Adult soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body. The soft tissues of the body include the muscles, tendons (bands of fiber that connect muscles to bones), fat, blood vessels, lymph vessels, nerves, and tissues around joints. Adult soft tissue sarcoma scan form almost anywhere in the body, but are most common in the head, neck, arms, legs, trunk, and abdomen.
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.
Risk Factors
Having certain inherited disorders can increase the risk of developing adult soft tissue sarcoma. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Risk factors for soft tissue sarcoma include the following inherited disorders:
- Retinoblastoma.
- Neurofibromatosis type 1 (NF1; von Recklinghausen disease).
- Tuberous sclerosis (Bourneville disease).
- Familial adenomatous polyposis (FAP; Gardner syndrome).
- Li-Fraumeni syndrome .
- Werner syndrome (adult progeria).
- Nevoid basal cell carcinoma syndrome (Gorlin syndrome).
Other risk factors for soft tissue sarcoma include the following:
- Past treatment with radiation therapy for certain cancers.
- Being exposed to certain chemicals, such as Thorotrast (thorium dioxide), vinyl chloride, or arsenic.
- Having swelling (lymphedema) in the arms or legs for a long time.
Symptoms
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:
Other conditions may cause the same symptoms that soft tissue sarcoma does. Check with your doctor if you have any of these problems.
Diagnosis
Adult soft tissue sarcoma is diagnosed with a biopsy.
If your doctor thinks you may have a soft tissue sarcoma, a biopsy will be done. The type of biopsy will be based on the size of the tumor and where it is in the body. There are three types of biopsy that may be used:
- Incisional biopsy: The removal of part of a lump or a sample of tissue.
- Core biopsy: The removal of tissue using a wide needle.
- Excisional biopsy: The removal of an entire lump or area of tissue that doesn’t look normal.
Treatment
There are different types of treatment for patients with adult soft tissue sarcoma.
Surgery
Surgery is the most common treatment for adult soft tissue sarcoma. For some soft-tissue sarcomas, removal of the tumor in surgery may be the only treatment needed. The following surgical procedures may be used:
- Mohs microsurgery: A procedure in which the tumor is cut from the skin in thin layers. During surgery, the edges of the tumor and each layer of tumor removed are viewed through a microscope to check for cancer cells. Layers continue to be removed until no more cancer cells are seen. This type of surgery removes as little normal tissue as possible and is often used where appearance is important, such as on the skin.
- Wide local excision: Removal of the tumor along with some normal tissue around it. For tumors of the head, neck, abdomen, and trunk, as little normal tissue as possible is removed.
- Limb-sparing surgery: Removal of the tumor in an arm or leg without amputation, so the use and appearance of the limb is saved. Radiation therapy or chemotherapy may be given first to shrink the tumor. The tumor is then removed in a wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone.
- Amputation: Surgery to remove part or all of a limb or appendage, such as an arm or leg. Amputation is rarely used to treat soft tissue sarcoma of the arm or leg.
- Lymphadenectomy: Removal of the lymph nodes that contain cancer.
Radiation/Chemotherapy
Radiation therapy or chemotherapy may be given before or after surgery to remove the tumor. When given before surgery, radiation therapy or chemotherapy will make the tumor smaller and reduce the amount of tissue that needs to be removed during surgery. Treatment given before surgery is called neoadjuvant therapy. When given after surgery, radiation therapy or chemotherapy will kill any remaining cancer cells. Treatment, given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.