Malignant Transformation of a Medulloepithelioma of the Optic Nerve

Lindegaard, Jens; Heegaard, Steffen; Toft, Peter Bjerre; Nysom, Karsten; Prause, Jan Ulrik

doi:10.3109/01676830903421200

Cited by 15 publications

(4 citation statements)

References 14 publications

(14 reference statements)

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“…Though typically arising from the ciliary body, medulloepitheliomas have on rare occasion been reported arising from the optic nerve [31]. Most such cases have been malignant tumors.…”

Section: Intraocular Neoplasmsmentioning

confidence: 99%

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A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

Rao

Naheedy

Chen

et al. 2013

Journal of Oncology

119

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While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed.

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“…Though typically arising from the ciliary body, medulloepitheliomas have on rare occasion been reported arising from the optic nerve [31]. Most such cases have been malignant tumors.…”

Section: Intraocular Neoplasmsmentioning

confidence: 99%

“…They can spontaneously resolve [33] but may become symptomatic by enlarging leaking, generating macular edema, accumulating lipid exudates, and forming serous retinal detachments [31, 33]. Additionally, elevated intraocular pressure and glaucoma have been reported in association with retinal astrocytic hamartomas.…”

Section: Intraocular Neoplasmsmentioning

confidence: 99%

A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

Rao

Naheedy

Chen

et al. 2013

Journal of Oncology

119

View full text Add to dashboard Cite

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“…However, the white mass in the optic disc was not typical for a glioma, and the four-year history of a growing papillary mass was not typical for a malignant process. This could be caused by low aggressiveness of the tumor or late malignant transformation [6]. Despite the unusualness of this presentation, this patient was successfully managed with surgery and adjuvant radiotherapy and maintained a disease-free survival of 25 years, which, to the best of our knowledge, is the longest reported to date.…”

Section: Discussionmentioning

confidence: 97%

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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“…The presence of poorly differentiated neuroblastic cells also implies increased mitotic activity and thus requires no separate evaluation. The progression from benign to malignant medulloepithelioma has been documented once, further strengthening the argument [19]. The reduction of two classifying tumor entities into one and the introduction of a simple grading scheme might facilitate consistency in pathology reports and make the comparison for clinical follow-up and treatment decisions easier.…”

Section: Tablementioning

confidence: 89%