Sarcoma Awareness

Articles

Jul 2

July is Sarcoma Awareness Month, and the Sarcoma Foundation of America takes action on July 21st for Virtual Advocacy Day. This organization aims to advocate for individuals who have sarcoma and encourage more scientific research into patient care and treatment development. Although this form of cancer is rare, it is important that more people in our communities are aware of its existence and those who suffer from it. Virtual Advocacy Day provides an opportunity for anyone to communicate about issues related to sarcoma and help to advocate for better outcomes for sarcoma patients.

What is Sarcoma?

Sarcoma is a type of cancer that occurs in the bones and the connective tissue (often called soft tissue). These structures in the body include the muscles, joints, tendons, blood vessels, fat, and nerves throughout the body. Since sarcoma can occur in all of these areas where bone or connective tissue exists, it can develop in any part of the body. In addition, there are over 70 different types of sarcoma. With so much variation, treatment and diagnosis are dependent on each specific case. Sarcoma represents about one percent of cancer occurring in adults, and twenty percent in children. In adults, sarcoma is most frequently detected in the limbs because these regions are comprised of a lot of connective tissue. Sarcoma is often not detected early and comes as a surprise for many individuals.

Common Types of Sarcoma

Although there are many different types of sarcoma with varying symptoms and affected areas of the body, there are a few which are more prevalent than others.

Liposarcoma

Occurring in the fat cells of the body, liposarcoma is typically found in the muscles and the abdomen. It can affect individuals of any age, although it is most often detected in older individuals.

Undifferentiated Pleomorphic Sarcoma

This type of sarcoma occurs in the arms and legs or in the abdominal area. It is characterized by the way the cells look, as they differ in appearance from the other cells in these regions of the body. Undifferentiated pleomorphic sarcoma cells grow in different sizes and shapes than other cells in the arms, legs, or abdomen.

Leiomyosarcoma

The urinary and digestive systems as well as the blood vessels are made of smooth muscle tissue. Leiomyosarcoma occurs in these smooth muscle tissues. It often forms in the abdomen or uterus and metastasizes quickly to spread throughout the other areas of smooth muscle tissue.

Uterine Sarcoma

Uterine Sarcoma is a rare cancer that takes place in the uterus’ muscles and supporting tissue. Sarcoma in the uterine lining attaches to tissues such as muscle, fat, and bone while cancers that start in the epithelial cells (cells that cover most organs) are called carcinomas.

In the uterus, the sarcoma attaches itself to the different types of connective tissue lining the uterus’ many layers. These layers are called:

Endometrium — the inner lining
Serosa — the layer of tissue coating the outside of the uterus
Myometrium — the thick middle layer of muscles that are used to push out a baby.

More than 95% of uterine cancers are classified as carcinomas. Carcinomas starting in the cervix are cervical carcinomas and if they start in the endometrium it is called endometrial carcinomas. Carcinosarcoma is another form of cancer that starts in the uterus where cancer starts in the endometrium and has features of both carcinomas and sarcomas. These cancers may also be classified as mixed malignant mixed mesodermal tumors or malignant mixed Mullerian tumors.

There are a few different types of Uterine Sarcomas:

Uterine Leiomyosarcoma (LMS): the most common form, it starts in the myometrium and can spread quickly.
Endometrial Stromal Sarcoma (ESS): rarer of the cancers, ESS starts in the connective tissue or in the endometrium where it can be either high or low grade meaning slower or faster to spread. If low grade the tumors may look very similar to healthy cells and with high grade it is very noticeable that the cancer is spreading faster.
Undifferentiated Sarcoma: start in the endometrium or myometrium and spread quickly.
Benign Uterine tumors: benign tumors can also develop in the uterine lining. These tumors have names such as leiomyomas, adenofibromas, and adenomyomas and are also known as fibroids. Since they are benign they may not need treatment or to be removed but if they do start to cause issues like heavy bleeding or pelvic pain it is worth considering a myomectomy where you would have the tumors removed.

Kaposi Sarcoma

Kaposi Sarcoma (KS) develops in the blood vessels and lymph systems. You will usually see KS in the form of tumors inside of the mouth or lymphs. If the tumors are in the lymph nodes that means there’s an easier chance of cancer spreading to the lungs and digestive tracts too. Kaposi Sarcoma can cause lesions that will appear on the legs or face. The lesions may be blotchy and red or purple, they usually shouldn’t cause any symptoms, however.

There are 4 different types of Kaposi Sarcoma:

Epidemic (AIDS-associated): KS is an AIDS-defining illness, meaning that when it is found in individuals who have HIV, they officially then have AIDS (and are not just HIV positive). For patients with AIDS, the US healthcare system has seen the highly active antiretroviral therapy (HAART) treatment has helped prevent many patients with HIV from having AIDS.
Classic (Mediterranean) Kaposi Sarcoma: mostly only occurs in older folks with Mediterranean, Eastern European, or Middle Eastern backgrounds. It’s more common in men and mostly shows up in lesions on the feet and legs. The immune system may be weaker but not as weak as epidemic KS. People who have KSHV (Kaposi Sarcoma-associated Herpesvirus) are more likely to develop KS.
Endemic (African) Kaposi Sarcoma: refers to those with KS in Equatorial Africa. A KSHV infection is more common in this part of the world. Endemic KS is often found in people under 30 and will affect the lymph system, it can progress fast through the body.
Iatrogenic (transplant-related) Kaposi Sarcoma: this means the immune system is weakened from an organ transplant and pushes the patient to have KS fully because of the types of drugs taken after surgery. Stopping the use of immune-lowering drugs or lowering the dose can help with the lesions that form after a transplant.

