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

Journal of Medical Case Reports
Natalia Pastora-SalvadorFernando Carceller-Benito

Abstract

Medulloepithelioma 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. A 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 rev...Continue Reading

References

Jun 1, 1974·Archives of Ophthalmology·W R GreenR R Trotter
Oct 24, 1997·Archives of Ophthalmology·M O'KeefeJ Dudgeon
Aug 10, 2000·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·B ChidambaramV Balasubramaniam
Jul 1, 1957·American Journal of Ophthalmology·A B REESE
Feb 21, 2004·Journal of Pediatric Ophthalmology and Strabismus·Mauricio ChavezDeepak P Edward
Jun 2, 2005·Neuroimaging Clinics of North America·Thasarat S VajaranantDeepak P Edward
Feb 16, 2007·Pathology International·Hidehiro TakeiMeenakshi B Bhattacharjee
Jun 6, 2008·Journal of Pediatric Hematology/oncology·Sameer BakhshiDaya Nand Sharma
Nov 26, 2008·Survey of Ophthalmology·Hui Bae Harold LeeJames R Patrinely
May 26, 2010·Orbit·Jens LindegaardJan Ulrik Prause
May 26, 2010·Clinical & Experimental Ophthalmology·Neelam PushkerSameer Bakhshi
Jun 17, 2011·Human Pathology·Zélia M CorrêaAbbot G Spaulding

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Citations

May 20, 2020·Ophthalmic Plastic and Reconstructive Surgery·Ryan A GalloAndrew J Rong

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