Significant fibrosis after radiation therapy in a patient with Marfan syndrome.
10.3857/roj.2014.32.3.208
- Author:
Eva M SUAREZ
1
;
Rebecca J KNACKSTEDT
;
Joseph M JENRETTE
Author Information
1. Department of Radiation Oncology, Medical University of South Carolina, Charleston, SC, USA. suarezem@musc.edu
- Publication Type:Case Report
- Keywords:
Marfan syndrome;
Radiation therapy;
Transforming growth factor beta1;
Fibrosis
- MeSH:
Biomarkers;
Brachial Plexus;
Collagen;
Diagnosis;
Fibrosis*;
Humans;
Marfan Syndrome*;
Plasma;
Range of Motion, Articular;
Transforming Growth Factor beta;
Transforming Growth Factor beta1;
Vascular Diseases
- From:Radiation Oncology Journal
2014;32(3):208-212
- CountryRepublic of Korea
- Language:English
-
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.
