Teratoid malignant medulloepithelioma of the optic nerve: report of a case and review of the literature

Abstract

Medulloepithelioma of the optic nerve is extremely rare and can simulate an optic nerve glioma. We report a histopathologically proven medulloepithelioma in a 3-year-old child with computer tomographic (CT) and magnetic resonance imaging (MRI) features.

Malignant Transformation of a Medulloepithelioma of the Optic Nerve

“…Only 8 cases have been reported in the literature (Andersen, 1971;Biswas et al, 1999;Chavez et al, 2004;Chidambaram et al, 2000;Fandino et al, 2007;Green et al, 1974;Reese, 1957;Takei et al, 2007). We here describe the first case of a benign medulloepithelioma of the optic nerve that later transformed into a malignant medulloepithelioma.…”

“…Of all patients reported with an optic nerve medulloepithelioma, three (37.5%) died as a consequence of their tumour, even though the time to last follow-up was short (median 18 months, range 0-66 months). Two patients died with direct intracranial tumour extension (Chidambaram et al, 2000;Reese, 1957) the third patient developed metastases to the brain (Biswas et al, 1999). Hence, medulloepithelioma of the optic nerve is a tumour with a considerable mortality rate.…”

Medulloepithelioma of the optic disc

“…Invasive intraocular medulloepitheliomas extend out of the eye occasionally via the sclera or optic nerve but rarely metastasize unless neglected or mismanaged. To our knowledge, only 8 other cases of medulloepithelioma of the optic nerve have been reported in the peer-reviewed literature at this time [1,[5][6][7][8][9][10][11] (Table 1). However, our case is unique in that the tumor was diagnosed by means of cytopathologic study of a tumor specimen obtained by incisional biopsy using a 25-gauge vitrectomy system.…”

“…Other therapeutic options include observation, chemotherapy, focal radiation therapy (stereotactic radiation therapy [SRT], gamma knife, and proton beam irradiation) [5,6]. Because there are few reports in the literature regarding the use of adjuvant chemotherapy and radiotherapy after enucleation and no scientific evidence of improvement in patient survival [5,7,11], the benefit of such additional treatment is speculative. In addition, it is not known if chemoreduction of the tumor mass to facilitate the resection and improve prognosis would be effective; and to our knowledge, there is no reported experience of intraocular chemotherapy.…”

Atypical clinical presentation and long-term survival in a patient with optic nerve medulloepithelioma: a case report

“…In patients with medulloepitheliomas arising from the posterior ON, mortality is usually caused by untreatable intracranial invasion or cerebral metastasis [3,4,9,10]. In contrast, medulloepitheliomas arising from the anterior ON may have a better prognosis because a greater proportion of them are symptomatic, leading to earlier diagnosis [2,4-7,11], and they have a smaller tendency to extend into the cranium [8].…”