What is sarcoma?
Sarcoma is a type of cancer found in connective tissues. It usually is found as a tumor, that is, a lump, bump, or mass. Sarcoma cancers are found in fat, blood vessels, nerves, bones, muscles, deep skin tissues, tendons, and cartilage. They are divided into two main groups—bone tumors and soft tissue tumors. These two main groups can be broken down into more specific sub-types based on the type of cells found in the tumor. There are over 100 different types of sarcoma tumors, most of which are very rare.
Of the 1.7 million people who are diagnosed with cancer in the United States each year:
- About 1% (17,000) will be diagnosed with sarcoma cancer.
- Some sarcomas are so rare that only a few people are diagnosed with that type each year.
- In children under age 18, where cancer is rare already, sarcomas are in the top 5 most diagnosed cancers.
Sometimes the diagnosis of cancer comes quickly, if a person has obvious symptoms and received prompt evaluation. However, diagnosing a new sarcoma can sometimes be challenging. Some reasons include:
- The symptoms might be vague. The symptoms are ones that many people experience, such as a lump bump or pain in a joint.
- Sarcoma tumors can form almost anywhere in the body and may be present for a long time before they are recognized.
- Sarcomas can be misdiagnosed. Since non-cancerous diagnoses are far more common, sarcoma tumors can be easily mistaken for trauma, a “pulled muscle” or a benign fatty tumor called lipoma.
Since sarcoma cancers are so rare, there is still a lot to discover about what causes these tumors. Researchers often study a group of sarcoma cancers together, which can make it difficult to understand the many nuances between them. However, combining these cancers in research may be the only way to find potential causes, treatment and cure for something very rare.
Cancer is basically a genetic disease.
Genes are like blueprints for the building blocks of your body. Changes in these genes are called mutations. There are many different types of mutations that can occur. Some genetic variants may be present at birth, others may occur throughout a person’s lifetime. These mutations can be harmful, beneficial, or have no effect on the risk of developing cancer. Sometimes, however, mutations in these genetic blueprints can cause cells to grow out of control and become cancerous.
Most of the time, gene mutations happen spontaneously. Sometimes they can be inherited from a parent and/or present at birth. Genes associated with cancer predisposition are thought to contribute to about 5 to 10% of all cancers, and small number are associated with sarcomas. Some of these risks can be detected by genetic testing of blood or saliva samples.
Environmental causes
Certain exposures can increase a person’s chances of developing sarcoma cancers. In particular, a certain viral infection, known as Human Herpes virus 8 (HHV-8), can lead to Kaposi’s Sarcoma cancer. This tends to occur in those with Acquired Immunodeficiency Syndrome (AIDS). Additionally, having received ionizing radiation and chemotherapy for a previous cancer can increase the risk of sarcoma tumors later in life (although this is rare).
Types of sarcoma cancers
Soft Tissue Sarcomas
Soft tissue sarcoma tumors can occur in muscles, fat, nerves, blood vessels, tendons, and other soft tissues that support, surround, or protect body organs and joints. They are found anywhere in the body, from head to toe, although more often in the extremities, such as arms or legs.
The most common types of soft tissue sarcoma cancers are described below. This is not intended to be a complete list and may not contain the specific sarcoma cancer type that you (or your loved one) have been diagnosed with. It is always best to work closely with your own oncologist and sarcoma cancer team who will help get you all the additional information that you will want to know.
Type:
Soft tissue tumors
How does it appear?
- Most patients have no symptoms (i.e., are asymptomatic).
- They will appear as a raised, purple area of skin that resembles a bruise, growing over time.
- May be painful or bleed at the tumor site.
- Can occur anywhere in the body, but most often in the skin, head, or neck.
General Description
Angiosarcoma is a connective tissue sarcoma tumor that grows from the cells that line blood vessels and lymph channels (“Angio”). They represent approximately 1-2% of all sarcoma cancers and is seen most commonly in individuals above the age of 70, but can also occur at any age.
- Can be caused by “lymphedema”, which is swelling due to the body unable to handle fluid that collects in tissues, often in the arms and legs.
- Other causes of these tumors include radiation exposure and/or exposure to chemicals, such as vinyl chloride, arsenic, and thorium dioxide.
Related links
Type:
Soft tissue tumors, not considered malignant
How does it appear?
- Most patients have no symptoms (i.e., are asymptomatic).
- There may be pain or swelling or a lump where the tumor is located.
- Can occur anywhere in the body, but most often are found in the abdomen, arms, shoulders or legs.
- They can be slow or fast growing.
General Description
Desmoid tumors are benign (not cancerous) tumors that grow from connective tissue, often deep in the soft tissues of the body. They are not considered sarcoma cancers but patients with Desmoid are often treated by experts in sarcoma cancer care. Typically slow growing, they spread locally and can be very invasive to the tissues where they are found.
- These tumors are occasionally seen in familial adenomatous polyposis (FAP), which is an inherited genetic condition.
