About sarcomas

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 groupsbone tumors and soft tissue tumors, and while all sarcoma tumors share certain characteristics, they are further sub-classified based on the type of cells found in the tumor. There are well over 100 different types of sarcoma tumors, most of which are very rare.

Of the over 1.7 million people who are diagnosed with any cancer in the United States each year, 

  • About 1% of them will be diagnosed with sarcoma cancer. 
  • Some sarcomas are so rare that only a few people are diagnosed each year.
  • In children under age 18, where cancer is rare to begin with, sarcomas are actually one of the top five most commonly diagnosed cancers.

Diagnosing a new sarcoma cancer can be hard for many reasons. The tumor can form almost anywhere in the body. The symptoms are ones that many people experience-a lump, a bump, or pain in a joint. They are easy to misdiagnose. Factors that determine treatment and recurrence include the type of tumor, where it is found, how far it has spread, and whether the cells have a known treatment. Even after all of these characteristics are known, it’s important to note that each person diagnosed with these rare sarcoma tumors may react differently to their disease and treatment of these tumors. Although there aren’t any studies nor statistics that can accurately predict an individual patient’s chances of survival, sarcoma cancer survivors have more often than not defied all the odds, despite what seems to be an impossible challenge at the onset of their diagnosis.

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.

Genetic causes

Genes are like blueprints for the building blocks of your body. Mutations, or changes, in genes cause too many or too few building blocks inside the body. Although there are many genes in the body that can prevent these mutations from happening, sometimes they fail and can lead to cancer tumors, including sarcoma tumors.

An example is the p53 gene (associated with Li-Fraumeni syndrome), which is linked with one of these genetic mutations. When this happens, it causes cells to grow and divide uncontrolled, thus leading to cancerous tumors, which can increase the likelihood of bone and soft tissue sarcoma tumors to form. In some cases, although not in all, parents can also pass these uncontrolled, spontaneous genetic mutations to their children.

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

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.

Related links