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Plaque Brachytherapy for DICER1-Associated Ciliary Body Medulloepithelioma
Mojtaba Abrishami1,2, Akbar Beiki-Ardakani3, Hatem Krema1
1Department of Ophthalmology and Vision Sciences, Ocular Oncology Service, Princess Margaret Cancer Center/University Health Network, University of Toronto, Toronto, Canada.
Purpose:
To report the outcome of a ciliary body medulloepithelioma associated with DICER-1 syndrome treated with iodine-125 plaque brachytherapy.
Methods:
An 11-year-old male with a DICER1 gene mutation, who was previously treated for pineoblastoma with craniospinal radiotherapy and systemic chemotherapy at the age of three, was referred to assess a vascularized retrolental mass in his left eye. The clinical and ultrasound evaluations, besides his initial diagnosis of DICER-1 syndrome, suggested a ciliary body medulloepithelioma.
Results:
No biopsy was performed given the risks of hemorrhage and tumor cell dissemination. The patient underwent iodine-125 plaque brachytherapy. Although the patient developed a radiation-induced cataract, it was eventually treated with cataract surgery. Five years after brachytherapy, the patient maintained excellent visual acuity with no signs of tumor recurrence.
Conclusion:
Plaque brachytherapy is effective in achieving long-term control of DICER1-associated medulloepithelioma.
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