The dream team vs. rare heart tumors

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.”

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.

Tough regimen

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.”

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.

Read more about the work of Drs. Ravi and Reardon in MD Anderson's Conquest magazine.