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Retinoblastoma

Slideshow

  • Colored CT scan of a coronal section of the skull from an 8-year-old with retinoblastoma tumors (light areas) in each eye.

    Colored CT Scan of an 8-Year-Old with Retinoblastoma

    Colored CT scan of a coronal section of the skull from an 8-year-old with retinoblastoma tumors (light areas) in each eye.

  • Colored axial MRI scan of the skull highlighting a retinoblastoma tumor (orange/white bulge) at the back of the right eye.

    Retinoblastoma Tumor at Back of Right Eye

    Colored axial MRI scan of the skull highlighting a retinoblastoma tumor (orange/white bulge) at the back of the right eye.

  • Prominent white spots (leukocoria) in the pupil of both eyes. The white glow is characteristic of the retinoblastoma tumor. To better access the tumor, fluorescein angiography is done under anesthesia.

    Characteristic White Dots

    Prominent white spots (leukocoria) in the pupil of both eyes. The white glow is characteristic of the retinoblastoma tumor. To better access the tumor, fluorescein angiography is done under anesthesia.

  • Multiple tumors of the retina appear in the dilated pupil of this patient. The tumors can easily be seen with the naked eye.

    Multiple Tumors of the Retina

    Multiple tumors of the retina appear in the dilated pupil of this patient. The tumors can easily be seen with the naked eye.

  • Abnormal malignant cell growth begins from the neural tissue of the retina at the back of the eye. The tumor has the potential to grow and take up the whole eye cavity.

    Abnomal Malignant Cell Growth

    Abnormal malignant cell growth begins from the neural tissue of the retina at the back of the eye. The tumor has the potential to grow and take up the whole eye cavity.

  • The malignant tumor growing from the retinal tissue blocks light from reaching the back of the eye, causing problems with vision.

    Tumor Growing from Retinal Tissue

    The malignant tumor growing from the retinal tissue blocks light from reaching the back of the eye, causing problems with vision.

  • To diagnose retinoblastoma, the fundus of the eye is examined with an ophthalmoscopy. The large white spot highlights a retinoblastoma. MRI, CT scan, blood work, and genetic testing are also performed to confirm diagnosis and identify if the cancer has spread.

    Ophthalmoscopy

    To diagnose retinoblastoma, the fundus of the eye is examined with an ophthalmoscopy. The large white spot highlights a retinoblastoma. MRI, CT scan, blood work, and genetic testing are also performed to confirm diagnosis and identify if the cancer has spread.

Retinoblastoma is a rare cancer that is caused by a mutation in the retinoblastoma gene (RB1) controlling cell division of the retina. The disease can be hereditary if the mutated RB1 gene runs in a family, and can cause a 50% chance of the child developing the disease. This malignant cancer of the retina can spread to the brain if not caught and treated early. Most often, retinoblastoma is seen during childhood between 6 and 24 months of age. In the United States, more than 95% of children survive when the disease is caught early and treated.

Usually, there is no pain associated with retinoblastoma. In most cases, only one eye is affected (70%), but sometimes both eyes are affected (30%). Over time, the child loses vision, and the eye may start to drift. The pupil usually appears white or has white spots. On examination, retinoblastoma has a classic chalky-white appearance with prominent blood vessels. Other symptoms include different-colored irises, “glazed” eyes, and pupils that are unresponsive to light.

Treatment options depend on the size, location, and quantity of tumors, and are often used in combinations. The treatment methods include chemoreduction, thermotherapy, laser photocoagulation, cryotherapy, plaque radiotherapy, external beam radiotherapy, chemotherapy and enucleation.

The goal is to save the patient’s life and protect their vision.

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