Clinical Outcomes of Patients with Metastatic Solitary Fibrous Tumors: A Japanese Musculoskeletal Oncology Group (JMOG) Multiinstitutional Study.

BACKGROUND Although the unpredictable malignant behavior of solitary fibrous tumors (SFTs) has been recognized, the clinical features and prognosis of metastatic SFTs have not been well documented due to the extreme rarity of these cases. The aim of this study is to investigate the clinical features, prognostic factors, and optimal management of patients with metastatic SFTs. PATIENTS AND METHODS Sixty patients with metastatic SFT were retrospectively reviewed. Univariate and multivariate analyses were performed to identify the factors associated with survival. Time to next treatment (TNT) was used to evaluate the effects of various chemotherapy regimens. RESULTS A total of 34 male and 26 female patients (median age 55 years, range, 23-87 years) were included in the study. The median follow-up period after metastasis was 32 months (range 1-126 months). Tumor location and local recurrence were correlated with late metastasis. The 3- and 5-year overall survival rates were 72.7% and 49.2%, respectively. Primary tumor location, number of metastases, and metastasectomy were significantly associated with survival. Metastasectomy was the only significant variable on multivariate analysis. The TNT was significantly different among the various regimens. CONCLUSIONS Patients with metastatic SFTs had relatively longer survival periods compared with those with other metastatic soft-tissue sarcomas. Tumor location and number of metastases was associated with survival. Surgical resection of the metastatic lesions offers the best chance of survival, however further studies are warranted to define patients who would benefit from metastasectomy, and the most effective chemotherapeutic regimen for patients with metastatic SFTs remains unknown.