Sarcoma is cancer that begins in the connective tissues of the body. Connective tissue includes bone, muscle, fat, blood vessels, nerves, tendons, and the lining of the joints (called synovial tissue).
Soft tissue sarcomas
Soft tissue sarcomas occur in muscles, tendons, fat, blood vessels, nerves, and synovial tissues. They have similar symptoms and are generally treated in similar ways. Soft tissue sarcomas can develop anywhere in the body.
The following list shows the type of sarcoma associated with each type of soft tissue:
- Fibrous tissue (tissue that holds bones, muscles, and organs in place): fibrosarcoma, malignant fibrous histiocytoma (MFH)
- Fatty tissue: liposarcoma
- Smooth muscle (for example, the muscle in the uterus): leiomyosarcoma
- Skeletal muscle (for example, the muscles used to walk or run): rhabdomyosarcoma
- Blood and lymph vessels: epithelioid hemangioendothelioma, angiosarcoma, lymphangiosarcoma, Kaposi sarcoma
- Perivascular tissue (tissue near or around blood vessels): glomangiosarcoma, malignant hemangiopericytoma
- Synovial tissue (tissue lining joints, tendon sheaths, and fluid-filled sacs between tendons and bones): synovial sarcoma
- Peripheral nerves: malignant granular cell tumor, malignant peripheral nerve sheath tumor (also called malignant schwannoma or neurofibrosarcoma)
- Mesenchymal cells (cells that develop into connective tissue, blood vessels, and lymphatic tissue): gastrointestinal stromal tumor (GIST), malignant mesenchymoma
Other types of soft tissue sarcomas include alveolar soft part sarcoma, epithelioid sarcoma, desmoplastic small cell tumor, and clear cell sarcoma. Scientists are studying the types of tissue in which these sarcomas begin. Many sarcomas result from specific genetic changes, and understanding these changes can help classify the tumors.
Other types of sarcoma
Osteosarcoma begins in osteoid tissue in the bone, most often in the knee or upper arm. Osteosarcoma occurs most commonly between ages 10 and 19. However, people over age 40 who have other conditions such as Paget disease (a benign condition in which abnormal bone cells develop) are at increased risk of this cancer.
Chondrosarcoma begins in cartilage tissue. Cartilage pads the ends of bones and lines the joints. Chondrosarcoma occurs most often in the pelvis, upper leg, and shoulder. Chondrosarcoma occurs mainly in adults over age 40. The risk increases with advancing age. This disease rarely occurs in children and young adults.
The Ewing's Sarcoma Family of Tumors (ESFTs) includes the following cancers:
- Ewing's tumor of bone (ETB or Ewing's sarcoma of bone)
- Extraosseous Ewing's (EOE) tumors
- Primitive neuroectodermal tumors (PNET or peripheral neuroepithelioma)
- Askin tumors (PNET of the chest wall)
ESFTs occur most commonly along the backbone, in the pelvis, and in the legs and arms. These cancers occur most often in children and adolescents under age 19, and boys are affected more often than girls.
Kaposi sarcoma causes purple lesions to grow under the skin, in the lining of the mouth, nose, and throat, or in other organs. Kaposi sarcoma is different from other cancers because lesions may begin in more than one place in the body at the same time. Kaposi sarcoma most commonly develops in people with human immunodeficiency virus (HIV) infection.
This information from the National Cancer Institute describes sarcoma in adults. For more information about this disease in children, visit the National Cancer Institute. Huntsman Cancer Institute's Sarcoma Services diagnoses and treats patients of all ages, including children. For information about making an appointment, call 801-585-5418.
Not all sarcomas have known risk factors, but some types do. Several factors influence the risk of developing a sarcoma.
Risk factors for osteosarcoma
- Previous radiation and chemotherapy treatment: Osteosarcoma occurs more often in people who have had external radiation therapy or treatment with certain chemotherapy drugs.
- Hereditary retinoblastoma: Children who have had hereditary retinoblastoma (a rare type of eye cancer) have a higher risk of developing osteosarcoma, particularly if they are treated with radiation.
- Bone defects: People who have hereditary defects of bones and people with metal implants, which doctors sometimes use to repair fractures, are more likely to develop osteosarcoma.
Risk factors for soft tissue sarcoma
- External radiation therapy: Soft tissue sarcomas are more common in people who previously received external radiation therapy for cancers of the retina, breast, cervix, ovary, testes, or lymphatic system. The risk appears to be related to the dose of radiation. To limit this risk, radiation treatment is planned to ensure that the greatest amount of radiation is delivered to diseased tissue while surrounding healthy tissue is protected as much as possible.
- Inherited diseases and genetic changes:
- Li-Fraumeni syndrome (associated with alterations in the p53 tumor suppressor gene)
- von Recklinghausen disease (also called neurofibromatosis type 1 and associated with alterations in the NF1 gene)
- Hereditary leiomyomatosis and renal cell cancer syndrome (with alterations in the FH gene)oHereditary retinoblastoma (with alterations in the RB1 gene)
- Exposure to certain chemicals: Another risk factor for soft tissue sarcomas is exposure to vinyl chloride, arsenic, herbicides such as phenoxyacetic acids, and wood preservatives that contain chlorophenols.
- Chronic lymphedema: This is a condition in which excess fluid collects in a tissue and causes swelling. This is common after lymph nodes have been removed or treated with radiation.
