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.)

High-grade localized soft tissue sarcomas have an increased potential for metastatic spread. For sarcomas of the extremities, local control comparable to that obtained with amputation may be achieved with limb-sparing surgery that involves wide local excision in combination with preoperative or postoperative radiation therapy and in some instances, chemotherapy.[1,2,3,4]

Several prospective randomized trials have been unable to confirm conclusively whether doxorubicin-based adjuvant chemotherapy benefits adults with resectable soft tissue sarcomas. The majority of these studies accrued small numbers of patients and did not demonstrate a metastasis-free survival or an overall survival (OS) benefit for adjuvant chemotherapy.[5] Interstudy variability was wide among the numerous trials, including differences in therapeutic regimens, drug doses, sample size, tumor site, and histologic grade. A quantitative meta-analysis of updated data from 1,568 individual patients from 14 trials of doxorubicin-based adjuvant therapy showed an absolute benefit from adjuvant therapy of 6% for local relapse-free interval (95% confidence interval [CI], 1–10), 10% for distant relapse-free interval (95% CI, 5–15), and 10% for recurrence-free survival (95% CI, 5–15); however, there was no OS benefit at 10 years.[6][Level of evidence: 1iiDi] Patients with high-grade tumors (grades 3 or 4) larger than 5 cm in diameter have the greatest tendency for disease to metastasize and are eligible for prospective clinical trials of adjuvant chemotherapy.

Complete surgical resection is often difficult for sarcomas of the retroperitoneum because of their large size before detection and anatomical location.[7,8] As opposed to soft tissue sarcomas of the extremities, local recurrence is the most common cause of death in patients with retroperitoneal soft tissue sarcomas. Complete surgical resection (i.e., removal of all gross tumor) is the most important factor in preventing local recurrence and, in many instances, requires resection of adjacent viscera. The role of adjuvant radiation therapy in the treatment of patients with retroperitoneal sarcoma has not been clearly defined. Prospective randomized trials have not shown improved survival with preoperative or adjuvant chemotherapy for this subgroup.[6,9]

STANDARD TREATMENT OPTIONS:

1. Surgical excision with negative tissue margins of several centimeters in all directions may be used.
2. If the tumor is greater than 5 cm in diameter, surgical excision with negative tissue margins of several centimeters in all directions followed by radiation therapy may be used.
3. If the tumor is unresectable, high-dose radiation therapy may be used, but poor local control is likely to result.
4. In some situations, radiation therapy or chemotherapy may be used prior to surgery to convert a marginally resectable tumor to one that can be adequately resected with limb preservation; this treatment may be followed by postoperative radiation therapy.

References:

1. Schmidt RA, Conrad EU 3rd, Collins C, et al.: Measurement and prediction of the short-term response of soft tissue sarcomas to chemotherapy. Cancer 72 (9): 2593-601, 1993.
2. Temple WJ, Temple CL, Arthur K, et al.: Prospective cohort study of neoadjuvant treatment in conservative surgery of soft tissue sarcomas. Ann Surg Oncol 4 (7): 586-90, 1997 Oct-Nov.
3. Watson DI, Coventry BJ, Langlois SL, et al.: Soft-tissue sarcoma of the extremity. Experience with limb-sparing surgery. Med J Aust 160 (7): 412-6, 1994.
4. Cormier JN, Huang X, Xing Y, et al.: Cohort analysis of patients with localized, high-risk, extremity soft tissue sarcoma treated at two cancer centers: chemotherapy-associated outcomes. J Clin Oncol 22 (22): 4567-74, 2004.
5. O'Byrne K, Steward WP: The role of adjuvant chemotherapy in the treatment of adult soft tissue sarcomas. Crit Rev Oncol Hematol 27 (3): 221-7, 1998.
6. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 350 (9092): 1647-54, 1997.
7. Heslin MJ, Lewis JJ, Nadler E, et al.: Prognostic factors associated with long-term survival for retroperitoneal sarcoma: implications for management. J Clin Oncol 15 (8): 2832-9, 1997.
8. Jaques DP, Coit DG, Hajdu SI, et al.: Management of primary and recurrent soft-tissue sarcoma of the retroperitoneum. Ann Surg 212 (1): 51-9, 1990.
9. Eilber FC, Eilber FR, Eckardt J, et al.: The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma. Ann Surg 240 (4): 686-95; discussion 695-7, 2004.