What is Soft Tissue Sarcoma?
Soft tissue sarcoma is a cancerous (malignant) tumor that starts in soft tissue cells of the body. Cancerous tumor is a group of cancer cells that can invade and destroy nearby tissue. The tumor can also spread (metastasize) to other parts of the body.
There are many types of soft tissue throughout the body, including fat, muscle, fibrous tissue, blood vessels, lymphatics, and nerves. Soft tissues surround, support, and connect organs and other tissues.
Soft tissue cells sometimes undergo changes that make the way they grow or behave abnormally. These changes can lead to the formation of non-cancerous (benign) tumors such as lipomas or hemangiomas.
But in some cases, changes in soft tissue cells can cause a type of cancer called soft tissue sarcoma. There are many different types of soft tissue sarcoma. They are named after where the cancer started. Some of the more common types are liposarcoma, myxofibrosarcoma, and leiomyosarcoma.
Soft tissue sarcoma can appear anywhere in the body. More than half do so in an arm or a leg. The others usually start in the abdomen, chest, head or neck area.
Soft tissues of the body
Soft tissue is found all over the body. There are many types of soft tissue, including fat, muscle, fibrous tissue, blood vessels, lymphatics, and nerves. Soft tissues surround, support, and connect organs and other tissues.
Soft tissue function
surround, support and connect organs and other parts of the body;
give shape and structure to the body;
move fluids, such as blood, from one part of the body to another;
Types of soft tissue
There are different types of soft tissue found in the body.
Fat is a soft tissue made up of fatty cells, or adipose, called adipocytes that are tightly packed together. We also speak of fatty tissue or adipose tissue. Fat is commonly found under the skin of the buttocks, hips, waist and abdomen. Fat also surrounds organs like the kidneys. Fat acts as a cushion for the body and for cushioning between organs, and it helps keep the body warm. The body stores fat and uses it for energy when you need it.
Fibrous tissue is connective tissue made up of pieces that resemble a cord and are called fibers. These fibers help move parts of the body and keep them strong and stable. Tendons, which connect muscles to bones, and ligaments, which connect bones to other bones, are made of fibrous tissue. Fibrous tissue is also found in the walls of blood vessels. Many organs are surrounded by fibrous tissue.
There are 3 types of muscles, namely smooth muscles, skeletal muscles and heart muscle (heart).
Smooth muscles work automatically, without you having to think about it (involuntary muscles). They are found in the walls of hollow organs in the body such as the stomach, intestine, bladder, uterus, and blood vessels. Smooth muscles allow organs to relax and enlarge (dilate) or tighten and shrink (contract).
Skeletal muscles are a type of muscle that you control to move your body (voluntary muscles). They are mostly found in the muscles that attach to the bones. Certain skeletal muscles of the face attach themselves to the skin. Skeletal muscles hold the bones of the skeleton together and help you stand up straight. They also allow you to move different parts of your body, such as your arms and legs.
The heart muscle forms the walls of the heart and allows it to pump blood. The heart muscle works automatically, without you having to control it.
Synovial tissue is a thin, loose connective tissue that lines joints, such as those in the elbows and knees. It is also seen around tendons and fluid-filled sacs between bones and tendons (bursae). Synovial tissue produces synovial fluid, a thick fluid that allows surfaces to slide off easily.
Blood vessels are long, hollow, elastic tubes found throughout the body. Arteries, veins, and capillaries are types of blood vessels. Blood circulates through the blood vessels and carries oxygen, nutrients, hormones, wastes, and other products throughout the body.
Lymphatic vessels are small tubes like blood vessels that run throughout the body. They contain lymph which collects and transports wastes, germs and damaged cells away from body tissues.
Nerves are soft tissues that control all the functions of the body as well as all of its movements. Nerve tissue is made up of 2 main types of cells, namely nerve cells (neurons) and glial cells (neuroglial cells). Nerve cells send messages from one part of the body to another in the form of electrical impulses. Glial cells support nerve cells. Most of the nervous tissue is found in the brain and spinal cord, which make up the central nervous system (CNS). Some nerve tissue is located outside the brain and spinal cord and forms the peripheral nervous system.
Gastrointestinal stromal tumors (GISTs)
Gastrointestinal stromal tumor (GISTs) starts in special cells in the digestive tract, or gastrointestinal (GI) tract, called interstitial Cajal cells. These cells exhibit characteristics of smooth muscle cells and nerve cells. They receive messages (signals) from the nervous system that tell them to tighten (contract) and relax (relax) the muscles of the digestive tract. The contraction and relaxation of these muscles (peristalsis) allow food and fluids to move through the digestive system.
GISTs can appear anywhere in the digestive tract, but it most often does in the stomach. The small intestine is the second most common location. GISTs can also start in the esophagus, colon and rectum.
Most GISTs have a change (mutation) in the KIT (c-KIT) gene that causes the body to make too much of the Kit protein. The second change that most commonly affects GISTs is in the PDGF-R alpha gene (platelet-derived growth factor receptor alpha gene). This change causes the body to make too much of the PDGF-R alpha protein. KIT and PDGF-R alpha proteins are enzymes called tyrosine kinases that help cells grow, divide, and move.
Types of GISTs
GISTs can be non-cancerous (benign) or cancerous (malignant).
Non-cancerous GISTs does not spread (does not metastasize) to other parts of the body. It is generally not life threatening. A GISTs that is less than 2 cm is usually not cancerous.
GISTs can also be cancerous (malignant). Cancerous GISTs can invade and destroy nearby tissue. It can also spread to other parts of the body.
GISTs can start at any age, but it usually affects people over 50.
Almost all GISTs appear sporadically. This means that there is no clear reason for their occurrence. In a small number of people, GISTs occurs because of an inherited disorder, including the following:
familial GISTs syndrome – large number of GISTs
neurofibromatosis type 1
Carney-Stratakis syndrome – presence of GISTs and paragangliomas
GISTs may not cause any signs or symptoms in the very early stages of the disease. Signs and symptoms often appear when the tumor grows and causes problems such as pain and bleeding.
