Mount Sinai
Dr. Robert Maki is chair of the Sarcoma Program and Pediatric Hematology-Oncology at Mount Sinai Hospital. He specializes in treating patients who have sarcomas, cancers of the connective tissue. He has been working in the field for 30 years.
The Specialist
As the chair of the Sarcoma Program and Pediatric Hematology-Oncology at Mount Sinai Hospital, Dr. Robert Maki specializes in treating patients who have sarcomas, cancers of the connective tissue. He has been working in the field for 30 years.
WHO’S AT RISK
July is sarcoma awareness month, a push to bring attention to a rare type of cancer that accounts for less than 1% of all cancers in adults but 15% of cancers in children. “Sarcoma is nature’s proof that you can get cancer anywhere, from the scalp down to the toes,” says Maki. “It’s defined as a cancer that arises from the stuff that holds us together, be it bone, cartilage, fat, or muscle.” The hip and thigh are the places where people most commonly develop sarcomas.
There doesn’t seem to be any clear underlying cause for sarcoma. “Recent data suggests that many cancers are simply due to bad luck and spontaneous events, and that’s especially true of sarcomas,” says Maki. “For a small fraction of people, sarcoma can be tied to receiving therapeutic radiation — it’s estimated that perhaps one out 500 people who receive radiation for cancer will later develop a sarcoma about a decade down the road.”
Another small subset of people are genetically predisposed to developing sarcomas. “There is a genetic trait tied to family disorders, but these are very rare syndromes,” says Maki. “However, it’s very serious if you do have those genetics, so we’re now trying to screen for these genetic problems.”
Sarcomas can arise during any phase of life. “Different types of sarcomas are more likely to affect different age groups,” says Maki. “In older people, the sarcomas can be due to accumulated genetic mutations, while children are more likely to have a single genetic event.”
In contrast to many familiar cancers, sarcomas don’t seem to be the result of exposure to toxins. “Unlike the association between sunlight and melanoma, smoking and lung cancer, or diet and colon cancer, sarcomas aren’t a question of environmental exposure,” says Maki. “We all have many genetic mutations in all of our cells every day, but some people happen to get those mutations in a gene that triggers the cancer to form — it’s a matter of genetics and bad luck.”
SIGNS AND SYMPTOMS
Sarcoma is one cancer that usually presents as a visible change. “They really do come out of the blue, usually just as a painless but growing lump,” says Maki. “These lumps develop over the course of weeks or months, depending on how aggressive they are.”
Any unexplained lump on your body is worth consulting a doctor about. “We’ve taught people well that if they have a breast lump, they need an examination, but if you have a lump somewhere else on the body, that should also be examined,” says Maki. “Chances are that the lump is something benign, such as a lipoma, but any lump that is growing is a matter of particular concern. It’s really a call to be aware of your body.”
TRADITIONAL TREATMENT
A biopsy is necessary to confirm the diagnosis of sarcoma. “We take a core sample of the tumor with a special needle and the pathologist looks at it,” says Maki. “It really helps to have a sarcoma expert look at the tissue directly, because there are more than 50 types of these tumors, and your treatment plan will depend on which type you have.”
The primary treatment for sarcoma is surgery. “The idea is to cut the tumor out completely with a clean margin — you want the tumor to come out in one piece without any residual cells left at the edges,” says Maki. “In many cases, surgery can be curative, but larger tumors may also require radiation to prevent recurrence.” Only a fraction of sarcomas benefit from chemotherapy.
Unlike the association between sunlight and melanoma, smoking and lung cancer, or diet and colon cancer, sarcomas aren’t a question of environmental exposure.
Early detection is the key to improving outcomes. “Don’t ignore any lump on your body that’s growing, because the smaller we can catch these things, the better,” says Maki. “The risk that a tumor will come back elsewhere in the body is 50% if the tumor is three inches in size — there’s a lower chance that the cancer will spread if the tumor is smaller.”
RESEARCH BREAKTHROUGHS
Sarcoma research is producing better and better treatment options. “Even within the past few years, there are an increasing number of types of therapy available,” says Maki. “In particular, immunotherapy and epigenetic treatments are two very promising lines of research.”
QUESTIONS FOR YOUR DOCTOR
If you are diagnosed with sarcoma, one of the first questions to ask is, “What treatment is necessary for me to have the best chance of a cure?” Followed by, “Do I need radiation or chemotherapy?” Don’t be shy about asking, “What will my life be like based on this treatment plan?” “Sarcoma is a complex cancer, and most of the treatment will involve more than one doctor,” says Maki. “There is not one-size-fits-all approach to treating sarcoma — the key is to figure out for each person what the best customized treatment plan is.”
WHAT YOU CAN DO
Get informed.
Places to start your online search include the American Society of Clinical Oncology’s patient website (cancer.net), the Sarcoma Foundation of America (curesarcoma.org), and Mount Sinai (mountsinai.org/patient-care/service-areas/cancer/cancer-services/sarcoma-cancer-program).
Don’t ignore lumps growing on your body.
If you notice that something on your body is changing, it’s worth consulting a health care professional.
Get a second opinion.
Maki recommends consulting an expert who specializes in sarcoma, “especially when it comes to the pathology review and coming up with a treatment plan-simply because, even among experts, there will be a variety of opinions.”
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