Common symptoms of sarcoma include the following:
- A lump or mass that develops anywhere on the body
- Pain in the abdomen (stomach area) that worsens
- Blood in the stool or vomit
- Black, tarry stools
These symptoms do not necessarily mean sarcoma. A person with any of these symptoms should see their health care provider so the problem can be diagnosed and treated as early as possible.
If a sarcoma is suspected, doctors will ask about the person's medical history and perform a physical exam. If a lump or mass has grown, doctors will need to take a sample (called a biopsy) to know if it is cancer. It is strongly recommended that the biopsy be removed and examined by a sarcoma specialist to make a diagnosis.
Biopsy options include the following:
- Needle biopsy: Doctors use a thin needle to remove small pieces of tissue from the tumor.
- Core biopsy: Doctors use a slightly larger needle to remove a solid core of tissue from the tumor.
- Image-guided biopsy: Doctors use ultrasound and computed tomography (CT) imaging to help the biopsy procedure.
- Surgical biopsy: Doctors make a cut in the skin to remove a portion of the tumor. This procedure may require a general anesthetic that puts the patient to sleep.
If the biopsy shows cancer is present, the doctor needs to know the stage (extent) of the disease to plan the best treatment.
Staging soft tissue sarcomas is based on the grade and size of the tumor, whether it is superficial (close to the skin's surface) or deep, and whether it has spread to the lymph nodes or other parts of the body.
Tests and procedures used in the staging process include the following:
- X-rays: An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body.
- Laboratory tests: These tests use samples of tissue, blood, urine, or other substances in the body to help diagnose disease, plan and check treatment, or monitor the disease over time.
- CT scan: Doctors often use CT scans to take pictures of tissue inside the body. An x-ray machine linked to a computer takes several pictures. The pictures may show a tumor, abnormal fluid, or swollen lymph nodes.
- Magnetic resonance imaging (MRI): This procedure uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body.
The results of these tests, together with the results of the biopsy, determine the stage of the sarcoma.
Stages of Sarcoma
Stage I: The tumor is any size and low-grade (likely to grow and spread slowly). It may be either superficial (close to the skin's surface) or deep in the body.
Stage II: The tumor is high-grade (likely to grow and spread quickly) and either:
- Five centimeters or smaller, either superficial or deep
- Larger than five centimeters and superficial
Stage III: The tumor is high-grade, larger than five centimeters, and deep in the body.
Stage IV: The tumor is any size, any grade, and has spread to nearby lymph nodes or to other parts of the body.
Ewing's sarcoma family of tumors, osteosarcoma, and malignant fibrous histiocytomaare described as either localized or metastatic.
- Localized: The cancer has not spread out of the bone where the disease started.
- Metastatic: The cancer has spread from the bone in which the disease began to other parts of the body.
Recurrent sarcoma: Cancer that comes back after it has been treated. The cancer may come back in the tissues where it first started or in another part of the body.
Sarcoma Services at Huntsman Cancer Institute (HCI) and Primary Children's Medical Center offers comprehensive, multidisciplinary treatment for sarcomas with surgical, medical, and pediatric oncologists; radiation therapists; pathologists; radiologists; pain and palliative care specialists; nurses; and social workers.
In addition to managing sarcomas, our team of experts evaluates and treats benign soft tissue and bone tumors. Diagnosis and treatment of these rare conditions are available for patients of all ages.
These are the common treatment options for sarcoma:
- Radiation therapy
- Clinical trials
This is the most common treatment for soft tissue or bone conditions and sarcomas—especially if cancer cells haven't spread to other parts of the body. Some types of sarcoma may also be treated with radiation therapy, chemotherapy, or both.
This treatment uses high-energy rays to kill cancer cells. Radiation therapy may be given alone, or with surgery, chemotherapy, or both. Doctors may use radiation to destroy cancer cells that remain in the area after surgery. They also use radiation to relieve pain and other problems caused by the cancer.
This treatment uses drugs that enter the bloodstream and destroy or control cancer throughout the body. Learn more about this treatment in our introduction to chemotherapy video.
This treatment uses liquid nitrogen to freeze and kill cancer cells. This surgery is sometimes used instead of conventional surgery to destroy bone tumors.
These studies discover and evaluate new and improved cancer treatments. Patients are encouraged to talk with their doctors about participating in a clinical trial or to ask any questions regarding research studies. For more information, also visit HCI's clinical trials website.
When you or someone you love is diagnosed with cancer, concerns about treatments and managing side effects, hospital stays, and medical bills are common. You may also worry about caring for your family, employment, or how to continue normal daily activities.
Here's where you can go for support:
- Your health care team can answer your questions and talk to you about your concerns. They can help you with any side effects and keep you informed of all your treatments, test results, and future doctor visits.
- Our Patient and Family Support Services professionals offer HCI patients and their families emotional support and resources for coping with cancer and its impact on your daily life.
- The Linda B. and Robert B. Wiggins Wellness-Survivorship Center offers support groups, classes, and activities aimed to increase the quality of life and well-being of HCI patients and their families.
Make an Appointment
Sarcoma Cancer Program
Care coordinator: Kimberly Andra
Did You Know?
- Soft tissue sarcomas are rare. In the United States, it is estimated they account for less than 1% of all new cancer cases in adults.
- Participation in clinical trials is an important treatment option for many people with sarcoma.
- Huntsman Cancer Institute's Sarcoma Services diagnoses and treats sarcoma cancers in patients of all ages.