When Gene Duncan visited his family doctor for a respiratory infection, little did he realize he’d be diagnosed with bronchitis — and something much more serious.
“While I was there, my heart started to race, says Duncan. “I got dizzy and almost blacked out.”
A cardiologist was consulted to perform a sonogram on the 40-year-old father of four. What he saw was startling. Lodged in the right side of Duncan’s heart was a golf ball-sized tumor. It was crushing the atrioventricular node — a group of muscle fibers that control heart rate.
“I’d been feeling light-headed and weak for a while, but I thought it was due to the hot Kansas summer,” says Duncan, a metal worker from Wichita who worked long hours outdoors making and installing airplane parts.
Months earlier, Duncan had consulted his family doctor about his dizzy spells, but received a clean bill of health.
“My treadmill test was normal and my heartbeat was fine,” he says. “I thought I was OK.”
Elusive and rare
Vinod Ravi, M.D., isn’t surprised by the delay in detecting Duncan’s tumor.
“Cardiac tumors are often missed altogether or misdiagnosed because they’re so rare,” says Ravi, an associate professor of Sarcoma Medical Oncology at MD Anderson.
“Most doctors see only one or two in their entire careers — if even that.”
Ravi partners with Michael Reardon, M.D., a professor of Cardiothoracic Surgery at both Houston Methodist and MD Anderson, to treat patients like Duncan who have primary cardiac sarcomas — malignant tumors that originate in the heart. Only about a dozen cases are recorded each year, according to a National Cancer Institute database.
“By the time these patients come to us, they’re in trouble,” Reardon says. “Their tumors had time to grow and spread because they went undetected for so long.”
Like Duncan, Chriss Schwiderski’s heart tumor eluded medical professionals. Doctors in his hometown of Dallas said the chest pain he was feeling was caused by pericarditis, an inflammation of the sac surrounding the heart.
The condition would resolve on its own, the doctors said.
When Schwiderski returned to the emergency room in severe pain for the fourth time, the perplexed staff ordered an MRI, which revealed a lemon-sized tumor growing in the right chamber of his heart.
The 36-year-old IT consultant was referred to Ravi and Reardon in Houston, who identified his tumor as a cancerous cardiac sarcoma.
Chemo first, surgery second
In the medical arena, Ravi and Reardon are known as the cardiac sarcoma “dream team.” Several years ago they developed a unique protocol that uses unconventionally high doses of chemotherapy to shrink the tumor before surgically removing it. Their approach has doubled survival time and is attracting patients and doctors from around the world.
When Reardon began operating on cardiac sarcomas in 1998, he learned removing them wasn’t easy.
“These tumors are big and bulky,” he says. “Often they’re attached to delicate structures in the heart.”
During a typical six-hour surgery, Reardon meticulously cuts out the tumor, carefully avoiding the heart’s vital network of valves, veins and arteries. Unless he can cut clearly around the entire tumor — achieving what’s known as negative margins — cancerous tissue will be left behind and the tumor likely will grow back.
Only two in five patients achieve negative margins because surgeons are afraid to cut out too much tissue and potentially damage the heart.
Reardon knew there had to be a way to get better results. He consulted Ravi, an expert in the use of chemotherapy, and together they devised their inventive protocol. Here’s how it works:
First, the tumor is biopsied to determine the stage of the cancer and to identify the molecular components inside the tumor. Next, Ravi prescribes chemotherapy to shrink the tumor to a fraction of its original size, making it easier for Reardon to eventually remove. Because no two patients’ tumors are alike, the chemo is tailor-made to target each one’s unique molecular makeup.
Patients get very high doses of chemo, for as long as they can tolerate. It’s a tough regimen, Ravi says.
“We push the envelope to shrink that tumor way down,” he says. “The smaller it is, the likelier Dr. Reardon will get it all out.”
Schwiderski had 32 rounds of chemo over the course of a year. With each successive round, his tumor shrunk a little more.
“It was brutal,” says the physical fitness enthusiast. “I lost 20 pounds of muscle mass. All my hair fell out.”
When his tumor had shrunk to the size of a thumbtack, Reardon was able to remove it completely.
Like Schwiderski, Duncan endured intense chemo, though his lasted almost a year and a half. After surgery with negative margins, he still shows no signs of cancer. He’s gone from a checkup with Ravi every six weeks to once a year.
Even with surgery, most cardiac sarcoma patients can expect their cancer to return somewhere in their body.
“Because their cancer originates in the heart, the cancerous cells are pumped out through the bloodstream to the rest of the body,” says Ravi. “The heart beats 70 times a minute, so it distributes these cells very efficiently.”
In patients whose margins were negative, the cancer usually shows up in the lungs or the brain. Average survival time is two years.
In patients whose margins were not negative, the tumor regrows in the heart. Average survival time is 10 months.
Still, there are exceptions. Duncan’s surgery was five years ago, while another patient Ravi and Reardon call their “greatest success” lived nine years and 10 months.
Surgery buys patients time, they say, but it’s an incomplete solution. The cure, they believe, lies at the cellular level.
The two doctors organized a cardiac tumor group of local physicians and scientists that meets monthly at MD Anderson to discuss advances and brainstorm treatments. Medical school and hospital staffs across the country join in through videoconferencing to share their most challenging cases.
“By sharing knowledge about this rare cancer, we’ll achieve better outcomes faster,” Reardon says.
The team plans to establish an international registry of cardiac sarcoma tissue samples that will be housed at MD Anderson and shared with researchers worldwide. The goal is to study the samples to learn more about the genetics behind these tumors.
“We have an excellent clinical program,” Ravi says. “Our goal now is to become the premier science center for cardiac tumor research.”