Strategies for diagnosing and treating sarcomas shift to a more patient-specific approach.

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The New Sarcoma Story

BY BEVERLY A. CALEY
PUBLISHED WEDNESDAY, SEPTEMBER 12, 2007
In August 2001, Erin Toole was a 19-year-old college student working part-time at a kayak rental business. She slipped on some algae and extended her right arm to break the fall. Tremendous pain followed. As an athlete, Toole, of Costa Mesa, California, had experienced sports injuries before and could distinguish between major and minor injuries. So after her first doctor dismissed the pain, she sought another opinion from a doctor who took an X-ray of the painful area. She remembers the “X-ray showed it as clear as day. The best way I can describe it was that it looked like a moth-eaten bone. You could see right through my bone in certain areas.” The doctor referred Toole to a sarcoma center, where a biopsy confirmed a bone cancer called osteosarcoma.

View Illustration: Picking Apart SarcomaSarcomas are a relatively rare group of cancers of connective or supportive tissue, such as bone, cartilage, fat, muscle, and blood vessels, that can occur in both the pediatric and adult populations. The two main kinds of sarcoma—soft tissue and bone—likely encompass more than 600 subtypes that can be hard to tell apart and have different prognoses and outcomes, says George Demetri, MD, director of the Center for Sarcoma and Bone Oncology at Dana-Farber Cancer Institute in Boston.

Based on the tricky and unique nature of sarcomas, doctors are using new tactics to diagnose and treat these tumors that’s more about the patient and less about the general type of sarcoma. And it’s starting to pay off. 

More than 9,000 cases of soft-tissue sarcoma are expected this year plus about 900 cases of bone sarcoma. Since benign lumps are common and sarcoma is uncommon, making a diagnosis of sarcoma can be difficult. The typical family doctor sees, on average, only two soft-tissue sarcomas during his or her career.

Given that they see hundreds of lumps, how can community doctors keep sarcoma on their mental list of potential diagnoses? “Many clues can indicate the presence of sarcoma, but they can be very subtle,” says Dr. Demetri. These clues include the size, firmness, and location of a lump, and its appearance on a CT (computed tomography) scan.

Based on the tricky and unique nature of sarcomas, doctors are using new tactics to diagnose and treat these tumors that’s more about the patient and less about the general type of sarcoma. And it’s starting to pay off.

Margaret von Mehren, MD, director of the sarcoma program at Fox Chase Cancer Center in Philadelphia, says not all patients have the benefit of appropriate imaging prior to surgery. “One thing we see is that patients who have a lump or mass will have either no form of X-ray study or have an inadequate X-ray study, such as an ultrasound, before going for a surgical procedure. Many times, that is not adequate, and better imaging with at least a CT scan, if not an MRI, is indicated.”

Last November, a CT scan revealed a 10-centimeter mass in Jim Foppiano’s mediastinum (the area between the right and left lung that contains the heart). But when a needle biopsy came back indicating the mass was benign, there seemed to be no hurry for surgery. Just before Christmas, surgeons removed a grapefruit-sized mass and sent it to the lab. “A week later is when I actually got the news it was cancer,” says Foppiano, 54, of St. Charles, Missouri.

Improvement in the initial diagnosis of sarcoma will ultimately have to come from education of community doctors, says Dr. Demetri. “When our colleagues in the community recognize that there are 600 different kinds of sarcoma, they’ll begin to say, ‘Why don’t we send the patient to a referral center and then we can work together to tease apart the differences.’ ”

Pathologists at sarcoma centers “have tremendous expertise and very sophisticated tools to enable them to tell these tumors apart from one another—and probably most importantly, from look-alikes that may be benign,” says Dr. Demetri. “We’ve seen people who have been given a death sentence, and they haven’t even had sarcoma. And we’ve seen people who had a sarcoma managed as if it were another sort of cancer.” 

There's an increased focus on quality-of-life issues, which are really important. We try to make sure that the surgery keeps these patients as functional as possible.’

Dr. Demetri envisions a partnership with community doctors where in some cases sarcoma experts provide detail on what they think should be done, but for the more complicated cases, explain why the treatment plan should be implemented at a sarcoma center. “That way, we maintain convenience for patients. Why should a patient drive six hours to get to us for a routine form of chemotherapy when they might be able to get the same care closer to home? However, deciding what is the right form of therapy is the key component.” 

Recent advances in surgery have made it possible to avoid amputation for many patients who have sarcoma in their extremities. Peter Pisters, MD, chief of the sarcoma service in the Department of Surgical Oncology at M.D. Anderson Cancer Center in Houston, says the main advances are improved surgical techniques and increased ability to do more complex procedures that replace joints and blood vessels.

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