NEOPLASMA

MAHARANI
 RETINOBLASTOMA

Rare malignant tumor of childhood is fatal if untreated

90% of cases
the diagnosis is made before the end of the third year
30% of cases bilateral
Because of the loss from both members of the
chromosome pair of the normally protective dominant
allele at a single locus within chromosome band 13q14
 RETINOBLASTOMA

Affected parents children have nearly 50% chance

of having the disease (bilateral tumor & multifocal)
Unaffected parents who have produce 1 child with
RB run 4-7% risk of having a subsequent child with the
disease
Therapy : focal laser therapy, radiotherapy ( episcleral
plaque, external beam),photocoagulation,
chemotherapy, enucleation