Malignant fibrous histiocytoma (MFH) of soft tissue is one of the most common sarcoma in adulthood. However, only a few series have separately studied the clinical behavior and prognosis of this malignancy.

We retrospectively reviewed 61 patients treated for extremity soft tissue high-grade MFH. Four patients had a history of another malignancy and were excluded from analysis. In 12 referred patients with incomplete excision, re-excision of the tumor bed was offered. Clinical and treatment variables were analyzed for their impact on treatment complications, local recurrence (LR), metastatic disease (MD) and overall survival (OS).

Four patients underwent primary amputation. Twenty-three patients necessitated a primary reconstructive procedure for wound closure. Wound healing complication (WHC) developed in 28.3 % of the limb sparing group of patients. LR developed in 11 patients (19.3 %), while 6 of them had second LR. Eighteen patients (31.5 %) developed MD, involving lung at least. Patients who developed MD < 12  vs >12 months, died within 19.3 vs 8 months mean time (p <  0.05). Overall survivorship was 66.7 % at 5 years. No statistically significant variables were identified for LR, while multivariate analysis for MD revealed tumor size >5 cm as the only statistically significant variable. For OS, development of MD and age >70 years emerged as independent prognostic factors.

The overall prognosis is poor. LR, although can be managed with tumor re-excision, has high second recurrence rate. Increased tumor size is associated with shorter metastasis-free interval which significantly decreases survival.

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