What Is Medulloepithelioma?
Medulloepithelioma of the eye is a rare tumour that develops from the non-pigmented ciliary epithelium, the tissue responsible for producing the fluid inside the eye. It most often occurs in young children, typically under the age of 10.
This tumour closely resembles embryonic retinal tissue, and depending on its cellular makeup, it may be benign (non-cancerous) or malignant (cancerous). It is sometimes referred to as “diktyoma,” particularly when describing its cystic or mixed solid-cystic appearance.
Though primarily arising from the ciliary body, in very rare cases it may originate from the optic nerve or retina.
Causes
The exact cause of medulloepithelioma of the eye is not well understood. It is believed to result from abnormal development of the primitive medullary epithelium during early eye formation. Unlike many other tumours, it is not associated with inherited cancer syndromes and does not typically run in families.
Signs and Symptoms
Symptoms depend on the size and growth of the tumour. Children may not always be able to describe their symptoms clearly, so careful observation is essential. Common signs include:
- Decreased or blurry vision
- Eye pain or discomfort
- A visible white, gray, or yellow mass inside the eye
- Occasionally, glaucoma, uveitis, or lens displacement may be present due to tumour growth or associated inflammation
Parents or caregivers may first notice a white reflex in the pupil (leukocoria), which also appears in other childhood eye conditions like retinoblastoma.
Diagnosis
A thorough eye examination is required, often under anesthesia in children. Diagnostic tests include:
- Fundoscopy: A detailed examination of the inside of the eye to identify any abnormal masses
- Ultrasound or MRI: Imaging to evaluate the size, extent, and location of the tumour
- Fine Needle Aspiration Biopsy (FNAB): Occasionally used to obtain a tissue sample for diagnosis
- Slit-lamp examination: May reveal subtle structural changes in the anterior segment of the eye
Treatment
Treatment depends on whether the tumour is benign or malignant and how far it has progressed:
- Surgical removal is the mainstay of treatment. If the tumour is localised and accessible, partial resection or iridocyclectomy (removal of part of the iris and ciliary body) may be performed.
- In more advanced cases, or when the tumour cannot be completely removed, enucleation (removal of the entire eye) may be necessary to prevent further spread and relieve symptoms.
- Radiation therapy or chemotherapy is rarely needed but may be considered if malignancy is confirmed or there is evidence of residual tumour or recurrence.
Regular follow-up is essential to monitor for recurrence or complications, although metastasis is extremely rare.
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