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Thomas Bacon, John Berk, Minh Truong, Rachel Sobel; External Beam Radiation in the Treatment of Primary Diffuse Periocular Amyloidosis. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2430.
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© ARVO (1962-2015); The Authors (2016-present)
Primary diffuse periocular amyloidosis can represent unique treatment challenges, as it can be progressive and unamenable to surgical resection. Due to the tolerability of external beam radiation in primary periocular malignancies and its recent efficacy in treating primary airway amyloidosis these treatment strategies have been applied to periorbital amyloidosis. The aim of this study to further evaluate the efficacy of low dose external beam radiation therapy at halting the progression of diffuse primary periocular amyloidosis.
A retrospective chart review was conducted after approval of an Institutional Review Board. Collaboration between the Boston Medical Center Amyloid Center, Department of Ophthalmology, and Department of Radiation Oncology revealed four patients with histologic and biochemically proven primary localized orbital amyloidosis who underwent external beam radiation. Clinical records were evaluated for clinical presentation, radiation treatment, and response to therapy at least 1 year following treatment. Pre and post radiation MRI images were also evaluated for disease activity.
Initial presentation included periocular swelling (n=2) and ptosis (n=2). Additional treatments prior to radiation included systemic chemotherapy, surgical debulking, and ptosis repair. Cumulative radiation dose ranged from 2000-3000 cGy which was fractioned over 10-21 days. No patient experienced complications during radiation treatment. At one year follow up, one of four patients had clinical and radiographic recurrence of amyloid deposition. Furthermore, two of four patients experienced new or worsening dry eye symptoms that required artificial tear use and topical cyclosporine 0.05%. Of the three patients who were negative for recurrence at 1 year, two reached a 3 years post radiation time mark and both demonstrated clinical and radiographic recurrence of amyloidosis.
The case series presented describe encouraging short term results in terms of disease progression with majority of cases lacking clinical or radiographic progression at 1 year. However, two of the remaining three patients were positive for recurrence at 3 years post radiation. Challenges in identifying and defining recurrence include the subjective nature of clinical evaluation as well as the ability to discern millimeters of change in eyelid anatomy on magnetic resonance imaging.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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