Circa 2010

We at ABILITY Magazine have reported on various cancers from time to time, most of which have been fairly familiar to our readership. Sarcoma, however, is a less well-known type of cancer that is nevertheless worthy of serious discussion.

Put simply, sarcoma is cancer of the body’s connective tissues. As their name suggests, connective tissues’ primary function is to cover and hold other tissues together. They exist between organs and around various structures like muscles, tendons, blood vessels, and nerves.

Since most connective tissues are “soft” tissues, most sarcomas are commonly called “soft tissue sarcomas”. It is not impossible, however, for sarcomas to arise amidst hard tissues like those of bone or cartilage. Sarcomas are assigned special names depending on the nature of the tissue from which the cancer arises. For example, sarcomas that form from muscles in the extremities are called “rhabdomyosarcoma”, while those that form amidst blood vessels are called “angiosarcoma”.

Regardless of the location of the cancer, symptoms and treatments of most sarcomas are similar from case to case. The cells from which the cancers arise are also similar, no matter where in the body these cells happen to be located. This is the reason why tumors that occur in so many different locations of the body are all referred to as sarcomas. About 45% of all sarcomas occur in the extremities and another 35% occur in the internal organs.

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As is the case with most cancers, the exact cause of sarcoma is unknown. However, sarcomas have frequently been associated with exposure to radiation (including radiation used to treat certain cancers) and various chemicals. It should be noted, however, that the doses of radiation used in treating cancers are carefully modulated to avoid development of sarcoma in a patient. Nevertheless, in some rare cases, patients have developed sarcomas many years after being treated with radiation for another cancer.

Chemical culprits commonly associated with the development of sarcoma include vinyl chloride, arsenic, and certain herbicides, most of which have long been removed from most storebought products. Certain genetic alterations have also been identified for development of sarcomas, such as loss of the “tumor-suppressor” gene known as “p53”. Sarcomas are also associated with the genetic disease known as Neurofibromatosis Type I or Von Recklinghausen disease. Another sarcoma, known as Kaposi sarcoma, occurs primarily in AIDS patients whose immune systems are incapable of fighting off a particular type of herpes virus.

Sarcomas occurring in the extremities typically begin as non-painful lumps. It should be noted, however, that not every non-painful lump a person might develop is indicative of cancer. Moreover, since sarcomas can arise anywhere in the body, they may generate a myriad of symptoms.

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The diagnosis of sarcoma usually requires such common diagnostic procedures as x-rays, CT scans, and MRI scans. If a mass is detected, a biopsy—the surgical removal of a piece of the mass for study—is required to determine the exact nature of the growth.

Treatment of a sarcoma is dependent upon its stage and grade. A cancer’s “grade” refers to the nature of its cells as they appear under a microscope during biopsy, and its “stage” describes the extent to which the tumor has invaded adjacent tissues or has spread elsewhere in the patient. This process of a cancer’s spread throughout the body is commonly known as metastasis.

As is the case with many cancers, the treatment of sarcomas generally includes some combination of surgery, radiation therapy, and chemotherapy. Complete surgical removal of a tumor is confirmed by biopsies of the periphery of the area from which the tumor is removed.

Just as with many types of cancer, most sarcomas are not cured, but are controlled with treatments for periods of several years. Novel treatments of sarcomas are continuously under research, requiring clinical trials or studies that employ experimental therapies on humans with the disease.

Thomas Chappell, MD

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