Aid®, and juices you can see through, such as apple or white grape juice. Milk is not a clear liquid.
• In the 2 hours before the scheduled arrival time, give them nothing to eat or drink.

Once your child has been registered for the surgery, a member of the anesthesia staff will meet with you to take your child’s vital signs, weight, and medical history.

A pediatric anesthesiologist — a doctor who specializes in anesthesia for children — will meet with you before the surgery. They will ask questions about your child’s health and about when your child last ate.

Because your child may have eaten more recently than is usually advised for surgery, the anesthesiologist may make adjustments to your child’s anesthesia. Once admitted, your child will not be able to have anything to eat or drink until after the operation.

• As the parent or legal guardian, you will be asked to sign a consent form before the anesthesia is given.
• If your child is very scared or upset, the doctor may give a special medication to help them relax. This medication is flavored and takes effect in about 10 to 15 minutes.
• If you wish, you may be allowed to go with your child to the room where the surgery will be done and stay while the sleep medication is given.
• Younger children will get their sleep medication through a “space mask” that will carry air mixed with medication. Your child may choose a favorite scent to flavor the air flowing through the mask. There are no shots or needles used while your child is still awake.
• Older children may choose between getting their medication through a mask or directly into a vein through an intravenous (IV) line.
• When your child has fallen asleep, you will be taken to the waiting room. If it has not already been done, an IV will be started so that medication can be given to keep your child sleeping throughout the surgery.

While your child is asleep, their heart rate, blood pressure, temperature, and blood oxygen level will be checked continuously. Your child will have a breathing tube placed while they are asleep. Your child might have a sore throat after the surgery.

To keep your child asleep during the surgery, they might be given anesthetic medication by mask, through the IV tube, or both. When the surgery is over, the medications will be stopped, and your child will begin to wake up.

After surgery, your child will be moved to the recovery room to allow the anesthetic to wear off. You will be called so that you can be there as they wake up.

• Children coming out of anesthesia react in different ways. Your child might cry, be fussy or confused, feel sick to their stomach, or vomit. These reactions are normal and will go away as the anesthesia wears off.
• While your child is in recovery, your surgeon will talk to you about the surgery. This is a good time to ask questions about pain medications, diet, and activity.
• When your child is awake enough, they might be given a Popsicle® or “slushy” to drink in the recovery room.
• Once the anesthesiologist sees that your child is recovering and their pain is controlled, they will be moved to their hospital room. This usually takes about an hour, but every child is different, so times may vary.

The most important role of a parent or guardian is to help your child stay calm and relaxed before the surgery. The best way to help your child stay calm is for you to stay calm.

• You are encouraged to talk to your child or hold their hand before the surgery, while sleep medication is given, and while in recovery.
• You may bring along a “comfort” item — such as a favorite stuffed animal or “blankie” — for your child to hold before and after the surgery.

During the surgery, your child will be given a numbing medication directly into the incision to relieve discomfort after the surgery.

• After the surgery, your child will most likely have belly pain.
• Your child may take Tylenol® or Motrin^®.
• If your child is still having pain, your child’s doctor may prescribe a stronger medication or a narcotic.
• Before you go home, your doctor will prescribe pain medication for your child.

After your child is discharged and goes home, they should avoid strenuous activity for the next two to four weeks.

Bathing

Your child may take a shower one to three days after the surgery. Baths are allowed five to seven days after surgery.

Activity

Following the surgery, your child's activity needs to be somewhat restricted.

• Your child's surgeon will determine when they can resume normal activities.
• Your child may return to school when they feel up to it.
• Participation in contact sports and gym class is not permitted until cleared by the surgical team (typically two to four weeks after surgery).
• Swimming is permitted one to two weeks after surgery.
• Walking and stair climbing are encouraged.

If your child has a fever higher than 101.5 F, bleeding, or foul-smelling drainage from the area around the incisions, call your surgeon right away.

Also, call the doctor if your child has:

• Increased or unexplained pain.
• Increased swelling or redness at the incision site.
• Increased tenderness near the incision.
• Nausea, vomiting, diarrhea, or constipation that is not improving.

If you have any specific questions about your child’s surgery, you should discuss them with the surgeon before the surgery. After the surgery, you may call the Division of Pediatric General and Thoracic Surgery at UPMC Children’s and ask to speak with your child’s surgeon or speak with them during the follow-up visit, if needed.

If your child has any special needs or health issues you feel the doctor needs to know about, please call the Division of Pediatric General and Thoracic Surgery at UPMC Children's Hospital of Pittsburgh before the follow-up visit and ask to speak with a nurse. It is important to notify us in advance about any special needs your child might have.

At UPMC Children’s Hospital of Pittsburgh, every child diagnosed with desmoplastic small round cell tumors or peritoneal cancers receives an individualized treatment plan and family-centered care.

• Cytoreductive surgery for rare abdominal cancers can be incredibly complicated. Our surgeons at UPMC Children’s have trained at the nation’s leading cancer centers and are among the country’s most experienced in pediatric cytoreductive surgery. We collaborate with UPMC surgeons who perform CRS in adults, as UPMC is one of the highest-volume CRC centers in the world.
• In addition, cutting-edge research and the latest technology ensure our patients' best possible outcomes.