Sarcoma arises in the connective tissue of the body. The connective tissue includes muscle, bone, fat, nerve, cartilage, blood vessel,
and deep skin tissue. Connective tissue is present throughout the body, so sarcoma can occur in any location. Sarcomas are divided
into two main groups:
- bone sarcomas
- soft tissue sarcomas.
Two thirds of sarcomas are located in the extremities (arms or legs). Because it is so rare and it takes multiple forms in multiple locations, sarcoma is difficult to detect, often misdiagnosed and complex to treat.
Source: Sarcoma Alliance: What is Sarcoma?
Sarcoma Types
Adult Sarcomas
Soft Tissue Sarcomas
There are many types of soft tissue sarcoma. The cells of each type of sarcoma look different under a microscope, based on the type of soft tissue in which the cancer began.
Soft Tissue Sarcoma Subtypes
- Liposarcoma
- Fibrosarcoma
- Dermatofibrosarcoma Protuberans
- Malignant Fibrous Histiocytoma
- Synovial Cell Sarcoma
- Epithelioid Sarcomas
- Rhabdomyosarcomas
- Leiomyosarcoma
- Uterine Sarcoma
- Gastrointestinal Sarcomas(GIST)
- Myxoma, Mesenchyomas
- Vascular Sarcomas(containing many blood vessels)
- Malignant Schwannoma
- Neurosarcomas
- Alveolar Soft-Parts Sarcoma
- Kaposi's Sarcoma
- Ewing's Sarcoma
- Chondrosarcoma
- Malignant Fibrous Histiocytoma
- Osteosarcoma
Pediatric Sarcoma
Pediatric sarcomas are of three types. They are called Pediatric Sarcoma because of their greater prevalence among children compared to adults. However, they can occur in young people and adults as well.
- Rhabdomyosarcomas - tumors in the skeletal (striated) muscle and one of the most common types of soft tissue sarcoma. Rhabdomyosarcomas are often widely spread by the time a diagnosis is made. Treatment generally consists of surgery and combination chemotherapy (that is, more than one drug is used).
- Osteosarcoma - the most common type of bone cancer. Osteosarcoma usually starts in osteoblasts, which are a type of bone cell that becomes new bone tissue. Osteosarcoma is most common in teenagers. It commonly forms in the ends of the long bones of the body, which include bones of the arms and legs. In children and teenagers, it often forms in the bones near the knee.
- Ewing's Sarcoma - cancer that occurs principally in bone, most commonly affecting the long bones of the arm and leg, the pelvis, and the ribs. However, Ewing's Sarcoma can also occur in soft tissues and as such is considered a "family" of cancers that include Peripheral Neuroectodermal Tumor (PNET). It is more common in girls than boys and is rare among Africans and African-Americans. Multi-drug chemotherapy is the primary treatment in association with surgery and radiation.
National Cancer Institute Sarcoma Alliance:
Risk Factors
Having certain inherited disorders can increase the risk of developing adult soft tissue sarcoma.
Anything that increases your risk of getting a disease is called a risk factor. Risk factors for soft tissue sarcoma include the following inherited disorders:
- Retinoblastoma.
- Neurofibromatosis type 1 (von Recklinghausen disease or NF1).
- Tuberous sclerosis.
- Familial adenomatous polyposis (FAP).
- Li-Fraumeni syndrome.
- Werner syndrome.
- Basal cell nevus syndrome.
Other risk factors for soft tissue sarcoma include past treatments with radiation therapy during childhood or radiation for certain types of cancer:
- Retinoblastoma
- Breast cancer
- Lymphoma
- Cervical cancer
Source: National Cancer Institute: General Information About Adult Soft Tissue Sarcoma
Risk factors for osteosarcoma include the following:
- Age and height: The risk of osteosarcoma is highest during the teenage growth spurts. Children with osteosarcoma are usually tall for their age. There may be a link between rapid bone growth and the risk of tumors forming.
- Gender: Osteosarcoma is more common in males than in females.
- Race/ethnicity: Osteosarcoma is slightly more common in African Americans than in whites.
- Radiation to bones: Young people who were treated with radiation for an earlier cancer have a higher risk of getting osteosarcoma later. Normal X-rays do not seem to increase the risk.
- Certain bone diseases: Children and teens with certain non-cancer bone diseases have an increased risk of getting osteosarcoma later, usually as an adult. Some of these diseases include Paget disease of bone and multiple hereditary osteochondromas.
- Certain cancer syndromes: Children with certain rare, inherited cancer syndromes have an increased risk of getting osteosarcoma. One of these syndromes is Li-Fraumeni syndrome. When children with retinoblastoma (a rare eye cancer) are treated with radiation it raises the chance of osteosarcoma in the bones of the skull.
