Soft tissue sarcoma
Soft tissue sarcoma is a broad term for cancers that start in soft tissues (muscle, tendons, fat, lymph and blood vessels, and nerves). These cancers can develop anywhere in the body but are found mostly in the arms, legs, chest, and abdomen. Soft tissue sarcoma is a form of cancer that occurs due to abnormal and uncontrolled cell growth of the "soft tissues" of the body. These tissues connect, support and surround other body parts and may include muscle, fat, blood vessels, lymph vessels, nerves, tendons and the lining of joints.
|Tissue of Origin||Type of Cancer||Usual Location in the Body|
|Fibrous tissue||Fibrosarcoma||Arms, legs, trunk|
|Fat||Liposarcoma||Arms, legs, trunk|
Uterus, digestive tract
|Blood vessels||Hemangiosarcoma||Arms, legs, trunk|
|Kaposi's sarcoma||Legs, trunk|
(linings of joint cavities, tendon sheaths)
|Peripheral nerves||Malignant peripheral nerve sheath tumour/Neurofibrosarcoma||Arms, legs, trunk|
|Cartilage and bone-forming tissue||Extraskeletal chondrosarcoma||Legs|
|Extraskeletal osteosarcoma||Legs, trunk (not involving the bone)|
|Tissue of Origin||Type of Cancer||Usual Location in the Body||Most common ages|
|Embryonal||Head and neck, genitourinary tract||Infant–4|
|Alveolar soft part sarcoma||Arms, legs, head, and neck||Infant–19|
|Fibrous tissue||Fibrosarcoma||Arms and legs||15–19|
|Fat||Liposarcoma||Arms and Legs||15–19|
|Blood vessels||Infantile hemangio-pericytoma||Arms, legs, trunk, head, and neck||Infant–4|
(linings of joint cavities, tendon sheaths)
|Synovial sarcoma||Legs, arms, and trunk||15–19|
|Peripheral nerves||Malignant peripheral nerve sheath tumors (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas)||Arms, legs, and trunk||15–19|
|Muscular nerves||Alveolar soft part sarcoma||Arms and legs||Infant–19|
|Cartilage and bone-forming tissue||Extraskeletal myxoid chondrosarcoma||Legs||10–14|
No one knows exactly what causes these cancers.
People who have previously received radiation therapy and those with certain inherited disorders (such as Gorlin syndrome, Gardner syndrome, Li-Fraumeni syndrome, Tuberous sclerosis, neurofibromatosis type 1, and Werner syndrome) have an increased risk of developing a soft tissue sarcoma.
Signs and symptoms
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 signs or symptoms until they get very big. As the sarcoma grows bigger and presses on nearby organs, nerves, muscles, or blood vessels, signs and symptoms may include:
- Trouble breathing.
The only reliable way to determine whether a soft-tissue tumour is benign or malignant is through a biopsy. The two methods for acquisition of tumour tissue for cytopathological analysis are:
- Needle aspiration biopsy, via needle
- Surgically, via an incision made into the tumour
A pathologist examines the tissue under a microscope. If cancer is present, the pathologist can usually determine the type of cancer and its grade. Here, 'grade' refers to a scale used to represent concisely the predicted growth rate of the tumour and its tendency to spread, and this is determined by the degree to which the cancer cells appear abnormal when examined under a microscope. Low-grade sarcomas, although cancerous, are defined as those that are less likely to metastasise. High-grade sarcomas are defined as those more likely to spread to other parts of the body. For soft-tissue sarcoma, the two histological grading systems are the National Cancer Institute system and the French Federation of Cancer Centers Sarcoma Group system.
The best treatment options depend on many factors but may include surgery, chemotherapy, and radiation therapy. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
- Olaratumab (Brand name: Lartruvo)In combination with doxorubicin, for the treatment of adult patients with soft tissue sarcoma (STS) with a histologic subtype for which an anthracycline-containing regimen is appropriate and which is not amenable to curative treatment with radiotherapy or surgery.
- Pazopanib (Brand name: Votrient)Advanced soft tissue sarcoma (STS) who have received prior chemotherapy.
Soft-tissue sarcomas are relatively uncommon cancers. They account for less than 1% of all new cancer cases each year. This may be because cells in soft tissue, in contrast to tissues that more commonly give rise to malignancies, are not continuously dividing cells.
In 2006, about 9,500 new cases were diagnosed in the United States.Soft-tissue sarcomas are more commonly found in older patients (>50 years old), although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma, synovial sarcoma).
Around 3,300 people were diagnosed with soft-tissue sarcoma in the UK in 2011.
NIH genetic and rare disease info
Soft tissue sarcoma is a rare disease.