Sarcomas are a relatively rare and heterogeneous group of malignant tumors of principally mesenchymal origin. The histologic grade and size (and possibly compartmental localization) are the main factors predicting local and distant biologic aggressiveness. Tumor localization and surgical margins are significant prognostic factors that relate to the adequacy of local-regional therapy. A general consensus management usually consists of an incisional biopsy for diagnosis and grade, staging of the primary tumor and lungs, and function-preserving surgery with margins free of tumor either followed by or preceded by tumor bed high-dose radiotherapy. Each of these concepts remains under active investigation. The role of adjuvant therapy is not yet established despite tantalizing biologic effects documented in their trials. Ifosfamide in addition to doxorubicin does appear to have major activity; however, further laboratory investigation of resistance and metastases mechanisms and new drug evaluations are necessary for further advance.