![Illustration of adult leiomyosarcoma soft tissue sarcoma](https://images.medicinenet.com/images/article/main_image/leiomyosarcoma-soft-tissue-sarcoma.jpg?output-quality=75)
Adult soft tissue sarcoma is an uncommon type of cancer that occurs when the cells in the soft tissues of the body grow out of control.
- Soft tissue sarcomas can develop in soft tissues such as fat, muscle, nerves, fibrous tissues such as tendons, blood vessels, lymph vessels, or deep skin tissues. They can occur in any part of the body.
- Sarcomas are not common, and some are very rare and others can be very deadly.
- Adult soft tissue sarcomas commonly develop in the head, neck, arms, legs, trunk, abdomen, and retroperitoneum, though they can occur anywhere in the body.
- There are many types of sarcomas, and not all of them are cancerous, however, when the word sarcoma is part of the name of a disease, it means the tumor is cancerous (malignant).
Adult soft tissue sarcoma is more able to be cured with surgical treatment when it is found early and is still localized.
- About 60% of sarcomas are found as a localized sarcoma
- The five-year survival rate for localized sarcoma is 81%
- About 19% of sarcomas are found in a locally advanced stage
- The five-year survival rate for locally advanced sarcoma is 56%
- About 15% of sarcomas are found in a metastatic stage
- The five-year survival rate for metastatic sarcoma is 15%
Treatments for adult soft tissue sarcoma can include one or more of the following:
- Surgery
- This most common treatment
- The goal of surgery is to remove the entire tumor along with at least 1 to 2 cm (less than an inch) of the normal tissue around it (wide margins)
- Wide local excision
- Amputation
- Limb-sparing surgery
- Lymph node dissection (lymphadenectomy)
- Mohs microsurgery, in which the tumor is cut from the skin in thin layers
- Chemotherapy (chemo), radiation, or both may be given before surgery (neoadjuvant treatment) or after surgery (adjuvant treatment)
- Radiation therapy
- External beam radiation
- Most often used to treat sarcomas
- Intensity modulated radiation therapy (IMRT) is used
- Intraoperative radiation therapy (IORT)
- One large dose of radiation given in the operating room after the tumor is removed but before the wound is closed
- Brachytherapy (internal radiation therapy
- Places small pellets (or seeds) of radioactive material in or near the cancer
- Proton beam radiation (not widely available)
- External beam radiation
- Chemotherapy
- Dactinomycin (Cosmegen)
- Doxorubicin (Adriamycin)
- Eribulin (Halaven)
- Imatinib mesylate (Gleevec)
- Pazopanib mydrochloride
- Tazemetostat mydrobromide
- Trabectedin (Yondelis)
- Targeted drug therapy
- Drug combinations
- VAC: vincristine sulfate, dactinomycin, cyclophosphamide
What Are Symptoms of Adult Soft Tissue Sarcoma?
Symptoms of adult soft tissue sarcoma include:
- A lump that may or may not cause pain
- About half of soft tissue sarcomas start in an arm or leg
- About 4 in 10 sarcomas start in the abdomen
- Rarely, sarcomas start in the chest, head, or neck
- Lump grows over time (weeks to months)
- If a sarcoma occurs in the back of the abdomen (the retroperitoneum), symptoms may include blockage or bleeding of the stomach or bowels
- As the sarcoma grows it can press on nearby organs, nerves, muscles, or blood vessels, and cause:
- Pain
- Trouble breathing
What Causes Adult Soft Tissue Sarcoma?
Adult soft tissue sarcoma is believed to be caused by genetic changes (mutations), however, the exact cause for these mutations is unknown.
Risk factors for developing adult soft tissue sarcoma include:
- Radiation therapy used to treat other cancers
- Family cancer syndromes
- Neurofibromatosis type 1 (NF1; von Recklinghausen disease)
- Familial adenomatous polyposis (FAP; Gardner syndrome).
- Li-Fraumeni syndrome
- Retinoblastoma
- Werner syndrome (adult progeria)
- Nevoid basal cell carcinoma syndrome (Gorlin syndrome)
- Tuberous sclerosis (Bourneville disease)
- Damaged lymph system/lymphedema
- Chemical exposure
- Vinyl chloride (a chemical used in making plastics)
- Arsenic
- Dioxin
- Thorium dioxide (Thorotrast)
- Herbicides (weed killers) containing phenoxyacetic acid at high doses (such as might occur in people who work on farms, though there is no evidence that herbicides or insecticides cause sarcomas in levels encountered by the general public)
How Is Adult Soft Tissue Sarcoma Diagnosed?
In addition to a patient history and physical exam, tests used to diagnose adult soft tissue sarcoma include:
- Biopsy
- A tissue sample will be taken and tests performed on the tissue may include:
- Immunohistochemistry
- Light and electron microscopy
- Cytogenetic analysis
- FISH (fluorescence in situ hybridization)
- Flow cytometry
- A tissue sample will be taken and tests performed on the tissue may include:
- Imaging tests
- X-ray
- Computed tomography (CT) scan
- Magnetic resonance imaging (MRI) scan
- Ultrasound
- Positron emission tomography (PET) scan
- Blood tests
- Complete blood count (CBC)
- Blood chemistry
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https://www.cancer.org/cancer/soft-tissue-sarcoma.html
https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq
https://www.cancer.net/cancer-types/sarcomas-soft-tissue/statistics