Between March 1983 and December 1994, 65 patients with malignant fibrous histiocytoma of bone in the extremities were treated with neoadjuvant chemotherapy administered according to four different regimens. A limb salvage was done in 58 patients (89%) and amputation in seven. The histologic response to preoperative chemotherapy was good (90% or more tumor necrosis) in 16 patients (25%) and poor in 49 (75%). At a median followup of 7 years (range, 2-13 years), 40 patients (69%) remained continuously free of disease and 20 patients experienced relapse (18 with metastases and two with local recurrences followed by metastases). The rate of disease free survival was significantly higher for patients who had a good response than for those who had a poor response (94% versus 61%), although no significant differences in histologic response and disease free survival were seen with the four different regimens. These results show that a high percentage of patients with malignant fibrous histiocytoma of the extremities can be cured with neoadjuvant chemotherapy and that for most of them it is possible to avoid amputation.