Solitary fibrous tumor | |
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Classification and external resources | |
Micrograph of a solitary fibrous tumor. H&E stain. |
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ICD-O: | M8815/0 |
MeSH | D054364 |
Solitary fibrous tumor (SFT) is a rare mesenchymal tumor originating in the pleura[1] or at virtually any site in the soft tissue. Approximately 78% to 88% of SFT's are benign and 12% to 22% are malignant.[2]
SFT was first mentioned in the scientific literature by Wagner.[3] The first discussion of its clinical and pathological properties was by Klemperer and Rabin.[2][4]
Over the years this entity acquired a number of synonyms, including localized fibrous tumour, benign mesothelioma, localized fibrous mesothelioma, submesothelial fibroma, and pleural fibroma. The use of names that include ‘mesothelioma’ for this tumour is discouraged because of potential confusion with diffuse malignant mesothelioma, a much more serious disease.[1]
About 80% of SFT's originate in the visceral pleura, while 20% arise from parietal pleura.[5] Although they are often very large tumors (up to 40 cm. in diameter), over half are asymptomatic at diagnosis.[5] While some researchers have proposed that a SFT occupying at least 40% of the affected hemithorax be considered a "giant solitary fibrous tumor",[6] no such "giant" variant has yet been recognized within the most widely used pleural tumor classification scheme.[1]
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The treatment of choice for both benign and malignant SFT is complete en bloc surgical resection.
Prognosis in benign SFT's is excellent. About 8% will recur after first resection, with the recurrence usually cured after additional surgery.[2]
The prognosis in malignant SFT's is much more guarded. Approximately 63% of patients will have a recurrence of their tumor, of which more than half will succumb to disease progression within 2 years.[2] Adjuvant chemotherapy and/or radiotherapy in malignant SFT remains controversial.[2]
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