Soft tissue sarcoma is a type of cancer that begins in the soft tissues of your body.
Soft tissues connect, support and surround other body structures. The soft tissues include muscle, fat, blood vessels, nerves, tendons and the lining of your joints.
Many types of soft tissue sarcoma exist. Some types are more likely to affect children, while others affect mostly adults.
Soft tissue sarcoma can occur anywhere in your body, but the most common types occur in the abdomen and in the arms and legs.
We don’t yet know what causes sarcoma, but we do know some things that raise the risk of developing one:
- Other people in your family have had sarcoma
- You have a bone disorder called Paget’s disease
- You have a genetic disorder such as neurofibromatosis, Gardner syndrome, retinoblastoma, or Li-Fraumeni syndrome
- You’re been exposed to radiation, perhaps during treatment for an earlier cancer
PRS can occur with orthovoltage (low-energy) and megavoltage (high-energy) radiation. With orthovoltage radiation, the dosages are lower and the latent periods are longer. The threshold dose for PRS is not known, although in most published series, a dosage of 40-60 Gy has been reported. Development of PRS also is influenced by other factors, including genetic tendency and influence of chemotherapeutic agents.
Ionizing radiation is thought to act via genetic alterations, including mutations of p53 and retinoblastoma (Rb) genes. Experimental studies revealed p53 gene alterations or increased p53 messenger ribonucleic acid (mRNA) levels in murine PRS.
A study by Mentzel et al used fluorescence in situ hybridization (FISH) to analyzeangiosarcomas and atypical vascular lesions occurring after treatment of breast cancer. In all postradiation cutaneous angiosarcomas, FISH analysis revealed MYC amplification in a variable number of counted nuclei; MYC amplification was not seen in any of the other cases. The authors concluded that MYC amplification may be an important diagnostic tool for distinguishing postradiation cutaneous angiosarcomas from atypical vascular lesions after radiotherapy.
A soft tissue sarcoma may not cause any signs and symptoms in its early stages. As the tumor grows, it may cause:
- A noticeable lump or swelling
- Pain, if a tumor presses on nerves or muscles
If your doctor thinks you may have a sarcoma, you’ll probably need a full exam and tests, including:
- A sample of cells from the tumor, called a biopsy
- Imaging tests, such as a CT scan, an ultrasound, or an MRI, to help see inside your body
- A bone scan, if you might have osteosarcoma
Your treatment options for soft tissue sarcoma will depend on the size, type and location of your tumor.
Surgery is a common treatment for soft tissue sarcoma. Surgery generally involves removing the cancer and some healthy tissue surrounding it.
When soft tissue sarcoma affects the arms and legs, it may be necessary to amputate the affected arm or leg. Doctors try their best to avoid amputation. One option to achieve this may be using chemotherapy and radiation to shrink the tumor before surgery, which increases the likelihood that an amputation will be unnecessary.
When soft tissue sarcoma spreads to another area of the body, it may be possible to remove the other tumor with surgery, as well.
Radiation therapy involves treating cancer with high-powered beams of energy, such as X-rays or protons.
Your doctor may suggest using radiation therapy before surgery to shrink a tumor to make it easier to remove with surgery. Radiation is also used after surgery to kill any cancer cells that remain.
Chemotherapy and radiation may be combined.
Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. Chemotherapy can be administered by pill, through a vein (intravenously), or both methods may be used.
Some forms of soft tissue sarcoma respond better to chemotherapy than do others. For instance, chemotherapy is often used to treat rhabdomyosarcoma.
TARGETED DRUG TREATMENT
Targeted drugs block specific abnormal signals present in sarcoma cells that allow them to grow.
Targeted drugs used to treat one type of sarcoma called gastrointestinal stromal tumor include:
- Imatinib (Gleevec)
- Sunitinib (Sutent)
- Regorafenib (Stigvara)
- Pazopanib (Votrient)
Many new targeted drugs are being studied.