Sarcoma

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A sarcoma is a rare kind of cancer. Sarcomas are different from the much more common carcinomas because they happen in a different kind of tissue. Sarcomas grow in connective tissue -- cells that connect or support other kinds of tissue in your body. These tumors are most common in the bones, muscles, tendons, cartilage, nerves, fat, and blood vessels of your arms and legs, but they can happen anywhere.

When the term sarcoma is part of the name of a disease, it means the tumor is malignant (cancer).

Although there are more than 50 types of sarcoma, they can be grouped into two main kinds: soft tissue sarcoma and bone sarcoma, or osteosarcoma. About 12,000 cases of soft tissue sarcoma and 3,000 cases of bone sarcomas were seen in the U.S. in 2014.
Sarcomas can be treated, often by having surgery to remove the tumor.

Soft Tissues Sarcoma

Soft tissue sarcomas can develop from soft tissues like cartilage, fat, muscle, fibrous tissue, blood vessels, and other connective or supportive tissues of the body. They can be found in any part of the body. Most of them develop in the arms or legs. They can also be found in the trunk, head and neck area, internal organs, and the area in back of the abdominal cavity (known as the retroperitoneum).

There more than 50 different types of soft tissue sarcomas (not all are listed here), such as:

• Adult fibrosarcoma usually affects fibrous tissue in the legs, arms, or trunk. It is most common in people between the ages of 20 and 60, but can occur in people of any age, even in infants.
• Alveolar soft-part sarcoma is a rare cancer that mostly affects young adults. These tumors most commonly occur in legs.
• Angiosarcoma can develop either from blood vessels (hemangiosarcomas) or from lymph vessels (lymphangiosarcomas). These tumors sometimes start in a part of the body that has been treated with radiation. Angiosarcomas are sometimes seen in the breast after radiation therapy and in limbs with lymphedema.
• Clear cell sarcoma is a rare cancer that often develops in tendons of the arms or legs. Under the microscope, it has some features of malignant melanoma, a type of cancer that develops from pigment-producing skin cells. How cancers with these features start in parts of the body other than the skin is not known.
• Desmoplastic small round cell tumor is a rare sarcoma of adolescents and young adults, found most often in the abdomen.
• Epithelioid sarcoma most often develops in tissues under the skin of the hands, forearms, feet, or lower legs. Adolescents and young adults are often affected.
• Fibromyxoid sarcoma, low-grade is a slow growing cancer that most often develops as a painless growth in the trunk or arms and legs (particularly the thigh). It is more common in young to middle aged adults. It is also sometimes called an Evans’ tumor.
• Gastrointestinal stromal tumor (GIST): This is a type of sarcoma that develops in the digestive tract. It is covered in Gastrointestinal Stromal Tumor (GIST).
• Kaposi sarcoma: This is a type of sarcoma that develops from the cells lining lymph or blood vessels. It is covered in Kaposi Sarcoma.
• Liposarcomas (includes dedifferentiated, myxoid, and pleomorphic liposarcomas) are malignant tumors of fat tissue. They can develop anywhere in the body, but they most often develop in the thigh, behind the knee, and inside the back of the abdomen. They occur mostly in adults between 50 and 65 years old.
• Leiomyosarcoma
• Malignant mesenchymoma is a rare type of sarcoma that shows features of fibrosarcoma and features of at least 2 other types of sarcoma.
• Malignant peripheral nerve sheath tumors include neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas. These are sarcomas that develop from the cells that surround a nerve.
• Myxofibrosarcomas, low-grade are most often found in the arms and legs of elderly patients. They are most common in or just under the skin and there might be more than one tumor nodule.
• Rhabdomyosarcoma: This is the most common type of soft tissue sarcoma seen in children and is discussed in Rhabdomyosarcoma.
• Synovial Sarcoma is a malignant tumor of the tissue around joints. The most common locations are the hip, knee, ankle, and shoulder. This tumor is more common in children and young adults, but it can occur in older people.
• Undifferentiated pleomorphic sarcoma, previously known as malignant fibrous histiocytoma (MFH), is most often found in the arms or legs. Less often, it can start inside at the back of the abdomen (the retroperitoneum). This sarcoma is most common in older adults. Although it mostly tends to grow locally, it can spread to distant sites.

Bone Sarcomas

Sarcomas that most often start in bones, such as osteosarcomas, and sarcomas that more often occur in children, such as the Ewing Family of Tumors and Rhabdomyosarcoma.

