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Desmoplastic small round cell tumor (DSRCT) had only been defined as a cancer for 15 years when Andrea Hayes-Jordan, M.D., pioneered a surgery for children with the rare disease. Associate professor in the Department of Surgical Oncology and director of pediatric surgical oncology, her surgery is known as HIPEC (hyperthermic peritoneal perfusion with chemotherapy).
Disease: In North America each year, fewer than 100 patients get DSRCT, which is found in the abdomen. Other tumors, such as rhabdomyosarcoma, a rare, soft tissue cancer originating in the skeletal muscles, may present in the same area.
Treatment: The entire surgery usually takes 10 to 12 hours, beginning with removal of multiple tumors followed by circulating heated cisplatin in the abdominal cavity.
Clinical trial: The success of the Phase I trial allowed for a Phase II trial, which is currently open to those with various tumors in the abdomen. Thus far, 13 pediatric patients have been enrolled on the study.
Results: The Phase I study and retrospective analysis showed that patients receiving HIPEC in addition to surgical resection had an overall three-year survival rate of 71%. For patients who received only standard treatment, the three-year survival rate was 26%.
“In the past, parents were told nothing else could be done, but now we can add months and often years to the lives of these young patients using this surgery. Moving forward, we hope to try different chemotherapies with the procedure to better the outcomes and decrease any toxicities,” Hayes-Jordan says.
The patient: One tough cookie
Madeline Cashion was facing aggressive rhabdomyosarcoma when her surgeon attended a presentation by Andrea Hayes-Jordan, M.D., associate professor at MD Anderson Children’s Cancer Hospital, on a special surgery she was performing in Houston.
Less than a month later, the family left their home in California to find out more about hyperthermic peritoneal perfusion with chemotherapy, better known as HIPEC.
“We just hit it off with Dr. Hayes-Jordan immediately. We felt like she had the team for us, and that we were a part of the team,” says mother, Rachelle Cashion.
Originally diagnosed at the age of 4 in September 2010, Madeline underwent chemotherapy and radiation for a year and was declared cancer-free on her first day of kindergarten.
Then, another lesion appeared in March 2012. After the second treatment protocol failed to keep Madeline’s tumor in remission, the Cashions and their MD Anderson team moved swiftly to perform the surgery on April 8.
“With the tumor presentation, we had concerns going into HIPEC because of how Madeline fared in two other surgeries,” Rachelle says. “But we found comfort in Dr. Hayes-Jordan’s experience and reputation for taking the time to do a thorough job.”
While recovering from a 12-hour surgery, 7-year-old Madeline spent close to three weeks in the hospital honing her artistic skills. Her main goal is to get well enough to again take up horseback riding, her favorite hobby. Each day, her progress was closely monitored and celebrated with Hayes-Jordan, who even knew that Madeline was an artist.
“This care and connection to Madeline, as a patient and as a child, is what makes Dr. Hayes-Jordan an incredible surgeon,” Rachelle says. “Madeline is one tough cookie. She’s got a great spirit and what some might say a sense of flare.”