Abstract

Solitary fibrous tumors (SFTs) are a unique group of mesenchymal neoplasms of fibroblastic or myofibroblastic origin. These tumors were originally described as "benign fibrous mesotheliomas" of the pleural cavity and were erroneously thought to be confined to the serosal surfaces (due to a putative mesothelial or submesothelial origin). It is now established that SFTs are ubiquitous neoplasms with both pleural and extrapleural distribution. Extrapleural SFTs commonly occur in middle-aged adults and manifest as asymptomatic, slow-growing, large tumors. Fewer than 5% of patients with SFTs present with symptomatic hypoglycemia. SFTs are histopathologically diverse with a variable admixture of fibroblasts or myofibroblasts, numerous thin-walled vessels, and dense fibrosis. Tumors previously categorized as hemangiopericytomas are now considered cellular variants of SFTs. At imaging, SFTs demonstrate remarkable heterogeneity, with variable degrees of enhancement, necrosis, or hemorrhage. Although most extrapleural SFTs have a benign clinical course, 10%-15% of these tumors demonstrate aggressive behavior in the form of recurrence or malignancy.

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