• Osteosarcomas (Osteogenic Sarcoma)
  • Osteosarcoma is the most common bone cancer.
  • It starts in bone cells that make new bone tissue.
  • It usually forms at the end of long bones, such as the large bones of the upper arm (humerus) and the leg (femur and tibia).
  • It is most common in teenagers and in adults older than 65 years.
  • It accounts for approximately 5% of the tumors in childhood.
  • In children and adolescents, 80% of these tumors arise from the bones around the knee. ** It is slightly more common in males than females.
  • It is the most common primary malignant bone tumor with the exception of myeloma.
  • Although osteosarcoma is a common malignant bone tumor, it is still rare with less than 1,000 new cases each year in the United States.
  • When osteosarcoma occurs in adults over age 40 it is usually associated with a pre-existing condition such as Paget’s disease.
• Ewing's Sarcoma
  • Ewing’s sarcoma is a very rare type of cancerous tumor that grows in your bones or the soft tissue around your bones, such as cartilage or the nerves.
  • It is most common in the bones of the lower body such as the pelvis, tibia (shin), fibula (shin), and femur (thigh).
  • It usually affects people from the ages of 10 to 20 and has a high rate of being cured.
  • Ewing’s sarcoma affects about 200-250 children and young adults every year in the United States and shows up slightly more often in males.
  • While adults can get Ewing’s sarcoma, it is uncommon.
  • It mostly affects white people and has rarely been found in African Americans or Asian Americans, but it is more likely to occur in people of European descent.
• Chondrosarcoma
  • Chondrosarcoma is a cancer that develops from the cells that produce cartilage.  
  • A little less than one-third of bone sarcomas are chondrosarcomas.
  • While the disease can affect people of any age, unlike most other forms of skeletal system cancer, it is more common among older people than among children.
  • Also unlike the other bone cancers chondrosarcoma is more often found in the spine and pelvis than in legs or arms. 

Epidemiology

Sarcomas are quite rare with only 15,000 new cases per year in the United States. Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year.

In 2014, it is estimated that approximately 12,000 cases of soft tissue sarcoma and 3,000 cases of bone sarcomas will be diagnosed in the United States; approximately 4,700 and 1,500 people are expected to die from soft tissue and bone sarcomas, respectively.

Sarcomas affect people of all ages. Approximately 50% of bone sarcomas and 20% of soft tissue sarcomas are diagnosed in people under the age of 35. Some sarcomas, such as leiomyosarcoma, chondrosarcoma, and gastrointestinal stromal tumor (GIST), are more common in adults than in children. Most high-grade bone sarcomas, including Ewing's sarcoma and osteosarcoma, are much more common in children and young adults.

Soft tissue and bone sarcoma incidence rates have increased over the past 35 years. Soft tissue sarcomas are more common than bone sarcomas. There are several subtypes of both soft tissue sarcoma and bone sarcoma, but the exact number of Americans with each sarcoma subtype is unknown. The 5-year relative survival rate for both bone and soft tissue sarcoma is approximately 65 percent.

Number of New Cases and Deaths per 100,000 for Bone/Joint Cancer: The number of new cases of bone and joint cancer was 0.9 per 100,000 men and women per year. The number of deaths was 0.4 per 100,000 men and women per year. These rates are age-adjusted and based on 2010-2014 cases and deaths. In 2017, it is estimated that there will be 3,260 new cases of bone and joint cancer and an estimated 1,550 people will die of this disease. (from: NIH Surveillance, Epidemiology, & End Results Program - Cancer Stat Facts: Bone & Joint Cancer)

Surviving Sarcoma

Most people diagnosed with a soft tissue sarcoma are cured by surgery alone, if the tumor is low-grade; that means it is not likely to spread to other parts of the body. More aggressive sarcomas are harder to treat successfully.

The survival rate for osteosarcoma is between 60% and 80% if the cancer has not spread. It is more likely to be cured if all of the cancer can be removed by surgery.

In the U.S., July is widely recognized as Sarcoma Awareness Month. The U.K. has a Sarcoma Awareness Week July led by Sarcoma UK:the bone and soft tissue cancer charity.

In December 2016, British medical company MidMeds launched Sacoma Awareness Day in the UK, with the sole aim of raising awareness of the cancer. The event gained both local and national press attention

For Additional Reading:

• WebMD Sarcoma Symptoms, Types, Causes, Treatments
• Cancer.org - Soft Tissue Sarcoma
• Wikipedia Sarcoma
• Wikipedia - Soft-Tissue Sarcoma
• National Cancer Institute Bone Cancer-Patient Version
• Sarcoma alliance - What is Sarcoma?
• Sarcoma alliance - Soft Tissue Sarcoma Subtype Information
• Sarcoma alliance - Bone Sarcoma & Subtypes
• National Cancer Institute - A Snapshot of Sarcoma - Incidence & Mortality

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