Orbital medulloepitheliomas—With extensive local invasion and metastasis: A series of three cases with review of literature

SummaryIntraocular medulloepithelioma is a rare embryonal neoplasm which usually presents in childhood as a ciliary body mass. This tumor is usually confined to the orbit and rarely tends to be locally aggressive and metastatic. We describe three rare cases of metastatic malignant teratoid medulloepithelioma. The patients were males aged 7, 16 and 25 years with right eye involvement in all the cases. All patients had local recurrence (multiple in two) and metastasis to the intraparotid and cervical lymph nodes. Distinction from round cell tumors and primary salivary gland neoplasms occurring in the parotid gland is strongly warranted, especially in biopsy samples, because the biological behaviour and treatment of medulloepithelioma differs greatly from the above lesions.