Retinoblastoma

About Retinoblastoma

Retinoblastoma is the most common tumor of the eye that develops from the retinal layer of the eye. It diagnosed in less than 3 years of age in about 80% of children, the mean age of diagnosis is around 14 months. The familial cases present early in life thus if any of the parents have Retinoblastoma or any child develops retinoblastoma (especially younger child with bilateral disease) genetic counseling should be done.

 

Why does it occur?

About fifty percent have familial history and these present earlier than sporadic ones and are often bilateral.

 

Clinical features

Abnormal looking eye- leukocoria
Loss of vision
Squint (strabismus)
Inflamed eye (reddish)

 

How do we diagnose?

  • Routine blood tests
  • Eye examination by an ophthalmologist and often requires anesthesia for small children. This examination is important as it maps the disease and has a large contribution in planning treatment for the eye. The decisions about the possibility of eye-saving procedure and follow up during the treatment for response assessment are based on this.
  • Sonography- most of the Retinoblastoma show the presence of calcification which can easily be picked up in sonography.
  • MRI orbit- The local extent of the retinoblastoma beyond eye is evaluated by MRI and it requires anesthesia for small kids. The orbits including brain need to be evaluated. There optic nerve and extraocular extension are seen on MRI and presence of these means extraocular disease.
  • Classification of Retinoblastoma

 

Treatment

It depends on the extent of the local disease. If it is diagnosed early and not extended outside eye it can be managed with intra-arterial chemotherapy and the eye can be saved. The treatment includes local therapy (intra-arterial chemotherapy, Laser photocoagulation, Thermotherapy, Cryotherapy etc), systemic therapy (chemotherapy), surgery (enucleating or exenterating) and radiation. The extent and choice of treatment are dependent on the extent of disease present and varies according to the stage at presentation.

 

Outcomes

The retinoblastoma is curable if detected early, even in a locally advanced disease where the eye cannot be saved and needs to removed the outcomes are good. Eye salvage is possible in very small tumors and also depending on the location of the primary disease.

 

Follow up

After treatment completion in the initial year, three monthly eye examination should be done under anesthesia. Any child falling into the hereditary group should be referred to the genetic clinic.

NH-SRCC Children's Hospital,

Haji Ali, Mumbai, India

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