The signs or symptoms of GISTs include the following:
- blood in the stool or vomiting blood
- fatigue caused by a low number of red blood cells (anemia)
- abdominal pain or discomfort
- nausea and vomiting
- feeling of fullness
- mass in the abdomen
- difficulty swallowing
The process of diagnosing a GISTs can begin with a visit to your family doctor. Your doctor will ask you about your symptoms and may do a physical exam. Sometimes you come across GISTs by accident when you take exams for other reasons. Your doctor may refer you to a specialist or order certain tests to check for the presence of GISTs or other health problems.
The following tests may be used to rule out or diagnose GISTs:
- blood tests, such as a complete blood count (CBC) or blood chemistry tests
- imaging tests, such as computed tomography (CT), magnetic resonance imaging (MRI), or positron emission tomography (PET)
- endoscopy (sometimes accompanied by an ultrasound), such as a gastroscopy or colonoscopy
- biopsy, often performed during an endoscopy
Description of GISTs
GISTs is described or categorized based on the amount of cancer present in the body and its location at the time of initial diagnosis. This is often referred to as the extent of cancer. The information revealed by the exams is used to determine the following:
- where the cancer started
- the size of the tumor
- the speed at which cancer cells grow and divide (frequency of mitosis)
- if the cancer has spread from its place of origin
- where the cancer has spread
Your healthcare team uses this information to plan your treatment and predict the outcome (your prognosis).
When doctors describe the extent of GISTs, they may use the words localized or metastatic. Localized means the cancer is only found in or near the organ where it started and has not spread to other parts of the body. Metastatic means in a part of the body further away from where GISTs originated, such as liver or retroperitoneal cavity.
A recurrence of GISTs means that the cancer comes back after treatment. If it reappears where it was first born or nearby, it is called a local recurrence. It can also reappear in another part of the body: this is called a metastatic GISTs.
If GISTs spreads
Cancer cells can spread from where GISTs started to other parts of the body. This spread is called metastasis.
Understanding how a type of cancer usually grows and spreads helps your healthcare team plan your treatment and future care. If GISTs spreads, it can spread through the abdomen to the liver or peritoneal cavity. It can also spread to the lungs and bones, but it is rather rare.
Risk of spread of GISTs
Knowing how quickly cancer grows and how likely it is to spread helps doctors plan your treatment. It can also help the healthcare team determine the possible outcome of the disease (prognosis) and how the cancer might respond to treatment.
A pathologist examines a sample of tissue taken from the tumor under a microscope to determine the number of times the cells are dividing (mitotic index). It also takes into consideration the size of the tumor and its location in the body. GISTs is then classified as low risk or high risk based on its risk of progression. Low-risk, slow-growing GISTs is small and present in the stomach, and tends to have a better prognosis.
GISTs is different from other types of soft tissue sarcoma, which is why it is treated differently. When your healthcare team decides which treatments to offer you, they take the following into consideration:
- the location of GISTs in the body
- whether the GISTs is low risk or high risk
- the possibility of having surgery (resectable tumor) or not (unresectable tumor)
- the types of genetic mutations in GISTs
- your overall health
- what you prefer or want
You may be offered one or more of the following treatments for GISTs.
Surgery is the main treatment for GISTs that has not spread (no metastases). The tumor is removed along with some normal tissue all around (complete resection). Depending on the size of the tumor and its location in the digestive tract, one of the following surgeries may be done.
Wedge resection and segmental resection remove the part of the stomach that contains a small GISTs.
Gastrectomy involves removing part or all of the stomach through a cut (incision) made in the abdomen. This procedure is done for GISTs located in the stomach.
Bowel resection involves removing part of the small or large intestine, or both.
One-piece resection involves removing the tumor and surrounding areas in one piece. It is used for a GISTs which is fixed to neighboring bodies.
Sometimes the surgery is done by laparoscopy. In this case, the surgeon makes small incisions in the abdomen to insert an endoscope (a thin tube-like instrument with a lumen and a lens) and tools to perform the surgery. If the laparoscopic surgery cannot be performed, an open method is used, which means a large incision is made in the abdomen to reach the intestine.
In targeted therapy, drugs are used to target specific molecules, such as proteins, present on the surface or inside cancer cells to stop the growth and spread of cancer while limiting damage to the cancer cells. normal cells. The targeted drugs used for GISTs are called tyrosine kinase inhibitors. These drugs block many different proteins, including the KIT protein, to help stop or slow the growth and spread of cancer cells. They also prevent tumors from forming new blood vessels.
The following medicines are taken each day in pill form. In many cases, they are given until the disease gets worse or you can no longer tolerate the side effects of the treatment.
Imatinib (Gleevec) can be used after removing GISTs with a mutation in the KIT gene and when there is a high risk of recurrence. It can also be used when the GISTs is unresectable or metastatic.
Sunitinib (Sutent) can be used for unresectable or metastatic GISTs if imatinib is not effective.
Regorafenib (Stivarga) can be used for unresectable or metastatic GISTs that has progressed after being treated with imatinib and sunitinib.
Kaposi’s sarcoma (KS)
Kaposi’s sarcoma (KS) is a rare cancer that starts in cells that line lymphatic or blood vessels. It differs from other sarcomas because it can appear in several parts of the body at the same time.
KS usually occurs on or under the skin. It can also do it in the lymph nodes or the layers of tissue that line the mouth, nose, throat and anus. KS can also appear in the lungs or other parts of the digestive tract (gastrointestinal tract, or GI).
The main risk factors for KS are:
infection with human herpesvirus 8, or HHV-8 (also called Kaposi’s sarcoma herpesvirus, or HVSK) – virus found in the tumor of people with AIDS-related KS
weakened immune system (immunosuppression)
Types of Kaposi’s sarcoma
There are 4 main types of KS.
AIDS-related Kaposi’s sarcoma
AIDS-related KS is also called epidemic KS. It is the most common of the 4 types of KS. It appears in people who are infected with HIV (human immunodeficiency virus). HIV is the virus that causes AIDS (Acquired Immune Deficiency Syndrome). It damages certain cells of the immune system, making the body unable to fight off infections and diseases. As the immune system weakens, the risk of KS increases. If KS develops in a person with HIV, they are also diagnosed with AIDS.
AIDS-related KS can start in many different parts of the body including the skin, mouth, lymph nodes, digestive tract, lungs, liver and spleen. AIDS-related KS eventually spreads throughout the body.
Classic Kaposi’s sarcoma
Classic KS is rare. It appears mostly in older people of Mediterranean, Middle Eastern or Eastern European descent. It affects men more than women.
