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KMID : 1038620140320030208
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Radiation Oncology Journal
2014 Volume.32 No. 3 p.208 ~ p.212
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Significant fibrosis after radiation therapy in a patient with Marfan syndrome
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Eva M. Suarez
Rebecca J. Knackstedt
BS
Joseph M. Jenrette
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Abstract
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Marfan syndrome is one of the collagen vascular diseases that theoretically predisposes patients to excessive radiation-induced fibrosis yet there is minimal published literature regarding this clinical scenario. We present a patient with a history of Marfan syndrome requiring radiation for a diagnosis of a right brachial plexus malignant nerve sheath tumor. It has been suggested that plasma transforming growth factor beta 1 (TGF-β1) can be monitored as a predictor of subsequent fibrosis in this population of high risk patients. We therefore monitored the patient's TGF-β1 level during and after treatment. Despite maintaining stable levels of plasma TGF-β1, our patient still developed extensive fibrosis resulting in impaired range of motion. Our case reports presents a review of the literature of patients with Marfan syndrome requiring radiation therapy and the limitations of serum markers on predicting long-term toxicity.
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KEYWORD
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Marfan syndrome, Radiation therapy, Transforming growth factor beta1, Fibrosis
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