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Soft tissue sarcoma

Malignant (cancerous) tumors that develop in soft tissue are called sarcomas, a term that comes from a Greek word meaning "fleshy growth." more...

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In this context, the term soft tissue refers to tissues that connect, support, or surround other structures and organs of the body. Soft tissue includes muscles, tendons (bands of fiber that connect muscles to bones), fibrous tissues, fat, blood vessels, nerves, and synovial tissues (tissues around joints).

There are many different kinds of soft tissue sarcomas. They are grouped together because they share certain microscopic characteristics, produce similar symptoms, and are generally treated in similar ways. (Bone tumors, also known as osteosarcomas, are also called sarcomas, but are in a separate category because they have different clinical and microscopic characteristics and are treated differently.)

Sarcomas can invade surrounding tissue and can metastasize (spread) to other organs of the body, forming secondary tumors. The cells of secondary tumors are similar to those of the primary (original) cancer. Secondary tumors are referred to as "metastatic soft tissue sarcoma" because they are part of the same cancer and are not a new disease.

Some tumors of the soft tissue are benign (noncancerous). These tumors do not spread and are rarely life-threatening. However, benign tumors can crowd nearby organs and cause symptoms or interfere with normal body functions.

What are the possible causes of soft tissue sarcomas?

Scientists do not fully understand why some people develop sarcomas while the vast majority do not. However, by identifying common characteristics in groups with unusually high occurrence rates, researchers have been able to single out some factors that may play a role in causing soft tissue sarcomas.

Studies suggest that workers who are exposed to phenoxyacetic acid in herbicides and chlorophenols in wood preservatives may have an increased risk of developing soft tissue sarcomas. An unusual percentage of patients with a rare blood vessel tumor, angiosarcoma of the liver, have been exposed to vinyl chloride in their work. This substance is used in the manufacture of certain plastics.

In the early 1900s, when scientists were just discovering the potential uses of radiation to treat disease, little was known about safe dosage levels and precise methods of delivery. At that time, radiation was used to treat a variety of noncancerous medical problems, including enlargement of the tonsils, adenoids, and thymus gland. Later, researchers found that high doses of radiation caused soft tissue sarcomas in some patients. Because of this risk, radiation treatment for cancer is now planned to ensure that the maximum dosage of radiation is delivered to diseased tissue while surrounding healthy tissue is protected as much as possible.

Researchers believe that a retrovirus plays an indirect role in the development of Kaposi's sarcoma, a rare cancer of the cells that line blood vessels in the skin and mucus membranes. Kaposi's sarcoma often occurs in patients with AIDS (acquired immune deficiency syndrome). AIDS-related Kaposi's sarcoma, however, has different characteristics and is treated differently than typical soft tissue sarcomas.


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Synchronous carcinoma and soft-tissue sarcoma: the importance of searching for incidental radiation exposure
From CHEST, 3/1/93 by Alan D. Steinfeld

A 68-year-old woman was found to have synchronous soft-tissue sarcoma of the anterior chest wall and adenocarcinoma of the breast. During her initial interview, she denied prior radiation therapy. On further questioning, it was learned that the patient had been treated for tuberculosis, as a young woman, by the induction of a pneumothorax that was monitored by repeated chest fluoroscopies. Biologically important doses of ionizing radiation can be given as an incidental part of a variety of medical treatments. The importance of searching for atypical radiation exposures, particularly in patients with unusual tumor presentations, is stressed.

The relationship between exposure to ionizing radiation and the development of cancer is well known. Obtaining a history of such exposure may, on occasion, be difficult because of lack of patient awareness (as in treatment given during childhood), forgetfulness, or because the physician does not appreciate that potentially carcinogenic amounts of radiation are involved in certain, perhaps now obsolete, forms of treatment.


A 68-year-old, gravida 2, para 2, woman noted a lump on her anterior chest wall, just to the left of the sternum, at about the third intercostal space. Examination showed a poorly defined 2-cm subcutaneous mass. There were no skin changes or regional lymphadenopathy. Results of the remainder of the physical examination were unremarkable.

She was specifically asked about, and denied, exposure to radiation. She did say that she was hospitalized for about a year in 1947, in Germany, for tuberculosis. Further questioning revealed that the tuberculosis was managed with a pneumothorax, and she underwent repeated fluoroscopies to monitor the treatment.

Chest roentgenogram showed chronic obstructive pulmonary disease and scarring in the apex on the right. A computed tomographic (CT) scan of the chest showed the mass in the subcutaneous tissues just lateral to the sternum. Thickening of the left pleura was noted, as well as fibrosis and calcified granulomata in the right apex. Routine mammograms showed a 2-cm ill-defined mass in the upper outer quadrant of the left breast. Microcalcifications were noted within the mass.

A wide local excision of the chest wall mass was done. Histologic examination showed an intermediate to high-grade malignant spindle cell tumor compatible with sarcoma. Several weeks later, the breast mass was excised. A 2-cm infiltrating lobular carcinoma was found. The patient was sent for postoperative radiation therapy.


Before the advent of systemic treatments, pulmonary tuberculosis was often managed by resting the lung through induction of a pneumothorax. The progress of pneumo or collapse therapy, as it was known, was monitored by multiple fluoroscopic examinations and roentgenograms. The text by Alexander[1] is a comprehensive treatise on this historically interesting form of therapy. The Magic Mountain, written by Mann[2] in 1924, gives a dramatic description of chest fluoroscopy:

The breastbone and spine fell together in a single dark column. The frontal structure of the ribs was cut across by the paler structures of the back. Above, the collarbones branched off on both sides, and the framework of the shoulder, with the joint and the beginning of Joachim's arm, showed sharp and bare through the soft envelope of flesh. The thoracic cavity was light, but blood-vessels were to be seen, some dark spots, a blackish shadow.

