Retinoblastoma Retinoblastom a BY KATHERINE LOUIE What is Retinoblastoma Retinoblastoma

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Retinoblastoma Retinoblastom a BY KATHERINE LOUIE What is Retinoblastoma Retinoblastoma Powered By Docstoc
                What is Retinoblastoma?

Retinoblastoma is a rare, cancerous tumor of a part of the eye called
the retina.

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
  name "retinoblastoma."

 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:
    Crossed eyes
    Double vision
    Eyes that do not align
    Eye pain and redness
    Poor vision
    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.
    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.

 Expectations:
   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,
 and bones.

 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.


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