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A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue 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 sarcomas can 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.
See the following summaries for more information on soft tissue sarcomas:
Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas that originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells, or fibroblasts. Because MPNSTs can arise from multiple cell types, the overall appearance can vary greatly from one case to the next. This can make diagnosis and classification somewhat difficult. In general, a sarcoma arising from a peripheral nerve or a neurofibroma is considered to be a MPNST. The term MPNST replaces a number of previously used names including malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma.A sarcoma is defined as a MPNST when at least one of the following criteria is met:
MPNSTs make up approximately 5-10% of all soft tissue sarcomas. They can occur either spontaneously or in association with neurofibromatosis-1 (NF1).The cause of MPNST is unknown but there is a higher incidence in patients with a history of radiation exposure. Also, up to 50% of MPNSTs occur in patients with NF1, demonstrating the tendency for this tumor to arise from a preexisting neurofibroma.MPNSTs generally occur in adulthood, typically between the ages of 20 and 50 years of age. Approximately 10-20% of cases have been reported to occur in the first 2 decade of life (Ref. 10), with occasional cases involving infants as young as 11 months of age.
MPNSTs usually present as an enlarging palpable mass. Pain is a variable complaint. Rapid enlargement occurs more often in the setting of NF1 and should raise concern for malignant degeneration of a neurofibroma. MPNSTs arising from peripheral nerves may result in a variety of clinical patterns, including radiating nerve pain, paresthesias, and motor weakness. Most MPNSTs occur in conjunction with large peripheral nerves such as the sciatic nerve, the brachial plexus and the sacral plexus.
Recurrence can be discussed in terms of local disease and distant or metastatic disease. The local recurrence rate for MPNSTs has historically been reported to range from 40-65% and the distant recurrence rate has similarly been reported to range from 40-68%. Five-year survival has been reported to range from 16-52%. Longer survival has been correlated with complete surgical excision, small tumor size (<5 cm), and the presence of a low grade component.One recent study showed a survival rate overall of 84% in patients treated at a sarcoma center. This has been largely attributed to improved imaging leading to early diagnosis and aggressive treatment, employing adjuvant and neoadjuvant treatment such as chemotherapy and radiation. In this study, patients with metastatic disease at presentation fared worse (33% survival) as would be expected.While patients with NF1 were previously thought to have a worse prognosis than did patients with sporadic MPNSTs, recent reports fail to support this.
Anything that increases your risk of getting a disease is called a risk factor. 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. Talk with your doctor if you think you may be at risk. Risk factors for soft tissue sarcoma include the following inherited disorders:
Other risk factors for soft tissue sarcoma include the following:
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:
Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus.
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:
Samples will be taken from the primary tumor, lymph nodes, and other suspicious areas. A pathologist views the tissue under a microscope to look for cancer cells and to find out the grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing.
High-grade tumors usually grow and spread more quickly than low-grade tumors.
Because soft tissue sarcoma can be hard to diagnose, patients should ask to have tissue samples checked by a pathologist who has experience in diagnosing soft tissue sarcoma.
The following tests may be done on the tissue that was removed:
The treatment options and prognosis (chance of recovery) depend on the following:
For more information on Soft Tissue Sarcoma click here
This link is to the National Cancer Institute (NCI) cancer website in the United States. There may be references to drugs and clinical trials that are not available here in Australia.
Information has also been sourced from hindawi.com, sarcoma.org.uk and sarcomahelp.org
Page last updated: 19/07/2018