Classic KS develops slowly. Usually, it is only seen in the skin, particularly of the legs, ankles and soles of the feet. But it can spread to the digestive tract, lymph nodes, and other organs. In some people with classic KS, secondary cancer develops, most often non-Hodgkin lymphoma.
Kaposi’s sarcoma in transplant recipients
KS in transplant recipients is also called iatrogenic KS. It occurs in people with weakened immune systems after an organ transplant. People who have had an organ transplant usually need to take medicines that weaken their immune system (immunosuppressive therapy) to reduce the risk of rejection of the transplanted organ by their body, which increases the risk of catching an infection. at HHV-8.
KS in transplant recipients often only affects the skin, but it can also spread to mucous membranes or other organs.
African Kaposi’s sarcoma
African KS is also called endemic KS. It is observed in parts of Africa, near the equator. It mostly affects young adult males.
African KS can grow slowly like classic KS, but there is also a form that grows very quickly (aggressive). In addition to causing skin tumors, African KS can invade tissues under the skin (subcutaneous tissue) and bones.
One of the forms of African KS frequently appears in young children. It starts in the lymph nodes and other organs. It tends to lead to a very poor prognosis.
Usually, KS first appears on the skin. The signs or symptoms of KS include the following:
- lumps, patches, or bumps on the skin that are often red, purple, or brown
- plaques or bumps in the mouth or throat
- swollen ankles and feet that can be painful
- swollen lymph nodes
- bleeding from the digestive tract, like blood in stool
- pain in the abdomen
- difficulty in breathing
The process of diagnosing KS usually begins with a visit to your family doctor. He will ask you about the symptoms you are experiencing and he will do a physical exam. Based on this information, your doctor may refer you to a specialist or order tests to check for KS or other health problems. You can do the following exams:
- biopsy, such as a punch biopsy or surgical biopsy
- pulmonary radiography
- computed tomography (CT)
- endoscopy, such as a colonoscopy or bronchoscopy
Stages and risk categories
Staging describes or categorizes cancer based on how much cancer is in the body and where it was initially diagnosed. This is often referred to as the extent of cancer. Your team of care uses stage to plan your treatment and predict the outcome (your prognosis).
There is an AIDS-related KS staging system that has been developed by the AIDS Clinical Trials Group. It is based on 3 factors:
- location of KS in the body – only in the skin and lymph nodes or widespread throughout the body
- immune system health – high or low number of CD4 cells (type of lymphocyte) in the blood
- extent of illness in the body – symptoms and illness that affect the whole body, such as fever, night sweats, or infections
Doctors then determine whether KS is bad risk or good risk based on these factors.
KS is different from other soft tissue sarcomas, so it’s treated differently. When your healthcare team decides which treatments to offer you, they take the following into consideration:
- the type of KS
- if KS is only present in a certain area (local disease) or in many parts of the body (generalized disease)
effectiveness of your immune system
- your general health
Treatment of infections and management of immunosuppression
An important part of treating KS is treating all infections and managing immunosuppression.
Combination antiretroviral therapy (cART) is a combination of drugs given to treat HIV. It is also the first treatment used for AIDS-related KS. Sometimes cART is the only treatment needed or it can be combined with chemotherapy.
Stopping immunosuppressive therapy may be the first step in treating KS in transplant recipients. Symptoms of KS in transplant recipients may improve if the immunosuppressive drugs are stopped or the dose is reduced, but this can increase the risk of organ rejection.
Antibiotics are used to treat and prevent bacterial infections.
Surgery may be used to remove small areas of the skin affected by KS, especially when it is classified as good risk. The following types of surgery can be done.
Surgical excision removes the cancer and some of the normal tissue around it.
During curettage and electrodessication, a sharp instrument called a curette is used to scrape the cancer. The area is then treated with an electric current to destroy any remaining cancer cells.
Cryosurgery uses extreme cold to freeze and destroy tissue.
In external beam radiation therapy, a device is used to direct a beam of rays to the area of the skin and a small amount of nearby tissue. It may be used to treat one or a few skin tumors in a specific area. In some cases, it can also be given to control a more common disease.
For topical treatment, a gel or cream is used to apply the drugs directly to the skin. Alitretinoin (Panretin) is a topical treatment that can be used for AIDS-related KS in the skin.
In chemotherapy, anti-cancer, or cytotoxic, drugs are used to destroy cancer cells. It is usually given as a systemic treatment, which means that drugs travel through the blood to reach and destroy cancer cells throughout the body. The following chemotherapy drugs are used alone or in combination to treat KS:
- pegylated liposomal doxorubicin (Caelyx)
- paclitaxel (Taxol)
- docetaxel (Taxotere)
- vinorelbine (Navelbine)
- gemcitabine (Gemzar)
- bleomycin (Blenoxane)
- etoposide (Vepesid, VP-16)
- vincristine (Oncovin)
A small amount of a chemotherapy drug may be injected directly into the skin tumor to destroy cancer cells, called intralesional chemotherapy. Vinblastine is most often used.
Find out more about chemotherapy.
Immunotherapy uses drugs to help the immune system fight cancer cells. Sometimes immunotherapy is only given or combined with chemotherapy for AIDS-related KS. Immunotherapeutic drugs that can be used include interferon alpha 2a, interferon alpha 2b and interleukin 12, among others.
Find out more about immunotherapy.
In targeted therapy, drugs are used to target specific molecules, such as proteins, on the surface or inside of cancer cells to stop the growth and spread of cancer while by limiting damage to normal cells. It is sometimes given to some people with AIDS-related KS. Targeted drugs used include imatinib (Gleevec) and bevacizumab (Avastin).
