Radiation Oncology Journal

The Korean Society for Radiation Oncology (대한방사선종양학회)
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Significant fibrosis after radiation therapy in a patient with Marfan syndrome

  • Suarez, Eva M. (Department of Radiation Oncology, Medical University of South Carolina) ;
  • Knackstedt, Rebecca J. (Department of Radiation Oncology, Medical University of South Carolina) ;
  • Jenrette, Joseph M. (Department of Radiation Oncology, Medical University of South Carolina)
  • Received : 2014.03.11
  • Accepted : 2014.08.29
  • Published : 2014.09.30
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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-${\beta}1$) can be monitored as a predictor of subsequent fibrosis in this population of high risk patients. We therefore monitored the patient's TGF-${\beta}1$ level during and after treatment. Despite maintaining stable levels of plasma TGF-${\beta}1$, 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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References

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