UNC Health Talk

A Destination for Treatment of a Rare Pediatric Cancer

There is a rare cancer that can produce hundreds, or even thousands, of tumors throughout the abdominal cavity. It’s called pediatric desmoplastic small round cell tumor, or DSRCT. Since the condition was first recorded in 1989, there have only been about 200 cases worldwide.

Even rarer than this aggressive cancer, however, are the physicians who can treat it.

The oncologist who pioneered the pediatric treatment for DSRCT is Andrea Hayes-Jordan, MD, chief of pediatric surgery at the UNC School of Medicine and surgeon in chief at N.C. Children’s Hospital.

“Before I started treating this disease, it was thought to be hopeless and many physicians would not even offer treatment to these children,” Dr. Hayes-Jordan says. “What I have discovered through my research over the last 13 years is that you can treat these children, you can help them live longer and in some cases you can even cure them.”

Dr. Hayes-Jordan was the first surgeon to use hyperthermic intraperitoneal chemotherapy, or HIPEC, to treat pediatric patients with this cancer; previously, it had been used only in adults. HIPEC delivers chemotherapy drugs that have been heated to over 100 degrees directly to the abdomen.

First, patients undergo a lengthy surgery—up to 20 hours—to remove the hundreds or even thousands of tumors that have grown throughout the abdomen. Then, Dr. Hayes-Jordan administers HIPEC surgically into the abdomen, killing any cancer that may have been missed or that was too small to see.

And it works.

“We have improved the survival rate for children with this condition from 15 to 30 percent to 60 percent over the last five years,” Dr. Hayes-Jordan says. “And, whereas 10 years ago this was considered an experimental procedure, it’s now the standard of care.”

Even so, Dr. Hayes-Jordan is the only surgeon in the Southeast who performs it, so her patient base extends across the country and even internationally. (Watch a video about 12-year-old Sebastian Gonzalez, who traveled from Costa Rica to be treated by Dr. Hayes-Jordan.)

“I have doctors and surgeons from around the world calling to ask me to teach them the surgery or to consult with them about a patient who might qualify,” Dr. Hayes-Jordan says.

In addition to treating this disease, Dr. Hayes-Jordan is leading the scientific charge to understand where it develops, which is still largely a mystery. In her lab, she has created mouse models that have produced clues about where this cancer begins and how it spreads. Her research is inspired by what she has seen in the operating room.

“I can make an observation in my patients and then go back to my laboratory and try and figure out how that works into that disease process and how we can either aggregate that or capitalize on that finding to help a child live longer,” Dr. Hayes-Jordan says. “Having the opportunity for my lab work to complement the work with patients in the operating room really allows me to move forward the understanding of this rare condition.”

UNC Children’s specializes in treating all kinds of pediatric conditions. Visit UNC Children’s for more information about care and treatment options.