BY KATHERINE LOUIE
What is Retinoblastoma?
Retinoblastoma is a rare, cancerous tumor of a part of the eye called
The cancer generally affects children under the age of 6. It is most
commonly diagnosed in children aged 1 - 2 years.
Retinoblastoma is a relatively uncommon tumor of childhood that
arises in the retina and accounts for about 3% of the cancers occurring
in children younger than 15 years.
The estimated annual incidence in the United States is approximately
10 to 14 per million children aged 0 to 4 years. Although
retinoblastoma may occur at any age, it most often occurs in younger
children, usually before age 2 years. Ninety-five percent of cases are
diagnosed before age 5 years. Retinoblastoma diagnosed in patients
older than 5 years has a poorer prognosis.
Retinoblastoma is usually confined to the eye, and as a
result, more than 90% of children with intraocular
retinoblastoma will be cured.
Background & History
There has been plenty of controversy over the
discovery of Retinoblastoma.
A man named Virchow first described the tumor as a
retinal glioma in 1864.
About 35 years later, Flexner and Wintersteiner
noted that many similarities existed between tumor
rosettes and photoreceptors of the adult retina.
It wasn't until the 1920s that Verhoeff, another top
scientist, claimed that the tumor arose from
embryonic retinal cells and hence proposed the
One or both eyes may be affected.
They pupil may appear white or have white spots. A
white glow in the eye is often seen in photographs taken
with a flash. Instead of the typical "red eye" from the
flash, the pupil may appear white or distorted.
Other symptoms can include:
Eyes that do not align
Eye pain and redness
Differing iris colors in each eye
If the cancer has spread, bone pain and other symptoms may occur.
The doctor will perform a complete physical exam,
including an eye exam. The following tests may be
Eye exam with dilation of the pupil
CT scan or MRI of the head
Ultrasound of the eye (head and eye echoencephalogram)
Bone marrow biopsy and cerebrospinal fluid examination in
the case of more aggressive tumors
Treatment options depend upon the size and
location of the tumor.
Small tumors may be treated by laser surgery or cryotherapy.
Radiation is used for both local tumor and for larger tumors.
Chemotherapy may be needed if the tumor has spread beyond
The eye may need to be removed (a procedure called
eucleation) if the tumor does not respond to other treatments.
In some cases, it may be the first treatment.
If the cancer has not spread beyond the eye, almost all patients
can be cured. A cure, however, may require aggressive
treatment and even removal of the eye in order to be
If the cancer has spread beyond the eye, the likelihood of a
cure is lower and depends on how the tumor has spread.
Blindness can occur in the affected eye.
The tumor can spread to the eye socket through the
optic nerve. It may also spread to the brain, lungs,
It has been recommended that children with
heritable retinoblastoma should be screened using
magnetic resonance neuroimaging or computerized
tomography (CT) scan every 6 months after
diagnosis until age 5 years, since these tumors are
not likely to occur after this time
Genetic counseling can help families understand the
risk of retinoblastoma, and is a vital step when more
than one family member has had the disease, or if
the retinoblastoma occurs in both eyes.