If you see yellow ribbons around your community during the month of July, it may mean something different than the usual “Support Our Troops” many think of when first sighted. Instead, they could be displayed in honor of Sarcoma Awareness Month, observed each July.
Sarcoma is a soft-tissue cancer that may occur in a variety of the body’s soft tissues, including the nerves, muscles, joints, blood vessels, fat and more. Sarcoma may also occur in the bones. Although rare in adults, making up just ONE percent of adult cancers, sarcoma is relatively common in children, accounting for fifteen percent of childhood cancer cases. Bone and joint cancer is most frequently diagnosed among teenagers, while soft tissue cancers typically affect those 55 years or older.
Sarcoma is most often found in the arms and legs, where the majority of connective tissues are located, but it can occur virtually anywhere. Because the disease often starts deep in the body, it may not be noticeable until a large lump or bump appears — and at this point the cancer may be difficult to treat.
The Sarcoma Foundation of America (SFA) estimates that about 20 percent of sarcoma cases are curable by surgery while another 30 percent may be effectively treated with surgery, chemotherapy and/or radiation. However, in about half of cases, the disease is resistant to all form of treatments, highlighting an urgent need for new therapies.
Soft tissue sarcomas form in cartilage, fat, muscle, blood vessels, tendons, nerves, and around joints. Osteosarcomas develop in bone; liposarcomas form in fat; rhabdomyosarcomas form in muscle; and Ewing sarcomas form in bone and soft tissue.
In 2018, over 13,000 cases of soft tissue sarcoma and 3,400 cases of bone sarcomas are expected to be diagnosed in the United States, according to data from the National Cancer Institute’s Surveillance, Epidemiology, and End Results Program (SEER). Approximately 5,100 and 1,590 people are expected to die from soft tissue and bone sarcomas, respectively. The five-year survival rate for soft tissue sarcomas is 50 percent, while the survival rate is 66 percent for bone sarcomas.
Because sarcomas are difficult to distinguish from other cancers when they are found within organs, their incidence is probably underestimated, according to the National Cancer Institute.
Fast facts on sarcoma
- There are over 50 types of soft tissue sarcoma.
- Symptoms may not be apparent; often, the only sign of sarcoma is a lump.
- Pain may occur depending on where the tumor is located, or if it presses on nearby nerves.
- Treatment, as for other forms of cancer, can include surgery, radiation therapy, and chemotherapy.
Symptoms of soft tissue sarcoma
People with soft tissue sarcomas often have no symptoms. There may be no signs until the lump grows to a large size and can be felt; however, even this may go unnoticed. The main symptoms are:
- Pain – the most obvious symptom. Once the tumor affects local tissues, nerves, or muscles, it can be felt as pain in the general area.
- Inflammation – the tumor grows and eventually affects the area with inflammation and swelling.
- Location specific symptoms – inability to move limbs properly (if the sarcoma is on the arms or legs for example) and other impairments depending on the location.
Specific symptoms may reflect the particular type of sarcoma. For instance, tumors in the gastrointestinal system may bleed, so these sarcomas might produce symptoms like blood in the stool, or a stool that has a black, tarry appearance.
Types of sarcoma
Types of soft tissue sarcoma are defined by the specific tissue or location affected; they include:
- Undifferentiated pleomorphic sarcoma – previously known as malignant fibrous histiocytoma – this tumor is most often found in the arms or legs but sometimes at the back of the abdomen.
- Gastrointestinal stromal tumor (GIST) – this affects specialized neuromuscular cells of the gut.
- Liposarcoma – sarcoma of fat tissue.
- Leiomyosarcoma – affects smooth muscle in organ walls.
- Synovial sarcomas – these are usually found around a joint in the arms or legs.
- Malignant peripheral nerve sheath tumor – also known as neurofibrosarcoma, it affects the protective lining of the nerves.
- Rhabdomyosarcoma – this forms in muscle and is a childhood cancer. While rare overall, it is a relatively common tumor in children.
- Angiosarcomas – these develop in the cells of the blood or lymph vessels.
- Fibrosarcomas – sarcomas that usually form in the limbs or on the trunk, forming from fibroblasts, the most common cell type in connective tissue.
- Kaposi’s sarcoma – a sarcoma that affects the skin and is caused by a virus. The most common form of Kaposi’s sarcoma is related to AIDS. Kaposi’s tumors often produce distinctive skin lesions but also affect other soft tissues. Kaposi’s sarcoma is caused by the human herpesvirus 8.
