According to the American Cancer Society (ACS), about 12,000 people (less than 1 percent of cancer cases) will be diagnosed with sarcoma in 2016.
In sarcoma, the cancerous cells form in the soft tissues of the body, which include: muscles, tendons, fat cells, blood vessels, lymph vessels, nerves and tissues around joints.
Therefore, sarcoma can be found anywhere in the body. Unlike the more common carcinomas that arise from carcinogenic exposure (i.e. diet, smoke, UV light), the deep tissues develop malignancies that arise spontaneously.
Malignant fibrous histiocytoma is found in the tendons and ligaments, usually in the arms, legs or trunk.
Liposarcoma is located in the fatty tissue, typically in the arms, legs or body cavities.
Leiomyosarcoma originates in the involuntary muscle (smooth muscle), such as that found in the uterus and digestive tract.
In the past, limb amputation was a common way to treat sarcomas in the extremities. Today, according to the ACS, less than 5 percent of surgeries to treat the disease result in amputations, with the majority of patients undergoing surgery to remove the tumor only with a follow-up regimen of radiation and chemotherapy.
Depending on the stage of the sarcoma, patients may use chemotherapy as a first line treatment or in addition to surgical removal. The most common chemotherapeutic treatments for the disease are: Ifosfamide and doxorubicin; cisplatin; dacarbazine; docetaxel; gemcitabine; methotrexate; oxaliplatin; paclitaxel; vincristine; and vinorelbine.
Typically, your oncologist will base your treatment on a standard protocol. With so many options, it would be advantageous to have your tumor tested to get your therapy right the first time with the Ex-Vivo Analysis of Programmed Cell Death (EVA-PCD) functional profiling platform from Nagourney Cancer Institute.
One of the most interesting aspects of sarcoma biology is the recognition of highly specific mutations. Some of these mutations may render sarcomas sensitive to specific forms of therapy. One such therapy is the use of Imatinib in the treatment of gastrointestinal stromal tumors, also known as GIST.
Additional targets for these diseases are the signaling pathways targeted by some of the newest classes of drugs. Many of these agents are under investigation at Nagourney Cancer Institute and can often be tested for activity when tumor tissues are submitted.
By the time Brittany Wnek was 16, she had been diagnosed with mast cell sarcoma, had already experienced 10 craniotomies and 13 rounds of chemotherapy, not to mention radiation. Still facing more surgery and therapies to try and reduce tumor growth, her family sought help from Rational Therapeutics (now Nagourney Cancer Institute).