1. Major Types of Soft-Tissue Sarcomas in Adults
|Tissue of Origin||Type of Cancer||Usual Location in the Body|
|Fibrous tissue||Fibrosarcoma||Arms, legs, trunk (torso)|
|Malignant fibrous hystiocytoma||Legs|
|Fat||Liposarcoma||Arms, legs, trunk|
|Muscle (striated)||Rhabdomyosarcoma||Arms, legs|
|Muscle (smooth)||Leiomyosarcoma||Uterus, digestive tract|
|Blood vessels||Hemangiosarcoma||Arms, legs, trunk|
|Kaposi’s sarcoma||Legs, trunk|
|Synovial tissue||Synovial sarcoma (Linings of joint cavities, tendon sheaths)||Legs|
|Peripheral nerves||Malignant peripheral nerve sheath tumour / Neurofibrosarcoma||Arms, legs, trunk|
|Cartilage and bone-forming tissue||Extraskeletal chondrosarcoma||Legs|
|Extraskeletal osteosarcoma||Legs, trunk (not involving the bone)|
2. Major Types of Soft-Tissue Sarcomas in Children
|Tissue of Origin||Type of Cancer||Usual Location in the Body||Most common ages|
|Embryonal||Head and neck, genitourinary tract||Infant–4|
|Alveolar soft part sarcoma||Arms, legs, head, and neck||Infant–19|
|Fibrous tissue||Fibrosarcoma||Arms and legs||15–19|
|Malignant fibrous histiocytoma||Legs||15–19|
|Fat||Liposarcoma||Arms and Legs||15–19|
|Blood vessels||Infantile hemangio-pericytoma||Arms, legs, trunk, head, and neck||Infant–4|
|Synovial tissue||Synovial sarcoma (Linings of joint cavities, tendon sheaths)||Legs, arms, and trunk||15–19|
|Peripheral nerves||Malignant peripheral nerve sheath tumors (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas)||Arms, legs, and trunk||15–19|
|Muscular nerves||Alveolar soft part sarcoma||Arms and legs||Infant–19|
|Cartilage and bone-forming tissue||Extraskeletal myxoid chondrosarcoma||Legs||10–14|
Risk factors for soft tissue sarcoma
A risk factor is something, like a behavior, substance, or condition that increases your risk for developing cancer. Most cancers are caused by many risk factors, but sometimes soft tissue sarcoma develops in people who do not have any of the risk factors described below.
Soft tissue sarcoma can start at any age, but it most often occurs in people over the age of 50.
Risk factors are usually ranked from most important to least important. But in most cases, it is impossible to rank them with absolute certainty.
- Genetic disorders
- Chronic lymphedema
- Viral infections
- Vinyl chloride
- Thorium dioxide
There is convincing evidence that the following factors increase your risk for soft tissue sarcoma.
People who receive radiation therapy as treatment for another cancer are at increased risk of developing soft tissue sarcoma. This is especially true for people who have received radiation therapy for Hodgkin lymphoma, non-Hodgkin lymphoma, or breast cancer. Soft tissue sarcoma often starts in the area of the body that has been treated with radiation therapy. It takes an average of 10 years between contact with radiation and the diagnosis of soft tissue sarcoma.
Less than 5% of sarcomas are caused by radiation therapy. The benefits of radiation therapy as a cancer treatment outweigh the increased risk of developing soft tissue sarcoma.
The following rare inherited disorders increase your risk of developing soft tissue sarcoma.
Retinoblastoma is a cancerous tumor that starts in the retina (membrane at the back of the eye). It mostly affects children.
Werner’s syndrome causes very rapid aging after puberty.
Li-Fraumeni syndrome is linked to an increased risk of developing several types of cancer, including soft tissue sarcoma.
Type 1 neurofibromatosis, also called von Recklinghausen disease, affects the nervous system. It is a condition that causes tumors to form inside nerve tissue, in the skin and under the skin. People with type 1 neurofibromatosis are at increased risk for certain cancers, including soft tissue sarcoma.
Gardner syndrome causes a large number of polyps to form in the colon, as well as tumors in the bones and soft tissues.
Lymphedema is a buildup of fluid in body tissues. Long-lasting (chronic) lymphedema can cause lymphangiosarcoma, also called Stewart and Treves syndrome. It is a rare type of soft tissue sarcoma that appears in the lymphatic vessels.
The following viral infections increase your risk for soft tissue sarcoma.
Kaposi’s sarcoma herpes virus (HVSK) is also called human herpes virus 8 (HHV-8). This virus can cause the development of a rare type of sarcoma called Kaposi’s sarcoma (SK).
The human immunodeficiency virus (HIV) increases the risk of KS and soft tissue sarcoma.
Vinyl chloride is used in the plastics industry to make polyvinyl chloride (PVC), which is found in many products. Being in contact with vinyl chloride at work increases the risk of angiosarcoma of the liver, a type of soft tissue sarcoma.
Thorium dioxide (Thorotrast) is a radioactive solution that was used in the 1950s as a contrast medium to increase the efficiency of X-ray images of blood vessels and arteries, nasal passages and sinus cavities. Thorium dioxide was injected into a vein and then accumulated in the liver, spleen and bone marrow. People who have been exposed to thorium dioxide are more likely to have angiosarcoma of the liver, which is a type of soft tissue sarcoma. High doses increase the risk. Cancer can appear for up to 45 years after exposure.
Possible risk factors
The following factors have been linked to some extent with soft tissue sarcoma, but there is insufficient evidence to suggest that they are risk factors. More research is needed to clarify the role of these factors in the development of soft tissue sarcoma:
chemotherapy already given
personal history of cancer
coming into contact with dioxin at work
Reduce the risk of soft tissue sarcoma
You can reduce your risk of soft tissue sarcoma by adopting the following behaviors.
Follow health and safety guidelines when handling certain chemicals
Coming in contact with vinyl chloride or thorium dioxide increases your risk for certain types of soft tissue sarcoma. Being in contact with dioxin can also increase your risk. Following health and safety guidelines can help reduce your contact with these chemicals.
Finding soft tissue sarcoma in its early stages
Finding and treating soft tissue sarcoma at an early stage increases the chances of successful treatment. Have a regular check-up and see your doctor if you experience any symptoms or are concerned about your health.
If your risk is above average, you may need to see your doctor more often to check for soft tissue sarcoma. This may be your case if:
- you have a history of cancer treated with radiotherapy;
- you have certain genetic disorders;
- you have long-lasting (chronic) lymphedema;
- you are in contact with vinyl chloride or thorium dioxide;
- you have certain viral infections, including Kaposi’s sarcoma herpes virus (HCVS) and human immunodeficiency virus (HIV).
Symptoms of soft tissue sarcoma
Soft tissue sarcoma may not cause any signs or symptoms in the very early stages of the disease. Signs and symptoms often appear as the tumor grows and puts pressure on nerves, muscles or other parts of the body. Other medical conditions can cause the same symptoms as soft tissue sarcoma.