Adult Fibrosarcoma

Fibrosarcoma gets its start usually in the legs. It gets its name from the fibrous tissue that gets wrapped around tendons, ligaments, and muscles. The fibrous tissue is called malignant spindled fibroblasts or myofibroblasts. It can be found in infants under the age of 1, then it is called congenital fibrosarcoma, it grows slowly at this age. In Fibrosarcoma there may be the development of lumps that at first seem painless but proceed to grow and cause issues with organs.

Angiosarcoma

Angiosarcoma develops in the lymph nodes and blood vessels. It may look like a purple bruised area in an area and these areas can bleed easily if scratched. These bruises may get larger as time goes on. If the Angiosarcoma grows deeper in the body you may not notice the symptoms. This form of sarcoma is fairly rare and diagnosed often after it has spread to other parts of the body. If it’s possible patients with this form of sarcoma should catch it in its initial phases so that it doesn’t spread to other organs.

Symptoms and Causes of Sarcoma

The cause of sarcoma is still unknown, although it follows the general understanding of how cancer forms. Changes to the DNA within cells create a difference in how the cells replicate and die. As the cancerous cells grow, the rapid rate of accumulation often results in a tumor, they can move or spread to other regions of the body. Symptoms of sarcoma typically include pain in the bones, easily broken bones, pain in the abdomen, sudden or unexpected weight loss, or detection of a lump under the skin.

Early Detection Opportunities

To find out if you have a predisposition to the same sarcomas or cancers that your family has a history of, you can see a genetic counselor. They will perform testing to see how likely your genes are to produce the same issues and what to do to prevent the onset if possible. Of course, if you have any immediate concerns or existing cancers you should see your healthcare professional right away to ensure you have more time to develop a plan of treatment.

Diagnosing Sarcoma

Unfortunately, sarcoma is usually detected after the cancer is quite prevalent, which is often already in an advanced stage. However, healthcare professionals conduct a physical exam, imaging tests such as CT scans or X-rays, or a biopsy to test a sample of the affected tissue. These exams will indicate the stage or severity of sarcoma and typically will also indicate the next steps for treatment options.

Treatment for Sarcoma

Removal of the cancer is the most common route for treatment, however, it always depends upon the type of sarcoma and severity. Surgery is certainly an option for some individuals, and in some cases, all of the cancerous cells can be removed. Complications can occur, especially in cases where the sarcoma occurs in the nerves or blood vessels, as these structures may be vital for the body’s overall function. As with other forms of cancer, radiation therapy, chemotherapy, immunotherapy, ablation therapy, or targeted therapy may be recommended as the most effective treatment for some individuals.

2022 Sarcoma Statistics

Sarcoma comprises 1% of adult cancers in 2022 and 15-20% of childhood cancers
Sarcoma is sometimes curable by surgery (20% of the time) or by surgery and chemotherapy which increases chances by (50-55%)
This year it is estimated that roughly 17,000 will be diagnosed with some form of sarcoma
The overall sarcoma survival rate is 65%.

Recent Developments in Sarcoma Treatment

There’s currently a vast amount of research happening to better understand how to treat the different forms of Sarcoma. The research being done shows that different forms of sarcomas look very similar under a microscope. With further testing, it’s been found that the cancers that used to be called malignant fibrous histiocytoma (MFH) are actually high-grade sarcomas called liposarcoma, rhabdomyosarcoma, leiomyosarcomas, and even carcinomas or lymphomas.

Treatment for sarcomas takes place as it does for other forms of cancer through chemotherapy, radiation, targeted therapy, and anti-angiogenesis drugs.

Chemotherapy — research being done actively to target the site of the sarcomas
Radiation — research is being conducted to see different types of radiation techniques and schedules to provide efficiency and comfort for the patient
Targeted therapy — target cells that cause cancer to grow
Anti-angiogenesis drugs — medicines that block new blood vessel formation are thought to help kill sarcomas because they prevent the sarcoma from being nourished.

Occupational or Physical Therapy for Sarcoma Rehabilitation

After surgery or after the loss of typical muscle function, individuals with sarcoma can work with physical therapists to regain as much of their previous strength as possible in a safe way. Physical therapists can also assist patients to adapt to a more limited range of movement and possibly increase their range of movement or flexibility over time. Occupational therapy targets the adjustment of an individual’s daily activities. After surgery or after the loss of strength, normal daily routine activities can become more complex. Occupational therapists can address everything from the layout of your home to the equipment around your home to assist in mobility. Some individuals recovering from sarcoma require assistive equipment or prosthetics. Occupational or physical therapists can aid in the adjustment to those devices in most cases.

Awareness promotes an understanding of what individuals with sarcoma may go through, and can help everyone to be more supportive. Diagnosis of sarcoma can be overwhelming and scary to experience, but learning about this form of cancer can help individuals make clear and informed decisions about their path to treatment. If you or a loved one has undergone sarcoma treatment and require rehabilitation assistance, contact the Tilton’s Therapy team. Our occupational and physical therapists operate in the mobile setting, meaning they come to you! The natural environment of your home provides our therapists the unique opportunity to address all aspects of your treatment plan in a holistic way.

Looking for additional resources and awareness efforts to continue learning? Read about all of this month’s campaigns in our resources section or download fun, interactive ways to “tune up” your therapeutic practice at home.