Ciliary body medulloepithelioma is a rare, non-hereditary embryonic tumor that can be either benign or malignant and occurs most commonly in children. The tumor typically appears as an irregular grayish-white, yellowish-white, or pinkish mass, often accompanied by translucent cysts within the tumor or in the anterior chamber and vitreous. On ultrasound biomicroscopy (UBM), it is commonly observed as a medium-to-high echogenic mass in the ciliary body containing multiple cystic anechoic areas.
In its early stages, medulloepithelioma primarily presents with incomplete lens dislocation or localized lens opacity. As the tumor grows to fill the pupillary area, a "leukocoria" (white pupil reflex) may become apparent. Differential diagnoses include retinoblastoma, Coats disease, and persistent hyperplastic primary vitreous (PHPV). A small proportion of tumors may exhibit extrascleral invasion and distant metastasis.
Treatment options include cryotherapy, local resection, plaque brachytherapy, enucleation, and chemotherapy. Enucleation is the treatment of choice for advanced-stage ciliary body medulloepithelioma. In cases involving orbital extension, orbital exenteration is required.