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Neuronal Dysplasia
Joseph Junewick, MD FACR
over 8 years ago
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Trilateral retinoblastom

Case Detail

Anatomy: Brain
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 8 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

Infant with history of laser treatment of bilateral retinoblastoma.


Case Images


Diagnosis

Retinoblastoma

Findings

MR – Axial T1 image demonstrates minimal bilateral ocular deformities related to treated retinoblastoma. Sagittal post-gadolinium T1 demonstrates a large lobulated and enhancing pituitary mass.

Discussion

Retinoblastoma is a congenital neoplasm of the eye arising from the neuroectodermal cells of the retina which become photoreceptors. The average age of presentation is between 13 and 18 months. There are hereditary and non-hereditary forms; the hereditary form is often bilateral and multifocal whereas the non-hereditary form is usually unilateral and unifocal. This case is an example of hereditary form with the so-called trilateral variant.

Leukokoria, strabismus, painful often infected eye and/or gluacoma may be present at presentation. There is an increased incidence of sarcoma and melanoma in patients with the hereditary form of retinoblastoma.

CT is best for initial evaluation since 90% of retinoblastoma lesions are calcified. Lesions may be endophytic and rupture into the vitreous, exophytic and extend through the retina or infiltrative. On MR the lesions are often hyperintense on T1. Metastases occur along the optic nerves, subarchnoid spaces and hematogenous. Pineal and/or pituitary involvement represents trilateral and/or quadrilateral involvement.



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