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General Information
Note: Separate PDQ summaries on Childhood Soft Tissue Sarcoma Treatment and Uterine Sarcoma Treatment are also available.
Note: Estimated new cases and deaths from soft tissue sarcoma in the United States in 2008:[1]
- New cases: 10,390.
- Deaths: 3,680.
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Soft tissue sarcomas are malignant tumors that may arise in any of the mesodermal tissues of the extremities (50%), trunk and retroperitoneum (40%), or head and neck (10%). Rarely, these tumors arise in the gastrointestinal tract or gastrointestinal stroma, and a small percentage of these are called gastrointestinal stromal tumors (GISTs). Malignant GISTs can occur from the esophagus to the rectum but occur most commonly in the stomach and small intestine. Soft tissue sarcomas occur with greater frequency in patients with:[2]
- von Recklinghausen disease (neurofibromatosis).
- Gardner syndrome.
- Werner syndrome.
- Tuberous sclerosis.
- Basal cell nevus syndrome.
- Li-Fraumeni syndrome (p53 mutations).
Soft tissue sarcomas may be heterogeneous, so adequate tissue should be obtained via either core-needle or incisional biopsy for microscopic examination to determine histologic type and tumor grade. Careful planning of the initial biopsy is important to avoid compromising subsequent curative resection. Since the selection of treatment is determined by the grade of the tumor, it is essential to have a careful review of the biopsy tissue by a pathologist who is experienced in diagnosing sarcomas. Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with this disease. In most cases, a combined modality approach of preoperative or postoperative radiation therapy is used, rather than the radical surgical procedures that were used in the past. The role of chemotherapy is less well defined. Because of the evolving nature of the state of the art in the treatment of this disease, all patients with such lesions should be included in a clinical trial whenever possible.
The prognosis for patients with adult soft tissue sarcomas depends on several factors, including the patient’s age and the size, histologic grade, and stage of the tumor.[3,4,5] Factors associated with a poorer prognosis include age older than 60 years, tumors larger than 5 cm, or high-grade histology.[6]
While low-grade tumors are usually curable by surgery alone, higher-grade sarcomas (as determined by the mitotic index and by the presence of hemorrhage and necrosis) are associated with higher local treatment failure rates and increased metastatic potential.[7] When feasible, wide margin function-sparing surgical excision is the cornerstone of effective treatment, with the goal of preservation of a functional extremity.[8,9] This may be facilitated by soft tissue reconstructive surgery.[10] Mohs surgical technique may be considered as an alternative to wide surgical excision for small, well-differentiated sarcomas when cosmetic results are considered to be very important, as margins can be assured with minimal normal tissue removal.[2,11] High-grade soft tissue sarcomas of the extremities can often be effectively treated while preserving the limb with combined-modality treatment consisting of preoperative or postoperative radiation therapy to reduce local recurrence. A phase II trial (SWOG-9119) was conducted of neoadjuvant therapy with DOX/DTIC/IFF for poor prognosis soft tissue sarcoma.[8,12,13,14,15,16,17,18,19] In adults, local control of high-grade soft tissue sarcomas of the trunk and the head and neck can be achieved with surgery, often in combination with radiation therapy with or without chemotherapy.[20]
WebMD Public Information from the National Cancer Institute
This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER
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