| Introduction | | | | irises |
| This is the most common primary tumor of the eye in | | | | Investigations:oExamination under general anesthesia |
| children. It arises from the retina. .The retina is a layer | | | | using RetcamoUltrasoundoCT (CAT) scanoMRIoCSF |
| of nerve tissue that coats the back of the eye, which | | | | examinationoBone marrow studiesoBone scan in |
| is important for vision of person. Retinoblasts | | | | advanced diseaseoChromosomal analysis (in certain |
| (immature cells of the retina) multiply during gestation | | | | cases) |
| and early life, to make enough cells to create the | | | | Management: |
| retina. As child grows, these cells mature & no | | | | 1.Surgery to remove the eye, known as enucleation. |
| longer differentiate. If these immature retinoblasts turn | | | | This s done when there is no vision in eye to save |
| into cancer cells, retinoblastoma develops, the cause of | | | | further spread & life. Enucleation may also be |
| which is unknown. The gene responsible is RB1 gene. | | | | recommended if the tumor does not respond to |
| Incidence: | | | | treatment. |
| The annual incidence is one in 20000 children. It occurs | | | | 2.Chemotherapy is used to shrink tumors in the eye. |
| most often in children under 4 years of age. | | | | This approach is often used in children with bilateral |
| Types: | | | | disease (both eyes) for saving at least one eye which |
| Hereditary:oHereditary form of Retinoblastoma occurs | | | | has less disease. It is also used in small tumors where |
| in 40%.oMay have more than one tumoroTumor often | | | | the eye vision is present to save the eye. in |
| affects both eyesoMay have tumors in other parts of | | | | combination with other measures such as |
| the bodyoAt increased risk for other cancers later in | | | | 1.Photocoagulation--using laser light to destroy blood |
| life | | | | vessels supplying the tumor. |
| Non-hereditary:oMost children with retinoblastoma | | | | 2.Thermotherapy--using heat to destroy tumor cells |
| (60%) do not have the genetic form.oThey develop | | | | 3.Cryotherapy--using extreme cold to destroy tumor |
| tumor in only one eye i.e. unilateral.oThese children do | | | | cells |
| not have an increased risk of developing other | | | | 4.Radiation Therapy-It is used for control of local |
| cancers.oTheir offspring have the same risk of | | | | disease with preservation of vision. Radiation plaque |
| developing retinoblastoma as other children in the | | | | therapy and particle beam radiotherapy are used |
| population. | | | | frequently. |
| Symptoms:oA pupil that looks white or red instead of | | | | Prognosis: |
| the usual black i.e. called as white or cat's eye | | | | The five-year survival rate for children with |
| reflex.oA crossed eye i.e. strabismus.oPoor visionoA | | | | retinoblastoma is more than 90%. |
| red, painful eyeoAn enlarged pupiloDifferently colored | | | | |