Solitary fibrous tumor
Encyclopedia
Solitary fibrous tumor (SFT) is a rare mesenchymal tumor
originating in the pleura or at virtually any site in the soft tissue. Approximately 78% to 88% of SFT's are benign
and 12% to 22% are malignant
.
SFT was first mentioned in the scientific literature by Wagner. The first discussion of its clinical and pathological properties was by Klemperer and Rabin.
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.
About 80% of SFT's originate in the visceral pleura, while 20% arise from parietal pleura. Although they are often very large tumors (up to 40 cm. in diameter), over half are asymptomatic
at diagnosis. While some researchers have proposed that a SFT occupying at least 40% of the affected hemithorax be considered a "giant solitary fibrous tumor", no such "giant" variant has yet been recognized within the most widely used pleural tumor classification scheme.
.
Prognosis in benign SFT's is excellent. About 8% will recur after first resection, with the recurrence usually cured after additional surgery.
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. Adjuvant chemotherapy and/or radiotherapy in malignant SFT remains controversial.
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
originating in the pleura or at virtually any site in the soft tissue. Approximately 78% to 88% of SFT's are benign
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
and 12% to 22% are malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
.
SFT was first mentioned in the scientific literature by Wagner. The first discussion of its clinical and pathological properties was by Klemperer and Rabin.
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.
About 80% of SFT's originate in the visceral pleura, while 20% arise from parietal pleura. Although they are often very large tumors (up to 40 cm. in diameter), over half are asymptomatic
Asymptomatic
In medicine, a disease is considered asymptomatic if a patient is a carrier for a disease or infection but experiences no symptoms. A condition might be asymptomatic if it fails to show the noticeable symptoms with which it is usually associated. Asymptomatic infections are also called subclinical...
at diagnosis. While some researchers have proposed that a SFT occupying at least 40% of the affected hemithorax be considered a "giant solitary fibrous tumor", no such "giant" variant has yet been recognized within the most widely used pleural tumor classification scheme.
Treatment and prognosis
The treatment of choice for both benign and malignant SFT is complete en bloc surgical resectionSegmental resection
Segmental resection is a surgical procedure to remove part of an organ or gland. It may also be used to remove a tumor and normal tissue around it. In lung cancer surgery, segmental resection refers to removing a section of a lobe of the lung.- External links :* entry in the public domain NCI...
.
Prognosis in benign SFT's is excellent. About 8% will recur after first resection, with the recurrence usually cured after additional surgery.
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. Adjuvant chemotherapy and/or radiotherapy in malignant SFT remains controversial.
External links
- http://www.iarc.fr/en/publications/pdfs-online/pat-gen/index.php World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. (Download Page)