Source: American Cancer Society - Osteosarcoma Overview
Risk factors for uterine sarcoma include the following:
- Past treatment with radiation therapy to the pelvis.
- Treatment with tamoxifen for breast cancer. A patient taking this drug should have a pelvic exam every year and report any vaginal bleeding (other than menstrual bleeding) as soon as possible.
Source: American Cancer Society - Uterine Sarcoma
Prevention/Screening
Prevention
What causes soft tissue sarcomas? Scientists still don't know exactly what causes most cases of soft tissue sarcoma.
What causes Osteosarcoma? The exact cause of most osteosarcomas is not known.
The only way to prevent some sarcomas is to avoid exposure to risk factors whenever this is possible. However, most sarcomas develop in people with no known risk factors, so there is no way known at this time to prevent most cases. And for people receiving radiation therapy, there is usually little choice.
Source: American Cancer Society - Sarcoma - Adult Soft Tissue Cancer and Osteosarcoma: Can osteosarcoma be prevented?
Screening
People with a strong family history of sarcomas or other cancers occurring at a young age may wish to discuss the benefits and disadvantages of genetic testing with their doctor. The genetic testing results should always be explained by a genetic counselor or a specially trained doctor who can interpret the results and advise high-risk patients about early cancer detection.
Families with a history of certain inherited conditions caused by mutated tumor suppressor genes have an increased risk of developing soft tissue sarcomas. The mutated genes can be detected by genetic testing, so family members should discuss this option with their doctors. They should let their doctor know about any lumps or growths right away.
No screening tests and exams are recommended for people who have no family history of sarcoma or other sarcoma risk factors. For these people, the best approach to early detection is to notify their health care professional of any unexplained lumps or growths or other symptoms that may be caused by a soft tissue sarcoma.
Source: American Cancer Society - Can soft tissue sarcomas be found early?.
Symptoms
Soft tissue sarcomas
Possible signs of adult soft tissue sarcoma include a lump or swelling in soft tissue of the body.
A sarcoma may appear as a painless lump under the skin, often on an arm or a leg. Sarcomas that begin in the abdomen may not cause symptoms
until they become very large. As the sarcoma grows larger and presses on nearby organs, nerves, muscles, or blood vessels, symptoms may include:
- Pain
- Trouble breathing
Source: National Cancer Institute - General Information About Adult Soft Tissue Sarcoma.
Osteosarcoma and malignant fibrohistocytoma(MFH)
Possible signs of osteosarcoma and MFH include pain and swelling over a bone or a bony part of the body.These and other symptoms may be caused by osteosarcoma or MFH. Other conditions may cause the same symptoms. A doctor should be consulted if
any of the following problems occur:
- Swelling over a bone or bony part of the body.
- Pain in a bone or joint.
- A bone that breaks for no known reason.
Source: National Cancer Institute - General Information About Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
Take Action
Clinical Trials Currently Enrolling at the University of Michigan
1. UMMC 2009.085 Randomized, Double-Blinded, Placebo-Controlled, Multi-institutional, Phase II.5 Study of AZD0530, a Selective Src Kinase Inhibitor, in Patients with Recurrent Osteosarcoma Localized to the Lung. Primary Investigator: Dr. Scott Schuetze
2. UMCC 2008.081 An Open-label, Multi-center, Phase 2 Study of Denosumab in Subjects with Giant Cell Tumor of Bone. Primary Investigator: Dr. Scott Scheutze
3. UMCC 2007.016/SARC 006 A phase II Trial of Neoadjuvant Chemotherapy in Sporadic and Neurofibromatosis Type I Associated High Grade Unresectable Malignant Peripheral Nerve Sheath Tumor. Primary Investigator: Dr. Scott Schuetze
4. UMCC 2010.001 Randomized, Double-Blind, Phase II Trial of Ganglioside Vaccine +/- Adjuvant OPT-821 in Metastatic Sarcoma Patients. Primary Investigator: Dr. Rashmi Chugh
Clinical Trials Enrolling Nationally
Resources
University of Michigan
Multidisciplinary Sarcoma Clinic
Sarcoma of Bone and Soft Tissues Information Guide
Psychology Oncology Program
Devoted to relieving the numerous negative social and psychological effects of the disease. The Program is comprised of staff from a number of
U-M departments, all specializing in helping people face the emotional issues unique to cancer. For more information, call 877-408-PFSS(7377).
Support Group
Young Adults with Cancer(patients ages 18 to 30): walk-in, meets 1st Tuesday of each month, from 7-9 pm in the Cancer Center.
National Resources
