Sarcoma Awareness Month: Facts About the Forgotten Cancer

Known as the "forgotten cancer," sarcoma's relative rarity makes it one of the least-discussed cancers. This lesser-known cancer has over 50 subtypes, however. Sarcomas can be found anywhere in the body, and begin in the bone, muscle, connective tissue, blood vessels, or fatty tissue.

The Facts About Sarcoma

The incidence rates of soft tissue sarcomas place them among the less common cancers, leading to a lack of discussion:

• 1 percent of cancers are sarcomas.
• Over 13,000 new sarcomas will be diagnosed in 2020 in America, according to American Cancer Society projections.
• Around 5,350 people will die of soft tissue sarcomas in 2020, according to the ACS.

Sarcomas are more common in children and young adults:

• Between 1,500 and 1,700 children are diagnosed with sarcomas every year.
• Around 15 percent of cancers in children and teens under 20 years old are sarcomas.

Types of Sarcomas

There is massive variability among sarcomas, which can develop in many different locations and tissues throughout the body. The three most common types are:

• Leiomyosarcoma: most commonly affecting people in their 70s, this malignant tumor grows from immature smooth muscle cells and accounts for 10-20 percent of all soft tissue sarcomas.
• Liposarcomas: accounting for 15-25 percent of all soft tissue sarcomas, these develop in fatty tissue and have different subtypes of varying severity.
• Malignant fibrous sarcoma: also known as pleomorphic undifferentiated sarcoma, this fibrous tissue sarcoma occurs often in the legs or abdomen.

Other types include:

• Angiosarcoma: forms in the blood and lymph vessels.
• Dermatofibrosarcoma: forms in the tissue under the skin, especially in the torso and limbs.
• Epitheloid sarcoma: typically appears as small nodules in the feet and hands of young adults.
• Ewing's sarcoma: typically found in children and young adults, this tends to begin in the bones of the pelvis, legs, and arms. Many Ewing's tumors can be extraosseous (outside the bones), however.
• Fibrosarcoma: forms in the fibrocytes, which make up the connective tissue surrounding muscles, tendons, and ligaments.
• Gastrointestinal stomach tumors (GISTs): develop in the walls of the digestive system.
• Kaposi sarcoma: cancer of the blood vessels or lymph system that often creates reddish or purple plaques on the skin. Most commonly associated with HIV infection, incidence rates in HIV patients are about an eighth of what they were in the early 90s thanks to improved treatments.
• Neurofibrosarcoma: forms in the peripheral nerves that connect the brain and spinal cord to the rest of the body.
• Rhabdomyosarcoma (RMS): an aggressive cancer that can form in any of the soft tissue, including muscles and connective tissue, and most often occurs to children and young adults.
• Synovial sarcoma: a tumor that most commonly begins in the areas around large joints.

Stages and Treatment

The overall relative survival rate for sarcoma is 50 percent.

Around 60 percent of sarcomas are found in a localized stage (a tumor only in one area of the body). The 5-year survival rate for localized sarcomas is 81 percent.

Around 19 percent of sarcomas are found in a locally advanced stage (a cancer that has spread to nearby areas). The 5-year survival rate for locally advanced sarcomas is 57 percent.

Around 15 percent of sarcomas are found in a metastatic stage (a cancer that has spread to other areas of the body). The five-year survival rate for metastatic sarcomas is 16 percent.

Treatment

These are among the most common types of treatment:

• Surgery: Used to locate and remove the tumor.
• Radiation Therapy: May be used before surgery (neoadjuvant therapy) or afterwards (adjuvant therapy). The use of radiation is strongly dependent on the type and severity of the sarcoma.
• Chemotherapy: Used in combination with surgery and radiation therapy to treat advanced-stage, soft tissue sarcoma.
• Targeted Therapies: drugs that block proteins in cancer cells or growth of blood vessels that nourish cancers. These are designed to impact the cancer cells while sparing the healthy cells nearby.