(Family Features) Although rare cancers don’t occur often, they can affect people of all ages and genders. A rare cancer is defined as fewer than 15 new diagnoses per 100,000 people per year. That is according to the National Cancer Institute (NCI). Additionally, as noted by the American Cancer Society (ACS), the 5-year survival rate is lower for people diagnosed with a rare cancer than for people living with more common cancers. Greater awareness of rare cancers like soft tissue sarcoma may lead to earlier diagnosis and management. Early diagnosis is the key to potentially better survival rates.
There are more than 50 types of soft tissue sarcomas (STS)
STS account for about 1% of all cancers and affect soft tissues. Muscle, fat, nerves, blood vessels, and skin can be hosts for STS. STS can be found in any part of the body. However, they are often in the arms or legs, internal organs, the back of the abdominal cavity or in the trunk, head and neck area.
If you have recently been diagnosed with STS, ask your doctor for more information about the specific sub-type you have. For example, if you received a diagnosis of undifferentiated sarcoma, ask your doctor for an integrase interactor-1 (INI1) test to see if you have a rare STS called epithelioid sarcoma (ES).
Soft tissue sarcomas can be visible or invisible depending on location.
STS may appear as painless bumps under the skin, usually on arms or legs. Some sarcomas begin in the abdomen. They typically don’t show symptoms until they grow and press on nearby organs, nerves, muscles or blood vessels. When this occurs, symptoms may include pain and trouble breathing.
Early diagnosis can help inform disease management.
As with other types of cancer, early diagnosis of STS is key. Earlier treatment may result in more favorable outcomes. Because other conditions can cause similar symptoms, it’s important to check with your doctor if you are experiencing any of the warning signs listed above. If your doctor decides it’s best to “watch and wait,” consider developing a six-week follow-up plan in partnership with your health care team if your symptoms have not improved.
Seeking care from a specialist is key.
Given the rarity of STS, finding a sarcoma specialist who understands the complexity of this rare disease is important. They can help determine which treatment option is best for you is important. Treatment options depend on multiple factors. They include your overall health, the location and type of tumor, its size and whether the disease has spread elsewhere in the body. Typical treatment for STS includes a combination of options including chemotherapy, radiation therapy, and surgery. A specialist may also speak to you about participating in a clinical trial where investigational therapies in STS are being studied.
Learning More About Epithelioid Sarcoma
A rare type of STS, epithelioid sarcoma (ES), accounts for less than 1% of all STS. STS themselves account for approximately 1% of all cancers, according to research published in “Archives of Pathology & Laboratory Medicine”. ES can present as a lump or sore on the skin.
Notably, more than 90% of ES tumors do not express the INI1 protein The INI2 protein when present acts to suppress tumor growth. INI1 loss plays an important role in the diagnosis of ES, according to researchers with “The American Journal of Surgical Pathology.”
Data from the NCI indicates that approximately 150-200 people in the United States are diagnosed with ES each year. Research published in “The Journal of Clinical and Aesthetic Dermatology” found that the disease often occurs in young adults in their 20s and 30s. Because most ES patients are adolescents and young adults, there is a gap in the unique psycho-social needs for this patient population, including resources for patients who miss school while undergoing treatments, as well as fertility considerations later in life.
If you or someone you love is living with ES, you can find resources, information and the real-life perspective of an ES survivor at ESsentialsforES.com.
Content courtesy of Epizyme, Inc.
SOURCE:
Epizyme