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Intraocular Retinoblastoma

What is Intraocular Retinoblastoma?

Retinoblastoma is a cancer that starts in the retina, the very back part of the eye. The retina is part of the eye, which is the organ that lets you see. The retina is the inner layer of cells in the back part of the eyeball. It is made up of special nerve cells that are sensitive to light.   

About 3 out of 4 children with retinoblastoma have a tumor in only one eye. In about 1 case in 4, both eyes are affected.

What are the symptoms of Intraocular Retinoblastoma? 

Signs and symptoms of retinoblastoma include:

  • A white reflection or glare in a child’s pupil when exposed to light, such as in a photograph taken with a flash (called leukocoria or cat’s eye reflex)
  • Eyes that are crossed or do not appear to look in the same direction (called strabismus) 
  • Redness and inflammation of the white part of the eye
  • Blurred vision
  • Eye pain
  • Squinting 
  • An enlarged eyeball bulging of the eye out of the eye socket (called proptosis)
  • 2 different coloured irises (called heterochromia)
  • Increased pressure in the eye (called glaucoma) that can lead to blindness  

What causes Intraocular Retinoblastoma? 

During the early stages of development, the eyes have cells called retinoblasts that divide into new cells and fill the retina. At a certain point, these cells stop dividing and develop into mature retinal cells.  

In some cases, instead of maturing into special cells that detect light, some retinoblasts continue to divide and grow out of control, forming a cancer known as retinoblastoma. 

This is caused by a change or mutation in a gene called the retinoblastoma (RB1) gene. The normal RB1 gene helps keep cells from growing out of control, but the change in the gene stops it from working like it should.

How is Intraocular Retinoblastoma diagnosed?

Diagnosing retinoblastoma usually begins with a visit to the doctor, who may refer the patient to a specialist or order tests to check for retinoblastoma or other health problems.   

Further tests include:

  • Physical
  • Eye exam
  • Ultrasound
  • RBI gene test
  • MRI
  • CT scan
  • Bone scan
  • Bone marrow biopsy 

What are the treatments for Intraocular Retinoblastoma?

Treatments include:

  • Cryosurgery 
  • Thermotherapy 
  • Laser surgery 
  • Chemotherapy 
  • Radiation therapy 
  • Stem cell transplant 
  • Eye-removal therapy (enucleation)

Where can I find out more about Intraocular Retinoblastoma? 

Intraocular Retinoblastoma Articles

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