Medulloepithelioma of the Optic Nerve

Chavez, Mauricio R.; Mafee, Mahmood F.; Castillo, Bienvenido V.; Kaufman, Lawrence M.; Johnstone, H. F.; Edward, Deepak P.

doi:10.3928/0191-3913-20040101-11

Cited by 34 publications

(27 citation statements)

References 7 publications

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“…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.…”

Section: Introductionmentioning

confidence: 61%

Malignant Transformation of a Medulloepithelioma of the Optic Nerve

Lindegaard

Heegaard

Toft

et al. 2010

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Section: Introductionmentioning

confidence: 61%

Malignant Transformation of a Medulloepithelioma of the Optic Nerve

Lindegaard

Heegaard

Toft

et al. 2010

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“…The treatment of medulloepithelioma of the optic disc is also controversial due to its rarity [5]. Nevertheless, total resection by means of enucleation is usually curative.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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Medulloepithelioma of the optic disc

2011

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“…This is the most frequent congenital tumor of the ciliary body but is extremely infrequent in the optic nerve (ON) [1]. To the best of our knowledge, only 10 cases of medulloepithelioma of the ON have been reported to date [2-12]. This rare tumor usually presents within the first two to six years of life and is considered to be clinically malignant and entails significant vital compromise and mortality because of intracranial spreading or cerebral metastasis [2].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2012

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IntroductionMedulloepithelioma is a rare congenital tumor of the primitive medullary neuroepithelium. A significant proportion of patients with medulloepithelioma arising from the optic nerve die from intracranial spread or cerebral metastasis. Because it has no known distinct clinical features and because of its low frequency, this tumor presents within the first two to six years of life and is usually misdiagnosed clinically as a different type of optic nerve tumor. Here, we describe a new and atypical case of medulloepithelioma of the optic nerve in a 12-year-old boy. To the best of our knowledge, he is the oldest reported patient to present with this disease and, now as an adult, has the longest documented period of disease-free survival.Case presentationA 12-year-old Caucasian boy with headache and unilateral amaurosis was referred for a presumed optic nerve glioma to our hospital. A computed tomography scan showed optic nerve enlargement, and fundoscopy showed a whitish mass at the optic disc. Our patient had been followed at his local hospital for four years for an 'optic disc cyst' with no change or progression. He experienced mild progressive visual impairment during that period. He was admitted for resection, and a histopathological analysis revealed a medulloepithelioma of the optic nerve. Supplemental orbital radiotherapy was performed. He remained disease-free for 25 years.ConclusionsMedulloepithelioma of the optic nerve can clinically mimic more common pediatric tumors, such as optic glioma, meningioma, or retinoblastoma. Thus, medulloepithelioma should be included in the differential diagnoses of pediatric optic nerve lesions. Fundoscopy in these patients may provide relevant information for diagnosis. Anterior optic nerve medulloepitheliomas may behave differently from and have a better prognosis than medulloepitheliomas that have a more posterior location. Our case report illustrates that long-term survival can be achieved in patients with this malignant tumor.

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Medulloepithelioma of the Optic Nerve

Cited by 34 publications

References 7 publications

Malignant Transformation of a Medulloepithelioma of the Optic Nerve

Malignant Transformation of a Medulloepithelioma of the Optic Nerve

Medulloepithelioma of the optic disc

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

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