The signs or symptoms of soft tissue sarcoma can vary depending on where the tumor started in the body.
When soft tissue sarcoma starts in an arm, leg, trunk, or head and neck area, the most common signs and symptoms are:
- lump or swelling
- tenderness or pain
When soft tissue sarcoma starts in the abdomen, it can cause the following:
- feeling of fullness
- pain in the abdomen or back
When soft tissue sarcoma starts in the digestive tract (gastrointestinal tract, or GI), it can cause the following:
- nausea and vomiting
- blood in stool
- pain in the abdomen
Here are other signs and symptoms of soft tissue sarcoma:
- abnormal vaginal bleeding
- pelvic pain
- difficulty in breathing
- abnormal masses or plaques on the skin
Diagnosis of soft tissue sarcoma
Diagnosis is a process of identifying the cause of a health problem. The diagnostic process for soft tissue sarcoma can begin with a visit to a doctor, often a specialist. Your doctor will ask you about your symptoms and may do a physical exam. You may be ordered for tests to check for soft tissue sarcoma or other health problems. But sometimes soft tissue sarcoma is caught accidentally while trying to find a different health problem.
The diagnostic process can seem long and overwhelming. It’s okay to worry, but try to remember that other medical conditions can cause symptoms similar to those of soft tissue sarcoma. It is important that the healthcare team rule out any other possible cause of the condition before making a diagnosis of soft tissue sarcoma.
The following tests are usually used to rule out or diagnose soft tissue sarcoma. Many tests that can diagnose cancer are also used to determine how far the disease has spread (extent of cancer). Your doctor may also give you other tests to check your general health and to help plan your treatment.
Health history and physical examination
Your health history consists of a checkup of your symptoms, your risks, and any medical events and conditions you may have had in the past. Your doctor may ask you questions about the following:
signs or symptoms that suggest soft tissue sarcoma, such as a lump or swelling
history of cancer and previous administration of radiation or chemotherapy
certain inherited disorders that you or members of your family have, such as type 1 neurofibromatosis
family history of soft tissue sarcoma and other cancers
The physical exam allows your doctor to look for any signs of soft tissue sarcoma. During the physical exam, your doctor may:
examine any lumps on your body, including checking their size and shape and feeling them;
check how parts of your body move, such as your arms and legs, and whether they are weak;
examine your lungs;
feel your abdomen to see if your liver is swollen or if there are any abnormalities.
Complete blood count and blood clotting tests
The complete blood count (CBC) is used to assess the quantity and quality of white blood cells, red blood cells and platelets. An CBC is done to check your general health, often before a biopsy.
Blood clotting tests can tell if your blood is clotting properly. Prothrombin time (PT), partial thromboplastin time (TT), and International Normalized Ratio (INR) are common blood clotting tests. They are usually done before a biopsy.
Blood biochemical analyzes
A blood chemistry test measures the level of chemicals in the blood. It makes it possible to evaluate the quality of functioning of certain organs and also to detect anomalies. The blood chemistry tests that are used often depend on the type of symptoms present. Among other things, an electrolyte profile, liver function tests (liver) and kidney function tests (kidney) can be done.
Magnetic resonance imaging
Magnetic resonance imaging (MRI) uses powerful magnetic forces and radio waves to produce cross-sectional images of the body’s organs, tissues, bones, and blood vessels. A computer assembles the images into 3-dimensional snapshots.
MRIs are frequently used to look at a lump that doctors think is soft tissue sarcoma. This is often the first imaging test done for masses on the arms or legs. MRI can help determine the size of the tumor and what tissues and areas are nearby and also whether it has spread to other parts of the body.
A computed tomography (CT) scan uses special x-ray machines to produce 3-dimensional and cross-sectional images of the body’s organs, tissues, bones and blood vessels. A computer assembles the photos into detailed images.
A CT scan is often used to look at a lump in a specific area, including things like determining its size and finding out what tissues and areas are nearby. We often do a CT scan to check the abdomen, back part of the abdomen (retroperitoneum), and chest. If you are diagnosed with soft tissue sarcoma, a CT scan can tell if the cancer has spread to other parts of the body. CT may be used instead of MRI.
In an x-ray, low-dose radiation is used to produce images of parts of the body on film. It can be used to examine a mass.
In an ultrasound, high-frequency sound waves are used to produce images of parts of the body. The ultrasound can tell if a lump is a fluid-filled cyst or a solid tumor.
During a biopsy, the doctor removes tissues or cells from the body for analysis in the laboratory. The pathologist’s report confirms whether or not there are cancer cells in the sample. If a diagnosis of cancer is made, the report will also indicate the type of cancer and the grade of the tumor. All of this is usually done in a large cancer treatment center where staff are experienced in the management of soft tissue sarcoma. It may take a few weeks to see results.
The type of biopsy performed depends on the location of the mass and its size.
In a core biopsy, or core biopsy, a hollow needle is used to remove tissue from the body. It is a type of biopsy that is often used to diagnose soft tissue tumors. An imaging test, such as a CT, can be done to guide the needle to the tumor.
In a surgical biopsy, tissue is removed from a lump or the entire lump is removed by surgery. It is a type of biopsy that is often used to diagnose soft tissue tumors.
In an endoscopic biopsy, tissue is removed from a lump or the entire lump is removed endoscopically. This type of biopsy can be done when the mass is buried deep in the body.
In a bone scan, radioactive materials that attach to the bones (radiopharmaceuticals) and a computer are used to create an image of the bones. It is used to find out if the soft tissue sarcoma has spread to the bones.
Histological classification of soft tissue sarcoma (Grading soft tissue sarcoma)
The histological classification defines the appearance of cancer cells compared to that of normal, healthy cells. The grade gives your healthcare team an idea of how quickly cancer grows and how likely it is to spread. It helps him plan your treatment. The grade can also help the healthcare team determine the possible outcome of the disease (prognosis) and predict how the cancer might respond to treatment. The grade is also used to establish the stage of soft tissue sarcoma.
