The Philadelphia Flyers’ Oskar Lindblom is being treated for Ewing sarcoma at a time when the outlook for young people with the ultra-rare bone cancer is better than ever.
The hockey team revealed the diagnosis last month but has not disclosed the stage or location of the cancer that has sidelined the 23-year-old left winger. He is receiving care at the University of Pennsylvania.
But for patients whose cancer has not spread beyond the site where it originated, cure rates now approach 80%, up from a dismal 10% in the 1960s. Advances in reconstructive surgery mean most patients can be spared amputations — once the only option. And therapies that target the molecular drivers of Ewing sarcoma are on the horizon.
“I feel like it’s an exciting time right now,” said Patrick J. Grohar, a pediatric oncologist and sarcoma researcher at the Children’s Hospital of Philadelphia. “I believe there will be a new targeted drug certainly in the next decade, and maybe in the next five years.”
Studies and anecdotal cases point to another sign of progress: Athletes can resume sports — including high-impact sports — in many instances. One notable example is Mark Herzlich, the Wayne, Pa., native who was diagnosed with a Ewing’s leg tumor while at Boston College in 2009. He returned to play football there in his senior year, and then had a six-year NFL career, including a Super Bowl win, with the New York Giants.
“Before his story, I would have thought it was unreasonable for anyone to return to professional sports with a diagnosis like this,” said Scot Brown, chief of orthopaedic oncology at Rothman Orthopaedics Institute.
One in a million
Ewing sarcoma is named for James Ewing, the Pittsburgh-born pathologist who first described the distinctive tumor cells in the 1920s. The malignancy is believed to arise with a primitive stem cell that turns into a renegade instead of making connective tissue such as bone and muscle.
About 250 people, most between ages 10 and 20, are diagnosed with Ewing every year in the U.S. — less than one in a million. The most common sites are the pelvis, hip, and leg bones, although any bone and adjoining soft tissue can be involved.
Even when imaging shows that the disease is limited to one limb, microscopic cancer cells may have traveled through the blood to seed the lungs or other organs. That’s why survival started to improve in the 1970s, when patients who relapsed began to be given chemotherapies that killed such micrometastases.
The current standard protocol is an aggressive mix of five intravenous cell-killing drugs given over about seven months before and after surgery. The presurgical regimen evolved partly because of the advent of limb-sparing operations.
“When doctors first attempted limb salvage surgery, they didn’t have premade implants available to make up for lost bone,” Brown at Rothman explained. “The implants would take months to manufacture. Having patients waiting with a potentially deadly disease, doctors started using chemotherapy before surgery. That led to much better survival.”
Today, technology has transformed how reconstructive surgery is planned and carried out. The advances include three-dimensional imaging, microsurgical techniques that preserve tiny blood vessels and nerves, and prefabricated cobalt chrome bone replacements that can be tailored to the patient and connected to muscles. Biological reconstruction, which uses grafts of the patient’s own bones and tissues, also has come a long way.
Even with these innovations, amputation is occasionally the best choice. Brown recalled a challenging case in which a teenager and her parents opted for below-the-knee amputation and a prosthetic limb because surgery was likely to leave her with permanent ankle disability.
“Despite that, she’s competed in gymnastics and I continue to get videos of her,” he said. “Prosthetics have advanced so far. There are different prosthetics for different activities.”
Of course, the physical and psychological resilience of youth also helps. Austrian researchers followed 30 Ewing patients, average age 18, after reconstructive surgery involving the pelvis or a leg bone. Five years after treatment, 25 of them (83%) were regularly doing athletic activities such as biking, swimming, soccer, tennis, and skiing.
Rachel Kovach, of Highlands, N.J., was just 11 years old when she underwent chemotherapy and surgery to replace her right thigh bone. Not only did she return to her passion — competitive swimming — but now, nine years later, she is majoring in biomedical engineering.
“I wouldn’t be geared to the major and career I’m headed for if not for Ewing’s,” said the College of New Jersey junior.
Herzlich’s football comeback also inspired her.
“I met him as part of my Make-a-Wish,” Kovach said, referring to the nonprofit that grants special requests of critically ill children.
Radiation instead of surgery
Unlike some other bone cancers, Ewing tumors are very sensitive to radiation. Radiotherapy may be added to chemotherapy and surgery, especially if all the cancer can’t be removed. Or, radiation may be used instead of surgery if operating would be too dangerous.
As Herzlich and his parents discovered, the choices are fraught with life-and-death implications. According to news reports, he was told that surgery to reconstruct his thigh bone would very likely be curative — but end his football career. If he had radiation instead of surgery, he could probably preserve his athletic prowess, but there was a 50-50 chance the cancer would not be eradicated, even with chemotherapy.
He decided to forgo surgery.
“I needed to get better but I also knew if I wanted to be myself I had to be able to play football,” he was quoted as saying by ESPN.com in 2011. “Those two goals went hand in hand.”
In the coming years, drugs that target the molecular abnormalities in Ewing’s tumor cells may lessen the dilemmas and toxicity of treatment, as well as offer new hope for patients who have metastatic disease or a relapse. A number of experimental drugs are in early testing. And some targeted drugs that are already approved for other cancers, including Avastin, Afinitor, and Lynparza, are being studied as possible Ewing therapies.
Grohar, at CHOP, is about to launch human testing of two already approved chemotherapies, trabectedin and irinotecan, because his lab research suggests that the combination can inhibit a crucial mutation in Ewing sarcoma. Trabectedin has also been shown to have other targeted and immune-boosting effects.
Although Grohar isn’t involved in Lindblom’s treatment at Penn — CHOP’s next-door neighbor in West Philly — the physician is among the legions rooting for the hockey player.
“I’m a huge hockey fan,” Grohar said. “My son plays youth hockey for the Delco Phantoms, and Oskar came up in the Lehigh Valley Phantoms,” the Flyers’ minor league affiliate.