- They can begin in areas of “trauma” or previous surgery, often in the wall of the abdomen, abdomen, arms or legs.
- Desmoid tumors may regress (shrink) on their own, stay the same size, or sometimes grow. If surgically removed they have a high risk of recurring in the same place.
Related links
Type:
Soft tissue tumors
How does it appear?
- Typically, it will appear as a small, painless lump under the skin of a finger, hand, forearm, lower leg, or foot.
- May be a sore or open wound that doesn’t heal.
General Description
Epithelioid sarcoma is a connective tissue tumor that grows from epithelial cells, which are cells that line the surfaces inside and outside of the body, including the skin, urinary tract, intestines, and other organs.
- Most commonly seen among teenagers and young adults.
Related links
Type:
Soft tissue tumors
How does it appear?
- Appears as painless, purple-ish spots on the legs, feet, or face.
- Can also appear in genital areas, the mouth, lymph nodes, and, in severe cases, the digestive tract or lungs.
General Description
Kaposi’s sarcoma is a connective tissue tumor named after the Hungarian doctor who discovered it. These tumors grow from cells that line blood and lymph vessels.
- Is caused by an infection with a virus called Human Herpes virus 8 (HHV-8).
- Immune system damage or suppression (especially in patients with organ transplant or HIV/AIDs) allows the cells infected with HHV-8 virus to multiply and form tumors.
Related links
Type:
Soft tissue tumors
How does it appear?
- Tumors located deep in the body that may not present with any symptoms until they grow larger.
- May appear as painless masses below the skin.
General Description
Leiomyosarcoma is a connective tissue tumor that grows from the type of muscle found in the intestines, gastrointestinal tract, large blood vessels, and in the uterus of women.
- Accounts for 5-10% of all soft tissue sarcoma tumors.
- No proven relation to any environmental exposures or lifestyle choices.
- Genetic conditions (Hereditary Retinoblastoma and Li Fraumeni Syndrome) may predispose patients to Leiomyosarcoma cancer.
Related links
Type:
Soft tissue tumors
How does it appear?
- Can occur anywhere in the body, but are mostly found in the limbs, muscles and abdomen.
- They are painless and slow growing so many patients may be symptom free (asymptomatic).
- May appear as a growing lump under the skin of the arms or legs.
General Description
Liposarcoma is a connective tissue tumor that grows from fat cells located in deep connective tissues.
- One of the most common soft tissue tumors mostly found in adults, but can occur at any age.
- Some risk factors include exposure to chemical. carcinogens, previous ionizing radiation and chemotherapy treatments.
Related links
Type:
Soft tissue tumors
How does it appear?
- May appear as a painless mass underneath the skin.
- Are commonly found in the head and neck region, followed by the extremities, such as arms and legs.
General Description
Rhabdomyosarcoma is a connective tissue tumor that grows from skeletal muscles, such as the muscles in your arms, legs, abdominal wall, and back.
- Mostly occurs in children and young adults, but can occur at any age.
- Is associated with Neurofibromatosis, Li-Fraumeni, Beckwith-Wiedemann, and Costello syndromes.
Related links
Type:
Soft tissue tumors
How does it appear?
- Slow growing, painless mass found in the arms or legs where the joints are formed.
- Masses may be painful if involving nerves.
General Description
Synovial sarcoma tumors develop from immature cells that line the inside of a joint (synovium).
- Typically affects people ages 15-40, but can occur at any age.
- No definite risk factors.
Has been associated with Chromosome 18 and Chromosome X Translocations.
Related links
Type:
Soft tissue tumors
How does it appear?
- Symptoms vary based on size and location of tumors.
- Small tumors may be asymptomatic while larger tumors may cause vomiting, GI bleeding, abdominal pain, nausea and weight loss.
General Description
A Gastrointestinal Stromal Tumor (GIST) is a sarcoma cancer that develops from cells that help coordinate your intestinal movements throughout the day.
- Most commonly occurs in adults between the ages of 50 and 70, but can occur at any age.
- Some association with rare genetic syndromes
Related links
Type:
Soft tissue tumors
How does it appear?
- Symptoms vary based on the size and location of tumors.
- May experience pain, numbness, weakness, or a burning/tingling sensation in the extremities at the tumor site.
General Description
A malignant nerve sheath tumor (MPNST) is a sarcoma cancer that develops from the cells that surround and protect the nerves of the peripheral nervous system (outside of the brain and spinal cord).
- Most commonly affects young and middle-aged adults.
- 50% of these tumors occurs in the setting of a genetic condition called Neurofibromatosis 1.
Related links
Type:
Soft tissue tumors
How does it appear?
- Slow-growing, painless lump on extremities, such as arms or legs, that extends deep into tissues.
- Symptoms vary based on size and location of tumors.
General Description
Myxofibrosarcoma is a connective tissue tumor that surrounds and separates muscles from each other and from the skin.
- Mostly occurs in people between the ages of 50 and 70.