- Further examples – these include dermatofibrosarcoma protuberans, a skin lesion; epithelioid sarcoma, which often affects young adults’ hands or feet; myxoma, which affects older adults, usually in the arms and legs; mesenchymomas, which are rare and combine elements of other sarcomas and can be found in any part of the body; vascular sarcomas, containing many blood vessels; and malignant neurilemmoma (also known as schwannoma). Sarcoma of the bone includes osteosarcoma, Ewing sarcoma, and chondrosarcoma.
Causes of sarcoma
The causes behind sarcoma are unknown, but there are some known risk factors. In adults, for instance, exposure to phenoxy acetic acid in herbicides or chlorophenols in wood preservatives may increase the risk. High doses of radiation are also known to cause sarcomas in some people, as are certain rare genetic alterations. The following inherited diseases are also associated with an increase sarcoma risk, according to SFA:
- Li-Fraumeni syndrome, which is associated with alterations in the p53 gene
- Von Recklinghausen’s disease (neurofibromatosis), which is associated with alterations in the NF1 gene
Having certain inherited disorders can increase the risk for other soft tissue sarcomas, including retinoblastoma, tuberous sclerosis, Werner syndrome, and nevoid basal cell carcinoma syndrome. Other risk factors for soft tissue sarcoma include past treatment with radiation therapy for certain cancers; exposure to certain chemicals, such as thorium dioxide, vinyl chloride, or arsenic; and long-term lymphedema in the arms or legs.
Past treatment with radiation can increase the risk of osteosarcoma and other types of bone cancers. Other risk factors for osteosarcoma include treatment with anticancer drugs called alkylating agents, having a certain change in the retinoblastoma gene, and having certain conditions including Paget disease, Diamond-Blackfan anemia, and Werner syndrome.
Tests and diagnosis of sarcoma
After understanding a patient’s medical history and completing a medical examination, medical imaging scans will help to inform a diagnosis, which will need to be confirmed through laboratory analysis of a tumor sample.
The following physical features should prompt a doctor to investigate:
- larger than 2 inches in diameter or growing in size
- fixed, immovable, or deep
- return of tumor previously removed
Medical imaging helps to locate, characterize, and give further information about a tumor to guide diagnosis. It may also be used to gauge the success of treatment or to look for spread of cancer.
Imaging techniques include X-rays, CT scan (computed tomography), MRI(magnetic resonance imaging), ultrasound, and PET (positron emission tomography).
Treatments for soft tissue sarcoma
The following treatment options can be used for sarcomas:
- Surgery – the most common sarcoma treatment. It aims to remove the tumor and even some of the normal surrounding tissue. A biopsy sample is often taken at the same time to confirm the exact type of tumor.
- Radiation therapy – this destroys cancer cells and may be done in addition to surgery, before or after the operation.
- Chemotherapy – this could also be used in addition to surgery, though less often than radiation therapy.
Some soft tissue sarcomas have other available treatments, such as specific drug treatments, gene-targeted therapies, or biological therapies such as immunotherapy.
Treatment choice and intensity are also guided by the stage and grade of cancer, by the size of the tumor, and extent of any spread.
Prevention of sarcoma
Sarcoma is not typically preventable. Radiation therapy is a risk factor, so reducing exposure to it may be preventive, although a need for radiation therapy usually outweighs any subsequent sarcoma risk.
Observing Sarcoma Awareness Month
Sarcoma is still considered to be the “forgotten cancer.” Efforts to encourage research and drug development are made more challenging due to a lack of awareness and understanding. How as a community do we raise funds for vital research if people don’t know that this cancer exists?
Though the Sarcoma Foundation of America works tirelessly every day to raise awareness, during Sarcoma Awareness Month, they aim to further highlight the extraordinary challenges sarcoma patients face and the need for more sarcoma research and better sarcoma therapies. Please join in efforts and pledge to bring awareness to your community. Here are a few ways you can help highlight the need for ongoing research on sarcoma:
- This July, take a moment to share a message via social media or speak with your friends and family about this relatively unknown condition.
- Join sarcoma patients, survivors and their loved ones for the Steps to Cure Sarcoma. Every dollar raised will be used to fund research, patient advocacy and education.
- Take a moment to listen to a Sarcoma Awareness Month Public Service Announcement campaign. Share it online to raise awareness.
- Get together with others in the sarcoma community and donate today!
Since sarcoma is a rare cancer, many people are unfamiliar with the disease and have not been affected personally. However, many children’s lives have been altered because of this disease, and advances in early detection and treatment could help save lives.
A number of clinical trails are underway for people with sarcoma. If you’ve been recently diagnosed, ask your doctor if a clinical trial, which could give you access to novel treatment options, is right for you.
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Han, MD, Seunggu. “Sarcoma: Symptoms, types, treatments, and causes.” Medical News Today. 23 May 2017.