Bilateral retinoblastoma

Case contributed by Shervin Sharifkashani
Diagnosis almost certain

Presentation

Bilateral leukocoria and exodeviation of the right eye.

Patient Data

Age: 5 months
Gender: Male
ct

There are relatively hyperdense solid mass lesions within both eyes' vitreous, occupying most of the vitreous. The anterior margin of each mass is in close contact with the posterior margin of the lens.

There is also extrabulbar extension of the mass at the superior temporal margin of the left eye. The optic nerve cups are also involved by the masses.

A few scattered foci of microcalcification within the right eye mass lesion and exodeviation of the right eye.

Case Discussion

Retinoblastoma usually develops in early childhood and is the most common primary intraocular malignancy in children. Most cases of retinoblastoma are unilateral. Mutations in the RB1 gene are responsible for most cases of hereditary retinoblastoma, which can be bilateral.

The two most common symptoms revealing retinoblastoma are leukocoria and strabismus. Nowadays, high-resolution MR imaging is the imaging modality of choice for the evaluation of the extension of the retinoblastoma but CT scan is still used for detection of microcalcification within the lesion which is a primary diagnostic clue.

In the management of retinoblastoma, one must consider the visual outcome, the possibly hereditary nature of the disease, and life-threatening risk.

In most bilateral cases, conservative treatment for at least one eye is possible including laser alone or combined with chemotherapy, cryotherapy, and brachytherapy.

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