- Slightly more common in men than women.
- Difficult to distinguish from other soft tissue sarcoma tumors, which can result in delayed diagnosis.
Related links
Type:
Soft tissue tumors
How does it appear?
- Often starts as a small, firm patch of skin that can be various tones of purple or red.
- Grows slowly over months to years and can become a raised nodule under the skin.
General Description
A Dermatofibrosarcoma Protuberans (DFSP) tumor arises from connective tissue in the middle layer of the skin, known as the dermis layer.
- Typically affects people ages 20-50.
- Women and African Americans are more at risk.
- Scars formed from burns or surgery can be risk factors.
- There are many subtypes of these tumors that are based on the type of cells found in the dermis layer. These subtypes include Bednar tumors, Myxoid Dermatofibrosarcoma Protuberans tumors, and Giant Cell Fibroblastoma.
Related links
Type:
Soft tissue tumors
How does it appear?
- Appears as a lump in the extremities, such as arms or legs, and can also occur behind abdominal organs.
- Although it can be found in either soft tissues or bone, it is considered a soft tissue tumor.
General Description
An Undifferentiated Pleomorphic sarcoma tumor has cells of different sizes and shapes. The tissue type it originates from is not able to be identified, which is known as “undifferentiated”.
- Formerly known as Malignant Fibrous Histiocytoma.
- Typically affects older adults.
- Previous ionizing radiation therapy is a risk factor.
Related links
Bone sarcomas
Bone Sarcoma tumors develop in the bone tissue itself or in the cartilage that provides cushion between bones in the joints.
- Approximately 3,600 new cases of bone sarcoma cancers are diagnosed in the United States each year, accounting for less than 0.2% of all cancers.
- Unexplained pain that does not go away, and/or a lump that may appear under the skin, are the most common symptoms of this type of cancer. As a tumor gets bigger, it can cause a joint, such as the knee or elbow, to swell, often being mistaken for an injury instead of a bone tumor. These tumors can also weaken the bones, causing easy fractures.
- A variety of imaging, such as X-Rays, CT scan or an MRI scan, can decide if a lump or mass is a bone tumor.
- An orthopedic bone surgeon, who is very familiar with sarcoma cancers, should be consulted for a diagnosis and surgical and treatment plans.
- Bone sarcomas are diagnosed by either removing the entire tumor or a small piece of it through a surgical biopsy.
The most common types of bone sarcomas are described below. Working closely with your oncologist and cancer team will help get you all the information you need. This is not intended to be a complete list.
Type:
Bone tumors
How does it appear?
- Can experience unexplained pain in an affected area, such as in the knee, which can cause a limp or similar disability.
- Easy fractures can occur, due to the bone weakening by the tumor.
- Most common tumor sites are found in the femur and tibia, both by the knee, and the humerus, near the shoulder.
General Description
Osteosarcoma tumors come from bone forming cells, called osteoblasts, in immature bone tissue.
- Most common bone tumors.
- Most commonly diagnosed in children and young adults, under the age of 25.
- Can also occur in older adults over 40.
Related links
Type:
Bone tumors
How does it appear?
- May feel a hard lump under the skin, causing pain, swelling, or limited movement.
- Usually starts in the long bones of the arms, legs, or pelvis, often near the joints.
General Description
Chondrosarcoma is a sarcoma that arises from cells that produce cartilage, which is connective tissue surrounding bones and other organs in the body.
- Typically affects adults between the ages of 20 and 60.
- These tumors are commonly found in men more than women.
- This type of sarcoma is associated with other conditions, such as Ollier’s Disease, Maffucci Syndrome, and Multiple Hereditary Exostoses.
Related links
Type:
Bone tumors
How does it appear?
- May feel a lump under the skin causing pain, swelling, or limited movement.
- Typically develops in the pelvis, ribs and long bones (femur, tibia, humerus).
General Description
Ewing’s sarcoma is named after the American doctor who discovered this sarcoma tumor in the 1920’s. It is a tumor that arises from immature bone cells, but can also occur outside the bone in cartilage, nerves, or soft tissue.
- Second most common type of bone cancer, behind Osteosarcoma.
- Most often occurs in children and young adults between the ages of 10 and 20, but can also rarely occur in older adults.
- Is more common in males than females.
- Most cases involve a fusing of genetic material between chromosomes 11 and 22, causing the Ewing’s sarcoma gene (EWS) to be “turned on”.
Related links
Type:
Bone tumors
How does it appear?
- Can occur anywhere within the spine and the base of the skull/brain, causing pain and nerve symptoms, including tingling, numbness and weakness.
General Description
Chordoma sarcoma arises from cells that become the discs of the spine in the embryo. These cells typically go away after birth, but rarely some remain and become cancerous.
- Occurs most often in adults between ages 40 and 60, more in men than women.
- Can be caused by some hereditary conditions, such as familial Chordoma and Tuberous Sclerosis Complex.