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Causes of soft tissue sarcomas

There are established predisposing factors: The condition neurofibromatosis (syn. Von Recklinhausen’s syndrome) predisposes to neurofibrosarcomas as does the multiple endocrine neoplasia syndrome – the former as malignant change in benign neurofibroma.
 
The syndrome: Gardner’s syndrome predisposes to desmoids (benign fibroid tumours) and occasional sarcomas arise.
 
Li Fraumeni syndrome is a strongly familial syndrome with an hereditary predisposition to multiple cancers of which sarcomas are but one type – the causative genetic defect is an aberration of the p-53 gene, that has now been recognised as a strongly influential tumour suppressor gene in normal physiological life.
 
In the hereditary type of retinoblastoma (a childhood malignant eye tumour) the loss or mutation of another tumour suppressor gene called the RB gene leads to predisposition to sarcomas.
 
There are other predisposing syndromes that are less well elucidated. Radiation exposure predisposes to a higher risk of developing sarcomas (and malignant fibrous histiocytoma is the commonest radiation induced sarcoma type) and to a lesser extent so too does mutagenic cytotoxic chemotherapy.
 




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