Sarcomas of soft tissue and bone

Article Abstract:

Cancers are classed according to the embryonic cells they arise from. Sarcomas develop from tissues of the part of the embryo called the mesoderm. Such tissues include muscles, blood vessels, and the skeleton. However, some cancers traditionally termed sarcomas may ultimately be found to arise from cells other than mesodermal. About 75 percent of the body weight is composed of tissues that have arisen from the embryonic mesoderm. Nevertheless, only about 1 percent of all cancers are sarcomas. Sarcomas are proportionately more common among children; about 15 percent of all childhood cancers are sarcomas. Sarcomas are quite diverse, depending upon the tissue and location within the body where they arise. These factors also affect the optimum treatment and prognosis of sarcomas. For soft tissue sarcomas, that is sarcomas that have occurred in tissues other than bone, the most important predictor of outcome is the grade of the tumor. Low-grade sarcomas, in which the cancer cells resemble in appearance the normal adult cells of the same tissue, have the best prognosis. Conversely, the prognosis is the worst for high-grade sarcomas, in which the cancer cells appear highly abnormal and primitive in their features. If the sarcoma is in a suitable spot, surgery is the treatment of choice. Assuming the surgeon can remove the cancer with a wide margin of normal surrounding tissues, low grade sarcomas are likely to be cured. High-grade sarcomas are likely to recur and spread through the body. In some cases, usually involving limbs, extensive surgery, which may include amputation, may be avoided by combining conservative surgery with radiation therapy. Conversely, chemotherapy has not proved to be particularly useful in treating sarcomas in adults. However, several childhood sarcomas, rhabdomyosarcomas, osteosarcomas, and Ewing's sarcoma, can be responsive to chemotherapy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Prognosis, Sarcoma

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Adjuvant chemotherapy for osteosarcoma of the extremity: long-term results of two consecutive prospective protocol studies

Article Abstract:

Considerable controversy exists regarding the effectiveness of adjuvant chemotherapy in the treatment of bone cancer (osteosarcoma) of the arms and legs. Although initial results seemed promising, long-term follow-up in uncontrolled studies produced less convincing results. In order to more objectively evaluate the effectiveness of chemotherapy in these cases, 76 children and young adults with osteosarcoma were treated with chemotherapy after amputation of the affected extremity. All patients had a single tumor with no evidence of metastatic disease. After the appropriate amputation, the patients in the first study (OST 72) received adjuvant treatment with doxorubicin, cyclophosphamide, and methotrexate, while in the second study (OST 77) patients received methotrexate and doxorubicin only. In the first study, 13 of 26 patients developed metastases from 4 to 59 months after diagnosis. In the second group, 22 of 50 patients developed metastases. Comparison with historical controls who received no therapy after amputation revealed substantially improved disease-free survival. The protocol described here was also superior to a previously studied protocol using vincristine and cyclophosphamide, which was ineffective as adjuvant therapy in osteosarcoma. It is worth noting that 7 girls treated after puberty and one girl treated before, and 8 boys treated after puberty grew up to become mothers or fathers of a total of 21 children; with the exception of one child with a clubfoot deformity, all children were healthy and normal. (Consumer Summary produced by Reliance Medical Information, Inc.)

Chemotherapy

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Sarcomas (other than Ewing's) of flat bones in children and adolescents: a clinicopathologic study

Article Abstract:

Osteosarcomas arising in flat bones (other than Ewing's sarcoma), and other sarcomas (cancers) that are rare in children and young adults, such as malignant fibrous sarcoma, chondrosarcoma, and fibrosarcoma, have a poor prognosis. Although many studies have reported advances in chemotherapy, these studies have often excluded flat bone sarcomas and have focused on cancers of tubular bones of the arms and legs. Out of a total 228 bone sarcomas in children and adolescents that were recorded over a 25-year period, 30 patients had flat bone sarcomas. Twenty-eight of these histories were reviewed. Six tumors were second malignancies that arose after radiotherapy for an unrelated tumor. In 10 of the 28 flat bone sarcomas, complete surgical resection was possible, and five of these patients remained disease-free for approximately eight years after diagnosis. Of the 18 patients for whom complete surgical resection was not possible, only two achieved prolonged remission after chemotherapy. The average survival time of the 18 patients was one year. Although 16 patients showed progressive disease, only two developed metastatic disease. This review suggests that surgery is the optimal therapy, and when the tumor is not resectable, the chances for survival are small. Unlike osteosarcoma of tubular bones, metastases in early stages are uncommon in flat bone sarcomas, and metastatic spread is rare even in cases of treatment failure. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research

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