Refer to the Treatment Option Overview section of this summary for a more detailed discussion of the roles of surgery and radiation therapy.
Low-grade soft tissue sarcomas have little metastatic potential, but they have a propensity to recur locally. Accordingly, surgical excision with negative tissue margins of 1 cm to 2 cm or larger in all directions is the treatment of choice for patients with these early-stage sarcomas.[1,2,3] The Mohs surgical technique may be considered as an alternative to wide surgical excision for the very rare, small, well-differentiated primary sarcomas of the skin when cosmetic results are considered to be important, as margins can be assured with minimal normal tissue removal.
It is possible that the main title of the report Wilms' Tumor is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Carefully executed high-dose radiation therapy using a shrinking-field technique may be beneficial for unresectable tumors or for resectable tumors in which a high likelihood of residual disease is thought to be present when margins are judged to be inadequate, and when wider resection would require either an amputation or the removal of a vital organ. Because of the low metastatic potential of these tumors, chemotherapy is usually not given.[6,7]
Standard treatment options:
Surgical excision of tumors no greater than 5 cm in diameter with negative tissue margins in all directions.[8,9,10,11,12]
Surgical excision with preoperative radiation therapy (preRX) or postoperative radiation therapy (PORT). Radiation decreases the risk of local recurrence but has not been shown to increase overall survival.[13,14,15,16]
If the tumor is unresectable, high-dose preRX may be used.
For tumors of the retroperitoneum, trunk, and head and neck, the following are options:
Surgical resection with the option of PORT if negative margins cannot be obtained. Wide margins are unusual in these sites, and radiation therapy is usually advocated for trunk and head and neck primary sites.
PreRX followed by maximal surgical resection. Radiation therapy may be used in sarcomas of the trunk and head and neck to maximize local control because of the inability to obtain wide surgical margins.