Ever treat a patient diagnosed with sarcoma? If not, you certainly aren’t the only medical professional. Often referred to as the “forgotten cancer,” sarcoma is relatively rare — and, because it can present with no symptoms at all, often deadly.
In honor of Sarcoma Awareness Month this July, here are eight things that may surprise clinicians about sarcoma.
There are over 100 types of sarcomas.
Sarcoma isn’t just one disease: It’s a collection of at least 100 different types of cancerous tumors. Sarcomas can arise from a variety of tissue structures—including nerves, muscles, joints, bone, fat, and blood vessels—found everywhere in the body, notes Vivek Subbiah, M.D., an associate professor of medicine and the clinical medical director at the Center for Targeted Therapy, Division of Cancer Medicine, at MD Anderson Cancer Center in Houston, TX.
Sarcoma is more common in children than adults.
Sarcoma is a relatively rare disease, accounting for just 1 percent of all cancers in adults. In children under the age of 20, however, it accounts for somewhere around 15 percent of all cancers. Between 1,500 to 1,700 children are diagnosed with a bone or soft tissue sarcoma in the U.S. every year, according to the National Foundation for Cancer Research.
Many doctors have never seen or treated a patient with sarcoma.
Because sarcoma is such a rare disease, “there might be times when oncologists see one or two cases every year,” says Subbiah. Patients are best served at a major cancer center, he adds, as treatment requires a multidisciplinary team of medical, surgical, radiation, and pediatric oncologists.
Even if a patient is receiving treatment from an oncologist at a major center, it’s a good idea to get a second opinion about the initial diagnosis and treatment plan from a doctor who specializes in sarcoma.
The causes behind sarcoma are still a mystery.
There is no single cause behind sarcomas. With so many sub-types, the disease is all the more challenging to nail down; however, there do seem to be some genetic variations specific to sarcomas, notes Subbiah. In addition to certain genetic conditions, exposure to some chemicals may also increase the risk of sarcoma in some people.
Radiation for another cancer can lead to sarcoma.
In the 1900s, doctors began to use radiation to treat noncancerous medical issues. They noted that patients developed sarcoma when radiation was delivered in high doses. Today, radiation is carefully targeted to tumors, so that sarcomas are radiation-induced in about 5 percent of cases, reports the NFCR.
Still, “most patients who undergo radiation have a risk to develop sarcoma,” Subbiah says. Patients who receive radiation to treat another type of cancer occasionally develop a sarcoma up to 10 years later. That’s one reason oncologists closely follow their patients with regular scans.
Sarcomas may not have any noticeable symptoms.
In the early stages of sarcoma, patients may not have any symptoms at all — and if they do, it’s usually a painless lump. Much more rarely, they might experience pain, vomiting, nausea, diarrhea, and blood loss. The only way to diagnose a sarcoma is with a biopsy.
“A lot of bumps are benign, but they should always be looked at by a doctor,” Subbiah says.
Once sarcoma is diagnosed, it’s often too late to be treated.
“The challenge is that sarcoma is not one disease but multiple subtypes that can come from a variety of tissues anywhere in the body,” says Subbiah. “It’s commonly hidden inside the body, so it’s often diagnosed when it’s already too large to expect to be cured.”
Diagnosed early enough, sarcoma is sometimes curable by surgery alone. Other times, amputation may be necessary (though amputations are increasingly rare). Patients also receive radiation and chemotherapy before and after surgery to kill any remaining cancer cells.
If sarcoma has already spread to other parts of the body, however, chemotherapy can reduce pain and discomfort but is unlikely to completely eradicate the disease.
“The important thing is diagnosis. The earlier we detect, the better,” says Subbiah.
Future treatments depend on understanding the causes behind.
Subbiah, who ran a successful clinical trial for Radium-223 as a treatment for relapsed osteosarcoma, says that the key to future research is to identify the genetic drivers behind sarcoma and the molecular abnormalities of these rare tumors. Researchers hope to develop screenings and customized therapies to treat the many various subtypes of sarcoma — but only with the support of a multi-institutional, collaborative platform.
“It’s a challenge,” says Subbiah. “As we look forward for novel therapies to conquer this disease, we want to understand why people get it in the first place. We need to understand the molecular underpinnings to develop customized therapies.”