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Medulloepithelioma: A triad of clinical features
Ani Peshtani1, Swathi Kaliki1, Ralph C Eagle2
1Department of Ocular Oncology, Wills Eye Institute, Thomas Jefferson University, 840 Walnut Street, 14 Floor, Philadelphia PA, 19107.
Background:
Intraocular medulloepithelioma arises from the primitive medullary epithelium and is diagnosed at a median age of five years. This tumor most commonly appears as a white, gray, or yellow-colored ciliary body tumor. The growth of medulloepithelioma is slow and it is locally invasive. Poor vision and pain are the most common presenting symptoms. The most common clinical signs include cyst or mass in iris, anterior chamber or ciliary body, glaucoma, and cataract.
Case:
A 22-month-old Caucasian female twin presented with leukocoria and poor vision in OS. Examination revealed normal findings OD and a mass in OS. Based on the clinical features of leukocoria, lens changes and a white cystic ciliary body mass in a young child, ultrasonographic, and transillumination features, the lesion was diagnosed as a non-pigmented ciliary epithelial medulloepithelioma. After enucleation, the diagnosis of malignant teratoid medulloepithelioma of the non-pigmented ciliary epithelium was confirmed. There was no evidence of tumor recurrence or systemic metastasis at three years follow-up.
Conclusion:
Medulloepithelioma in a child can present as a clinical triad of leukocoria, lens changes, and a white cystic ciliary body mass.
Insights
Intraocular medulloepithelioma, a rare ciliary body tumor, can present in children with leukocoria, lens changes, and a white cystic mass. Early diagnosis and treatment are crucial for managing this locally invasive tumor.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Pathology
Background:
- Intraocular medulloepithelioma originates from primitive medullary epithelium, typically diagnosed around age five.
- It presents as a slow-growing, locally invasive ciliary body tumor, often white, gray, or yellow.
- Common symptoms include poor vision and pain, with clinical signs like iris/ciliary body masses, glaucoma, and cataracts.
Observation:
- A 22-month-old female presented with leukocoria and decreased vision in her left eye.
- Clinical examination revealed a ciliary body mass in the left eye.
- Diagnostic features included leukocoria, lens changes, a white cystic ciliary body mass, and characteristic ultrasonographic/transillumination findings.
Findings:
- The lesion was diagnosed as a non-pigmented ciliary epithelial medulloepithelioma.
- Post-enucleation, the diagnosis of malignant teratoid medulloepithelioma of the non-pigmented ciliary epithelium was confirmed.
- No tumor recurrence or systemic metastasis was observed at three-year follow-up.
Implications:
- Medulloepithelioma in children can manifest as a distinct triad: leukocoria, lens changes, and a white cystic ciliary body mass.
- This case highlights the importance of recognizing this presentation for timely diagnosis and management.
- Prompt intervention is essential for improving outcomes in pediatric intraocular tumors.
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