To establish the grade of soft tissue sarcoma, the pathologist examines a sample of tissue taken from the tumor under a microscope. The following are some of the factors that are used to grade soft tissue sarcoma:
- number of times cells divide (mitotic index)
- number of dead cells or tissue (necrosis)
- number and types of cells in a tissue sample (cellularity)
- size and shape of cells and their nucleus (pleomorphism)
- type of cancer (histological type)
- how different the appearance of cancer cells from normal cells (differentiation)
The pathologist usually assigns a grade of 1 to 3 for soft tissue sarcoma. The lower this number, the lower the rank. There are several different histological classification systems for soft tissue sarcoma and some of them have 2 or 4 grades rather than 3.
Low-grade cancers are made up of well-differentiated cancer cells. These cells are abnormal, but they look a lot like normal cells and are arranged very similarly to normal cells. Low-grade cancers tend to grow slowly and are less likely to spread.
High-grade cancers are made up of poorly differentiated or undifferentiated cancer cells. These cells do not look like normal cells and are arranged very differently. High grade cancers tend to grow quickly and are more likely to spread than low grade cancers.
Stages of soft tissue sarcoma
Staging describes or categorizes cancer based on how much cancer is in the body and where it was initially diagnosed. This is often referred to as the extent of cancer. Information from tests is used to find out how big the tumor is, in what part of the body the cancer started, if the cancer has spread from where it started and where it is. has spread. Your healthcare team uses the stage to plan your treatment and predict the outcome (your prognosis).
The most frequently used staging system for soft tissue sarcoma is the TNM classification. In the case of soft tissue sarcoma, there are 4 stages. For stages 1 to 4, the Roman numerals I, II, III and IV are often used. But in order to make the text clearer, we will use the Arabic numerals 1, 2, 3 and 4. In general, the higher the stage number, the more cancer has spread. Talk to your doctor if you have questions about staging.
The stages of soft tissue sarcoma only apply to sarcoma of the soft tissue of the arm, leg, outer parts of the center of the body (superficial trunk) or the back part of the abdomen (retroperitoneum). Sarcoma of the soft tissues of the head and neck and the large organs inside the chest or abdomen (viscera) is not assigned a stage. These tumors are grouped only according to the type of tumor, its size and its invasion of neighboring regions.
The Soft Tissue Sarcoma Staging System does not apply to the following tumors:
- Kaposi’s sarcoma
- Darier and Ferrand dermatofibrosarcoma of the head and neck
- sarcoma that starts in the brain, the lining around the brain and spinal cord (dura), a hollow viscus like the gut, or an organ that has a specific function (parenchymal organs)
- angiosarcoma of the head and neck or skin
- gastrointestinal stromal tumor (GST)
- orbit sarcoma (a type of eye cancer)
- desmoid tumor, also called fibromatosis
When doctors describe the stage, they may also use the words localized, regional, or metastatic. Localized means that the cancer is only found in one area of the body and has not spread to other parts of the body. Regional means near or around the place where it originated. Metastatic means in a part of the body farther from where the cancer started.
The stages of soft tissue sarcoma also depend on the grade. The grade describes how much cancer cells differ in appearance from normal cells, how quickly they grow and divide, and how likely they are to spread.
Find out more about cancer staging and the histological classification of soft tissue sarcoma.
The tumor is 5 cm (2 in) or less. Soft tissue sarcoma is low grade.
The tumor is over 5 cm (2 in). Soft tissue sarcoma is low grade.
The tumor is 5 cm (2 in) or less. The soft tissue sarcoma is high grade.
The tumor measures more than 5 cm (2 in) but not more than 10 cm (4 in). The soft tissue sarcoma is high grade.
The tumor measures more than 10 cm (4 in). The soft tissue sarcoma is high grade.
The cancer has spread to nearby lymph nodes or to other parts of the body (distant metastases), such as to the lungs or liver. It is also called metastatic soft tissue sarcoma. It can be of any rank.
Recurrent soft tissue sarcoma
Recurrence of soft tissue sarcoma means that the cancer comes back after treatment. If it reappears where it first started, it is called a local recurrence. If it reappears in tissues or lymph nodes near where it first started, it is called a regional recurrence. It can also reappear in another part of the body: this is called a recurrence or distant metastasis.
If the soft tissue sarcoma spreads
Cancer cells can spread from where they started to other parts of the body. This spread is called metastasis.
Understanding how a type of cancer usually grows and spreads helps your healthcare team plan your treatment and future care. Soft tissue sarcoma can spread almost anywhere in the body. Where it is most likely to do so depends on factors such as the type of soft tissue sarcoma and where it started in the body. If soft tissue sarcoma spreads, it can spread to the following parts of the body:
- tissues or regions surrounding the original location
- lungs – especially soft tissue sarcoma which originated in an arm or a leg
- liver – especially soft tissue sarcoma that started in the abdomen
- nearby lymph nodes – rare site of metastasis for most soft tissue sarcomas
Prognosis and survival for soft tissue sarcoma
If you have soft tissue sarcoma, you may be wondering about your prognosis. A prognosis is the act by which the doctor best assesses how cancer will affect a person and how they will respond to treatment. The prognosis and survival depend on many factors. Only a doctor who is familiar with your medical history, the type of cancer you have, the stage and other characteristics of the disease, the treatments chosen and the response to treatment can review all of this data together with survival statistics. to arrive at a prognosis.
A prognostic factor is an aspect of the cancer or a characteristic of the person, such as their age, that the doctor takes into account when making a prognosis. A predictor factor influences how cancer responds to a certain treatment. We often discuss prognostic and predictive factors together. They both play a role in choosing the treatment plan and establishing the prognosis.
The following are the prognostic or predictive factors for soft tissue sarcoma.
The size of a soft tissue tumor is an important prognostic factor. A tumor that is 5 cm or less has a better prognosis than a tumor that is more than 5 cm.
The grade tells you how quickly the cancer grows and how likely it is to spread. The grade also helps to establish the prognosis. Low-grade soft tissue sarcoma has a better prognosis because it is less likely to spread (metastasize) or come back (recur) than high-grade sarcoma.
A soft tissue tumor near the surface of the body (superficial tumor) has a better prognosis than a tumor that grows deep in the body. Tumors that are deep in the connective tissue surrounding muscles, nerves, blood vessels, and organs (fascia) tend to have a poor prognosis.
Location where the cancer started
Soft tissue sarcoma of the arm, leg, or trunk surface usually has a better prognosis than soft tissue sarcoma that starts in another part of the body.
