July is Sarcoma Awareness Month, making it an appropriate time to spread information about this disease and medical advancements surrounding its treatment. The Sarcoma Alliance estimates there are about 14,000 new diagnoses of sarcoma every year. While it is a relatively rare cancer, researchers and physicians continue to try and better understand why it develops and how to treat patients with the its various subtypes.
Sarcoma is a broad term for a type of cancer that creates malignant tumors in bone and soft tissue. Soft tissue encompasses a number of areas of the body, including muscle, fat, nerves, deep skin tissue, and blood vessels. There are more than 50 separate types of soft tissue sarcomas with unique names for each type of cancer that develops in each of these tissues. There are three major types of bone sarcomas. Some bone and soft tissue sarcomas are predominately found in children, and both types can develop anywhere in the body, though soft tissue sarcoma is seen most often in the arms and legs.
The ability to detect and diagnose sarcoma early can increase a person’s likelihood of successful treatment and remission. People need to be aware of what’s normal and what’s not normal for their bodies. Symptoms that should send someone to a physician right away include:
- A lump on their body. Sarcoma regularly forms on the arms and legs, but the lump could be anywhere. It doesn’t have to hurt to be a malignant tumor, though it may cause discomfort or pain.
- Abdominal pain. This will only worsen over time, it does not subside.
- Blood in stool or vomit. This can indicate internal bleeding.
- Black, tarry stools. Blood from the stomach or bowels turns black during digestion.
The most common signs of bone sarcoma are pain, swelling, and tenderness in the joints. The cancer can also weaken the bones causing individuals to break bones when they normally wouldn’t.
Anyone with a family history of sarcoma should speak with their physician about genetic testing or at least being on the lookout for signs. Genetic testing can expose mutated genes that increase the likelihood of this type of cancer. However, this test has its limits, and it may not be covered by insurance. The role of genetic testing in diagnosing a variety of diseases is evolving within the current health care landscape, and in future years, it may be more commonly performed and covered by insurers.
Individuals without any family history but with certain risk factors like previous radiation treatment or exposure, issues with their lymph system, and exposure to some chemicals should also speak with their physician about warning signs and possible early detection.
Patients can expect to undergo imaging tests like X-rays, MRIs, CTs, and PET scans to determine if they have a tumor and if it is malignant. To determine if the tumor is cancerous, physicians remove a sample of the tissue for a biopsy. This can be a separate procedure or the sample can be taken during surgery to remove the tumor.
Like with many cancers, the basic treatment for sarcoma starts with some combination of surgery, chemotherapy, and radiation. Surgery to remove sarcoma is highly common, which can lead to amputation when the tumor is in a limb. Some physicians recommend chemo, targeted drugs, and/or radiation prior to surgery in the hopes of reducing the size of the tumor, which is one way to avoid amputation. Depending on the type of sarcoma, drugs targeted toward the specific type of malignant cell may be used to block the tumor from growing.
The 5-year survival rate for sarcoma patients heavily depends on the type of soft tissue sarcoma initially discovered. For individuals who were diagnosed with localized sarcomas, there is an 83 percent 5-year survival rate, according to information from the National Cancer Institute. A localized cancer has not spread beyond a specific areas of the body. Individuals with regional stage sarcoma have a 54 percent 5-year survival rate. Regional stage means the cancerous cells have spread into the lymph nodes. Individuals with distance spread, which means the cancer has metastasized into other areas of the body, have a 5-year survival rate of 16 percent.
Other organizations will measure survival rates differently, such as based on the stage of cancer and not its spread at the time of diagnosis. Overall, individuals with early stages of sarcoma in the arm and legs have a high rate of survival over a 5-year period. However, survival rates for more rare sarcomas are lower.
In March 2015, the U.S. Food and Drug Administration approved Zarxio, the first biosimilar version of the biologic cancer drug Neupogen, which is used to treat a specific type of sarcoma. Biologic drugs are formed using living cells to target specific cancer cells. Biosimilar drugs are to biologics as generics are to brand name drugs – almost. Many generics are exactly the same as the brand name, while a biosimilar drug is close, but not identical to the original biologic. The approval of Zarxio means people with rare forms of sarcoma can receive a cheaper medication to treat their illness.
Researchers are also developing more targeted chemotherapy and drugs, anti-angiogenesis agents that stop blood vessel growth, proton therapy, and other treatment methods to improve the long-term survival rate of patients diagnosed with sarcoma.
How we make a difference
Every year Advanced Medical Reviews receives independent medical reviews on sarcoma patients and thereby plays a vital role in deciding the medical necessity of the treatment options for this rare type of cancer including proton beam therapy, intensity modulated radiation therapy and stereotactic body radiation therapy. AMR’s oncology panel reviewers also give their expert opinions on the latest and new experimental drugs in the market for sarcoma.