Local management of adult soft tissue sarcomas

Soft tissue sarcomas (STS) are rare tumors classified into multiple histological subtypes and categorized into four sites: extremity and trunk, head and neck, retroperitoneal, and visceral, the latter now predominantly consisting of gastrointestinal stromal tumors. Well-planned, complete surgical resection is the mainstay of curative therapy for tumors at each of these sites. The success of surgery alone in controlling disease varies with the site, histologic grade, depth, and size of the tumor. For high-risk tumors, adjuvant therapy should be considered. In high-risk extremity tumors, adjuvant radiation has been proven in randomized trials to improve local control. Limb-sparing surgery combined with adjuvant radiation achieves equivalent local control to amputation, with the same distant relapse-free survival. Due to anatomical constraints, tumors of the head and neck and retroperitoneum are typically excised with close margins, providing a rationale for adjuvant radiation; the available evidence suggests but does not prove a benefit. Large-scale trials of adjuvant imatinib for gastrointestinal stromal tumors are currently being conducted. For tumors of the extremity/trunk, head and neck, and retroperitoneum, biopsy prior to definitive resection is recommended to establish the diagnosis and permit intelligent treatment planning with appropriate choice and sequencing of adjuvant therapies. This planning is most expeditiously done through multidisciplinary consultation at an experienced sarcoma center.