Several studies have documented and quantified the increased risk of breast cancer in women treated in such a fashion. Boice and Monson[3] compared the incidence of breast cancer in 1,050 Massachusetts women treated for tuberculosis with pneumotherapy to 717 women treated by other methods. The mean follow-up time was 27 years. There were 41 cases of breast cancer in the irradiated group as compared with 23.3 expected, for a relative risk of 1.8. A typical patient was fluoroscoped 100 times with a cumulative breast dose of about 150 rad.[4] A latency of 10 to 15 years was noted, and the risk continued up to 40 years. Younger patients were more susceptible. No excess of lung cancer, lymphoma, or leukemia was noted. This study has recently been updated.[5] A linear relationship between breast cancer risk and dose was found up to 400 rad, with the increased risk present even at 50 years of follow-up.

In Nova Scotia, Myrden and Hiltz[6] compared the incidence of breast cancer in 300 women who had repeated fluoroscopies with 483 women who did not. There were 22 cancers in the former group (7.3 percent) as compared with four in the control group (<1 percent). The risk of developing a cancer appeared to be proportional to the number of fluoroscopic examinations the patient had. A study from Ontario has produced similar findings.[7]

While the fluoroscopy studies do not mention an increased risk of soft-tissue sarcomas, the ability of radiation to induce these tumors is well documented. Radium dial painters and patients given radium-containing solutions have shown an increase in osteosarcomas[8] as well as breast cancer.[9,10] A closer model may be patients who receive multiple external radiation treatments for other benign conditions. Excess cancers have been reported in patients treated for metropathia haemorrhagica,[11] though sarcomas are not specifically mentioned. An abnormally high number of sarcomas have been seen in children treated with radiation for malignant tumors,[12] though the possibility of a genetic predisposition to tumor formation in these children cannot be excluded. While there are many case reports of adults developing sarcomas following radiation therapy alone (without chemotherapy) for a variety of tumors, clear-cut epidemiologic data are lacking.

The case presented herein represents the simultaneous occurrence of two separate neoplasms in which ionizing radiation, from multiple fluoroscopic examinations, may be implicated as a causative factor. The physician must be aware of conditions for which ionizing radiation may have been used, either as part of the treatment itself or to evaluate the patient's condition during treatment. Such conditions include, among others, ankylosing spondylitis, tinea capitis, thymic enlargement,[13] spinal tuberculosis, tonsil or adenoid enlargement, acne, postpartum mastitis,[14,15] and infertility.


[1] Alexander J. The collapse therapy of pulmonary tuberculosis. Springfield, Ill: Charles C Thomas, 1937

[2] Mann T. The magic mountain. New York: Vintage Books Edition, 1969:217

[3] Boice JD, Monson R. Breast cancer in women after repeated fluoroscopic examinations of the chest. J Natl Cancer Inst 1977; 59:823-32

[4] Boice JD. Multiple chest fluoroscopies and the risk of breast cancer. Read before the 12th International Cancer Congress, Oct 5-11, Buenos Aires, Argentina, 1978

[5] Hrubec Z, Boice JD, Monson RR, Rosenstein M. Breast cancer after multiple chest fluoroscopies: second follow-up of Massachusetts women with tuberculosis. Cancer Res 1989; 49:229-34

[6] Myrden JA, Hiltz JE. Breast cancer following multiple fluoroscopies during artificial pneumothorax treatment of pulmonary tuberculosis. Can Med Assoc J 1969; 100:1032-34

[7] Cook DC, Dent O, Hewitt D. Breast cancer following multiple chest fluoroscopy: the Ontario experience. Can Med Assoc J 1974; 111:406-10

[8] Mays CW, Lloyd RD. Bone sarcoma incidence versus alpha particle dose. In: Stove BJ, Lee WSS, eds. Radiology of plutonium. Salt Lake City: JW Press, 1972

[9] Baverstock KF, Papworth D, Vennart J. Risks of radiation at low dose rates. Lancet 1981; 1:430-33

[10] Stebbings JH, Lucas H, Stehney A. Mortality from cancers of major sites in female radium dial workers. Am J Ind Med 1984; 5:435-59

[11] Smith PG, Doll R. Late effects of X irradiation in patients treated for metropathia haemorrhagica. Br J Radiol 1976; 49:224-32

[12] Meadows AT, D'Angio GJ, Mike V, Banfi A, Harris C, Jenkin RDT, et al. Patterns of second malignant neoplasms in children. Cancer 1977; 40:1903-11

[13] Hildreth N, Shore R, Hempelmann L. Risk of breast cancer among women receiving radiation treatment in infancy for thymic enlargement. Lancet 1983; 2:273

[14] Dvoretsky PM, Woodward E, Bonfiglio T, Hempelmann L, Morse I. The pathology of breast cancer in women irradiated for acute postpartum mastitis. Cancer 1980; 46:2257-62

[15] Mettler FA, Hempelmann L, Dutton A, Pifer J, Toyooka E, Ames W. Breast neoplasms in women treated with x-rays for acute postpartum mastitis: a pilot study. J Natl Cancer Inst 1969; 43:803-11

COPYRIGHT 1993 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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