Background: The purpose of this study was to report the outcomes of an extremely rare and misdiagnosed embryonal
neuroepithelial intraocular tumor.

Material and methods: The medical records of 28 nucleated eyes with unilateral vision loss in childhood and young adults were reviewed retrospectively. Demographic information, clinical history, visual acuity, indication for surgery, and histopathological findings were all noted. Cases of visual loss related to ocular trauma or endophthalmitis were not included in this study. B-scan ultrasonography was performed on patients due to media opacity.

Results: A total of 7 eyes of 28 patients, age between 1–18 years, who were underwent enucleation surgery for intraocular malignancy were included in the study. The mean follow-up period was 29.4 ± 10.2 months (range, 15–60 months ). The mean age of the patients was 5.85 ± 7.33 years. There were four females and three males with equal eye distribution. The patients had no light perception. Five of 7 patients were operated on for retinoblastoma in the early period of life with easily recognized clinical signs. The other two patients wanted surgery just because they were uncomfortable with the appearance of their eyes. One of them was a 15-year-old girl with a history of blind eye and glaucoma. She had been treated for congenital glaucoma since the age of 5. At the time of the research she was complaining about the painful, blind eye as an aesthetic defect. Histopathological diagnosis was reported as medulloepithelioma. As a long-term complication, submandibular lymph node metastasis was detected during
the follow-up period. Otolaryngologists performed the radical neck dissection. The patient received radiotherapy
and chemotherapy. She is now in her 30s. No other complication was observed during the follow-up period. The
other patient was an 18-years-old boy, who had lost his vision for an unknown cause in childhood, and wanted to
have surgery for aesthetic purposes due to opaque cornea. Ultrasonography detected a mass in the ciliary body. The
enucleation surgery was performed, and histopathological diagnosis was reported as medulloepithelioma. The patient
received radiotherapy, and no metastasis developed during the follow-up period.

Conclusion: Ophthalmologists need to be more familiar with early diagnoses and screening of the eye, especially
detecting tumor cases “medulloepithelioma” which is often misdiagnosed and treated as glaucoma.