Intraocular malignant teratoid medulloepithelioma in an adult: clinicopathological case report and review of the literature

Abstract

ABSTRACT.Purpose: To report a case of intraocular medulloepithelioma, an embryonal tumour with extremely rare presentation in adults. Method: The case of a 44-year-old man with intraocular malignant teratoid medulloepithelioma, primarily diagnosed as intraocular teratoma, is described and the literature on this subject is reviewed. Results: The patient presented with progressive proptosis caused by a tumour in the left eyeball. He had a 28-year history of loss of vision in the left eye. Histopathological examination of the enucleated eye demonstrated an intraocular teratoma. No adjuvant treatment was given. Six months later the patient presented with massive progression in the left orbit and intracranial invasion. Cisplatin-based chemotherapy was administered, but discontinued after two cycles due to poor tolerance and lack of response. At subsequent pathology review, a final diagnosis of malignant teratoid medulloepithelioma was made. Salvage radiotherapy (60 Gy in 30 fractions) resulted in partial response of the intracranial lesion. However, the patient died 6 months later due to intracranial tumour progression. Conclusion: Medulloepithelioma should be considered in the differential diagnosis of intraocular tumours in adults, especially in the case of coexisting, longstanding ocular symptoms. In some cases this disease is very aggressive.

Intraocular Medulloepithelioma

“…8,11 At least 11 cases of medulloepithelioma have been reported in persons older than 20 years. 12 The oldest patient with a histologically documented tumor was 79 years. 13 Based on the series from the AFIP and previous literature reviews, there is no sex or racial predilection.…”

“…13 Based on the series from the AFIP and previous literature reviews, there is no sex or racial predilection. [8][9][10][11][12] Tumors arise in the ciliary body, unilaterally. Exceptional cases have been reported in the optic nerve and retina.…”

“…8,16,17 The fact that most cases in adults are malignant suggests that clinical recognition may follow belated malignant transformation. 11,12,17 Benign tumors are presumably detected because decades of slow growth lead to eventual clinical detection. The terms acquired medulloepithelioma or adult medulloepithelioma are contradictions based on the current concept of tumor development.…”

Nonteratoid Medulloepithelioma Presenting in a 78-Year-Old Male

“…Though experimental tumors have been created from ciliary body stem cells, in general, the term medulloepithelioma is reserved for embryonic neoplasms believed to arise from embryonal or fetal tissues, making ‘embryonal medulloepithelioma' redundant and ‘adult medulloepithelioma' contradictory [5,6,7]. At least 16 cases of medulloepithelioma have been reported in patients older than 20 years, with the oldest in a 79-year-old man [9,10]. We documented another male whose congenital tumor became symptomatic in his 7th decade of life.…”

On the Classification and Grading of Medulloepithelioma of the Eye

“…Neither could single nucleotide polymorphism array detect differences in copy number aberrations between benign and malignant medulloepithelioma. The fact that adult patients are more often diagnosed with malignant medulloepithelioma reflects the increased chance of malignant progression in time (table 1) [9,10,11,12,13,14,15]. Three documented cases of medulloepithelioma in adults have metastasized, which is a relatively high percentage when compared to the overall chance of metastasis for this tumor (table 1) [8,16].…”