Radiat Oncol J.
2014 Sep;32(3):208-212. 10.3857/roj.2014.32.3.208.
Significant fibrosis after radiation therapy in a patient with Marfan syndrome
- Affiliations
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- 1Department of Radiation Oncology, Medical University of South Carolina, Charleston, SC, USA. suarezem@musc.edu
- KMID: 2316736
- DOI: http://doi.org/10.3857/roj.2014.32.3.208
Abstract
- 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-beta1) can be monitored as a predictor of subsequent fibrosis in this population of high risk patients. We therefore monitored the patient's TGF-beta1 level during and after treatment. Despite maintaining stable levels of plasma TGF-beta1, 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.
