Successful treatment of metastatic congenital intraocular medulloepithelioma with neoadjuvant chemotherapy, enucleation and superficial parotidectomy

PURPOSE: To report a case of metastatic intraocular medulloepithelioma successfully treated with neoadjuvant chemotherapy, superficial parotidectomy, and enucleation. OBSERVATIONS: A 5-year-old male with history of cataract surgery, glaucoma drainage device, endocyclophotocoagulation, scleral patch grafting, and chronic posterior “inflammation” in a blind left eye presented with a rapidly enlarging painful mass under the left upper eyelid. Biopsy of the conjunctival mass and fine needle aspiration of an enlarged preauricular lymph node revealed medulloepithelioma, which was also seen in the left parotid gland on positron emission tomography (PET) scan. The patient's father refused exenteration, so the patient received 3 cycles of vincristine, cisplatin, cyclophosphamide, and etoposide per a retinoblastoma protocol. Repeat magnetic resonance imaging (MRI) showed regression of ocular extension, and an enucleation was performed, histologically confirming the diagnosis of malignant, non-teratoid medulloepithelioma. The child later underwent superficial parotidectomy and received an additional round of chemotherapy. There has been no evidence of recurrence for 9 years. CONCLUSIONS AND IMPORTANCE: There is no standard treatment for metastatic intraocular medulloepithelioma. The neoadjuvant chemotherapy regimen used in our patient led to regression of the extrascleral extension of the tumor, allowing for enucleation rather than a more disfiguring exenteration, as well as likely improving his prognosis. We believe that it is reasonable to consider neoadjuvant chemotherapy for patients with extrascleral and/or metastatic medulloepithelioma.