Some types of soft tissue sarcoma tend to have a poorer prognosis, such as malignant tumor of the peripheral nerve sheath, leiomyosarcoma, desmoplastic small round cell tumor, and epithelioid sarcoma.
The lower the stage of soft tissue sarcoma at diagnosis, the better the prognosis. Stage 4 soft tissue sarcoma, or metastatic soft tissue sarcoma, has a poor prognosis. Soft tissue sarcoma that has invaded bones, nerves or vessels usually has a poor prognosis.
Surgery is the primary treatment for many soft tissue sarcomas. When you remove a tumor, you also remove a margin of healthy tissue around it. If cancer cells are observed in the margin, that is, the surgical margin is positive, the prognosis is poorer than if there were no cancer cells in the margin (negative surgical margin).
Surgery performed to remove the tumor
Surgery is the primary treatment for many soft tissue sarcomas. A soft tissue sarcoma that is completely removed by surgery has a much better prognosis than a soft tissue sarcoma that is not completely removed or will not be removed by surgery.
People with soft tissue sarcoma who are under the age of 50 tend to have a better prognosis than those over the age of 50.
Treatments for soft tissue sarcoma
If you have soft tissue sarcoma, your healthcare team will create a treatment plan just for you. It will be based on your health and specific cancer information. When your healthcare team decides which treatments to offer you, they take the following into consideration:
- grade and stage
- where the cancer started in your body
- the type of soft tissue sarcoma
- what you prefer or want
The care and treatment received by people with soft tissue sarcoma is often provided by a healthcare team experienced in the management of soft tissue sarcomas. In most cases, many specialists are required, including a radiologist, surgeon, medical oncologist, and radiation oncologist.
You may be offered one or more of the following treatments for soft tissue sarcoma.
Surgery is the primary treatment for most soft tissue sarcomas. Depending on the size and location of the tumor, one or more of the following types of surgery may be done.
Large local excision removes the cancer along with some of the normal tissue around it. It is used to treat soft tissue sarcoma that is only found in the area where it started (localized). A wide local excision can also be done when the soft tissue sarcoma has reappeared in the area where it started (local recurrence).
Limb conservation surgery removes a tumor in the arm or leg, that is, a limb, without removing the entire arm or leg. It is used to treat soft tissue sarcoma that is found only in one arm or leg and has not spread elsewhere in the body.
Mohs surgery involves removing the cancer in layers, little by little, until all is left. It may be used for soft tissue sarcoma located near the surface of the skin.
Reconstructive surgery repairs the skin and the area around the tumor that has been removed. When a large area has been removed to make sure there is no cancer at all, the doctor reconstructs that area using a little tissue from another part of the body.
Amputation is the removal of part or all of a limb. It is not usually used for soft tissue sarcoma. Amputation may be necessary if the cancer has invaded certain nerves, blood vessels and muscles, preventing the limb from functioning properly, or if there will be too many long-term problems after surgery.
Lymph node dissection involves removing a group of lymph nodes. It may be used for soft tissue sarcoma that has spread to nearby lymph nodes.
Lung metastasis surgery removes metastatic soft tissue sarcoma that has spread to a single area or to a few areas of the lungs.
In radiation therapy, high-energy rays or particles are used to destroy cancer cells. External beam radiation therapy can be given before or after surgery. This is most often done for high-grade soft tissue sarcoma to reduce the risk of the cancer coming back where it started.
In chemotherapy, anti-cancer, or cytotoxic, drugs are used to destroy cancer cells. It is most often used to treat advanced soft tissue sarcoma. It can also be given to reduce the risk of the cancer coming back. The most commonly used chemotherapy drug is doxorubicin (Adriamycin). Read more about chemotherapy.
Targeted therapy uses drugs to target specific molecules, such as proteins, on the surface or inside of cancer cells to stop the growth and spread of cancer while limiting damage to normal cells. It is usually given to people with advanced soft tissue sarcoma. Pazopanib (Votrient) is a targeted drug that may be used.
Follow-up after treatment is an important part of caring for people with cancer. You will need to have regular follow-up visits, especially during the first 5 years after treatment. These visits allow your healthcare team to monitor your progress and know how you are recovering from treatment.
Ask your doctor if there are clinical trials underway in your country for people with soft tissue sarcoma. Clinical trials aim to find new methods of preventing, detecting and treating cancer.
Supportive care for soft tissue sarcoma
Supportive care empowers people to overcome the physical, practical, emotional and spiritual barriers of soft tissue sarcoma. It is an important component of the care of people with this disease. There are many programs and services that help meet the needs and improve the quality of life for these people and their loved ones, especially after treatment is over.
Recovering from soft tissue sarcoma and adjusting to life after treatment is different for each person depending on the location of the cancer in the body, the stage of the cancer, the organs and tissues removed during the surgery, the type processing and many other factors. The end of cancer treatment can lead to mixed emotions. Even if the treatment is finished, there may be other issues that need to be addressed, such as coping with long-term side effects. A person who has been treated for soft tissue sarcoma may be concerned about the following.
Rehabilitation after treatment
Rehabilitation can be an important part of post-treatment recovery, especially for soft tissue sarcoma of the arm or leg. The type of rehabilitation you will undergo depends on the location of the cancer in the body and the type of surgery performed (limb conservation surgery or amputation).
Self-esteem and body image
Self-esteem is how we feel about ourselves. Body image is how we perceive our own body. Soft tissue sarcoma and its treatments can affect a person’s self-esteem and body image. This is often because cancer or treatments can cause changes in the body such as the following:
- scars and skin changes
- hair loss
- changes in mobility and body function
- loss of a limb
Some of these changes may be temporary. Others can last a long time or be permanent.
For many people, body image and how other people look is closely related to self-esteem and can be a source of real concern and significant distress. They may be angry or upset, afraid to go out, or fear being rejected by others, even though the effects of treatment may not be visible. The loss of function or mobility of a limb or the amputation of a limb can be particularly painful. Grieving over the loss of function or mobility of the limb or the amputated limb is often long, as does the emotional and practical adaptation to amputation.
Some people experience pain or sensory changes in the amputated limb. We then speak of phantom pain, or illusion of amputees. It occurs because nerves were cut and damaged during the surgery, which may cause the transmission of abnormal nerve impulses.
