(CR44) A Rare Case of Adult-Onset Medulloepithelioma With Malignant Transformation And Extra Orbital Spread

Introduction:

Intraocular medulloepithelioma is a rare congenital neoplasm, which is rare in adults. Malignant intraocular medulloepithelioma is frequently associated with local invasion, however rarely have extra-orbital spread. We report an atypical case of adult malignant transformation of a benign medulloepithelioma with intracranial extension.

Case Presentation:

A 54-year-old gentleman was diagnosed with left eye benign medulloepithelioma at a primary hospital which was consistent with fine needle biopsy. After 2 years, he had painful progressive proptosis of the left eye. On examination, the left eye was grossly proptosed with inferior displacement. A protrusion of intraocular mass with overlying scleral tissue erosion. Left eye vision was non preceptive to light with positive RAPD. CT orbit revealed a large heterogenous mass engulfing the entire left globe and lacrimal gland with infiltration of retro-orbital structures as well as left eyelid and canthi. No intracranial extension or sinus involvement was observed at this time. Patient subsequently underwent left total exenteration. Histopathology showed malignant non-teratoid medulloepithelioma with clear margins. However, four months after exenteration, noted recurrence of tumor with intracranial extension into left middle cranial fossa and left cavernous sinus involvement. Patient then underwent chemotherapy and radiotherapy. Unfortunately, after 2 cycles of chemotherapy, he succumbed to the illness.

Discussion/Conclusion:

Medulloepithelioma is predominantly a pediatric tumor but must be included as one of the differential diagnosis of orbital tumors in adults. Most important prognostic feature to predict metastasis is extraocular extension of the tumor. Hence, we need to be highly vigilant in the management of adult    medulloepithelioma once extra ocular spread set in.