Solitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal neoplasm that originates from CD34+ dendritic stromal cells. While the majority of SFTPs are considered benign, an estimated 10%-20% are malignant. Malignant SFTP (MSFTP) is highly invasive and has propensities for local recurrence and distant metastasis. In recent years, an increasing number of MSFTPs have been reported. However, the diagnosis of MSFTP can be difficult owing to a lack of typical clinical manifestations and imaging features. To facilitate its diagnosis, the clinical, imaging, and histopathological characteristics of MSFTP are reviewed herein, followed by the clinical management such as surgery, radiotherapy, chemotherapy, targeted drugs and immunotherapy. Detection of NAB2-STAT6 fusion and expression of nuclear STAT6 can facilitate the diagnosis of SFTP. Furthermore, the recurrence risk stratification and prediction models of MSFTP as well as progress will be discussed. An improved understanding of these factors may contribute to more efficient diagnosis and management of MSFTP patients.
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