Plaque Brachytherapy for DICER1-Associated Ciliary Body Medulloepithelioma.

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.