Symptoms of phantom pain in the amputated limb may include the following:
- burning sensation
- throbbing pain
Phantom pain usually begins 1 to 4 weeks after surgery and usually subsides within the first year. In some cases, this pain can become a long-term problem. Phantom pain can be made worse by stress and fatigue.
There are a number of ways phantom pain can be treated. Treatment options include the following:
- pain relievers and muscle relaxants
- stump shrinkage that puts pressure on the stump
- massage, heat or distractions
- additional surgery – the limb may need to be amputated even higher
Pain specialists can help treat long-term problems associated with phantom pain.
Fear the cancer will come back
Some types of soft tissue sarcoma are more likely to come back (recur). It is normal to be worried, but over worried will not be good for you.
Living with the possibility of the cancer coming back can be very stressful, and having to visit the doctor regularly can make it even more difficult. These visits can be a constant reminder of this. But regular follow-up with your doctor and other members of the healthcare team increases the chances that cancer will be detected earlier, when it is easier to treat.
During these visits, you can discuss your concerns and ask questions. You should report any signs and symptoms that may suggest the cancer has come back as soon as possible.
You might consider talking to a therapist or a close friend if the fear of the cancer coming back keeps you from moving forward. You can also join a support group or an online community whose participants are cancer survivors. Talking with other people who have the same fears as you may be helpful. Look for ways to help you cope, manage your stress, and improve your health. You could try the following:
- prayer (some people may find comfort in spiritual beliefs)
- mental imagery and relaxation
- physical activity
- healthy eating
Rehabilitation after treatment for soft tissue sarcoma
Rehabilitation is an important part of returning to activities of daily living after treatment for soft tissue sarcoma. You may need to get your body back on track or find new ways to do things you used to do. Rehabilitation differs for everyone depending on the location of the cancer in the body, the stage of the cancer, the organs or tissues removed during surgery, the type of treatment and many other factors. A person with soft tissue sarcoma may be concerned about the following:
- muscular weakness
- difficulty using and moving parts of the body, such as an arm or a leg
- difficulty in carrying out daily activities
Different health professionals may be part of your rehabilitation team including the following.
The physiotherapist helps you increase your strength, flexibility (range of motion) and mobility by, for example, helping you walk (gait training) and other activities of daily living.
The occupational therapist makes sure that you are able to perform your daily activities to the full. He may suggest that you use assistive devices or make changes at home, at work or at school so that you can do things yourself.
The prosthetist is specially trained to design, manufacture and fit artificial limbs called prostheses.
If you have been treated for soft tissue sarcoma, you may be concerned about the following aspects of rehabilitation.
Members of the rehabilitation team can help you recover from treatment for soft tissue sarcoma. Often they will meet with you after surgery and help you get well.
Rehabilitation after surgery
Rehabilitation is necessary after limb conservation surgery or amputation. Preserving limb surgery is more complex than amputation, and physical rehabilitation after this surgery is different from physical rehabilitation after amputation.
It may take up to 1 year of leg conservation surgery to learn to walk again without a cane, crutches or walker. Some people may still need a walking aid after rehabilitation.
After the amputation of a leg that has healed, a prosthesis may be fitted for you. You will learn how to use it during treatment. With proper physiotherapy, most people can walk again in 3 to 6 months. But it can vary depending on one’s recovery.
A rehabilitation healthcare professional will show you what exercises to do to keep your muscles strong and your joints mobile, which will aid in your recovery. Physical therapy exercises that help maintain range of motion and strengthen the arms and legs are very important for people who have had limb surgery. Some restrictions may apply after surgery until the area has healed. For example, you might be asked not to lift heavy objects, put too much pressure on the limb, or move it in a certain way.
Some people may also need to wear an orthotic or splint to help support their lower limbs to make sure they are within the restrictions. Most people will need a cane, crutches or walker at different times during their rehabilitation, which will help them walk after leg surgery. Sometimes a sling, orthosis or splint is necessary after upper limb surgery.
Rehabilitation after radiation therapy
People who receive radiation therapy for soft tissue sarcoma in a limb may experience joint stiffness and decreased range of motion in their joint and reduced muscle strength. It is important to continue to move the joint as normally as possible during and after treatment. Physiotherapy helps prevent and minimize disability after radiation therapy to a limb. An exercise program is often started at the start of treatment to help you stay strong and move properly.
Living with an artificial limb
The artificial limb is also called a prosthesis. It can be used after the amputation of an arm or a leg. Sometimes you cannot use a prosthesis. Members of the rehabilitation team can help you recover from the limb amputation. They show you how to use and care for your prosthesis, how to heal your stump and change the way you go about your entire daily activities.
The prosthetist makes an artificial limb adapted to each person. He can show you various artificial limbs. The necessary measures can be taken before or after the surgery. Taking measurements ensures that the prosthesis will fit properly. Sometimes a temporary prosthesis is used until the wound has healed enough to allow a permanent prosthesis to fit, which can take up to 3 months. The prosthesis may need to be readjusted if you lose or gain weight or if the size of the stump changes.
When you have to take care of your prosthesis and stump, you must learn to:
- keep the stump clean and dry and the skin in good condition;
- check the stump daily for any signs of skin damage;
- applying a bandage to the stump after surgery to help reduce swelling and give it shape, which helps prepare it for wearing the prosthesis;
- do exercises that strengthen the limb and maintain the range of motion of the joint;
use an elastic bandage or a stump covering cap before putting on the prosthesis and make sure it is cleaned often;
- put on the prosthesis and gradually increase the time it is worn;
- use your new prosthesis.
The prosthesis allows you to walk, run, play certain sports, and use your limb to carry out your activities of daily living. Artificial limbs can be very sophisticated, and some are specially designed for sports or swimming. It can take many months to learn how to use an artificial limb. Mobility aids such as a cane, crutches, a walker or a wheelchair are sometimes required to learn to walk with an artificial lower limb. Many people who wear an artificial limb regain the same level of activity as before.
The word “cancer” is a generic term for a large group of diseases that can affect any part of the body. We also speak of malignant tumors or neoplasms. One of the hallmarks of cancer is the rapid multiplication of abnormal growing cells, which can invade nearby parts of the body and then migrate to other organs. This is called metastasis, which is the main cause of death from cancer. Types of cancer (in alphabetical order of the area concerned):
Information: Cleverly Smart is not a substitute for a doctor. Always consult a doctor to treat your health condition.