Page 131 - NAME OF CONDITION: REFRACTIVE ERRORS

NAME OF CONDITION : RETINOBLASTOMA

I. WHEN TO SUSPECT/ RECOGNIZE?

a) Introduction:

Retinoblastoma is the most common intraocular malignancy of childhood. It is
second only to uveal melanoma in the frequency of occurrence of malignant
intraocular tumors. Although it is highly malignant, it is eminently curable. The

recent advances such as identification of genetic mutations, replacement of external
beam radiotherapy by chemoreduction as the primary management modality, use of
chemoreduction to minimize the size of regression scar with consequent
optimization of visual potential, identification of histopathologic high-risk factors
following enucleation and provision of adjuvant therapy to reduce the incidence of
systemic metastasis, protocol-based management of retinoblastoma with accidental
perforation or intraocular surgery and aggressive multimodal therapy in the
management of orbital retinoblastoma have contributed to improved outcome in
terms of better survival, improved eye salvage and potential for optimal visual
recovery. Life salvage has improved from 30% in the 1930s to nearly 95% now. Early
diagnosis and appropriate referral are the keys to better outcome.

b)Case definition:

Retinoblastoma is a malignant tumor arising from the retinal progenitor cell or the
retinal stem cell during the period of retinal development and occurs predominantly
in children under 3 years of age.

II.INCIDENCE OF THE CONDITION IN OUR COUNTRY

The reported incidence of retinoblastoma ranges from 1 in 10,000 to 1 in 20,000 live
births. There are no good epidemiological studies that provide the accurate data on
incidence of retinoblastoma in India. It is estimated that 1500 new cases of
retinoblastoma occur in India every year. The ICMR Indian Retinoblastoma Registry

has recorded 1019 cases from 8 treatment centers between April 2009 to December
2010. There is no racial or gender predisposition in the incidence of retinoblastoma.
Retinoblastoma is bilateral in about 25 to 35% of cases. The average age at diagnosis
is 18 months, unilateral cases being diagnosed at around 24 months and bilateral
cases before 12 months.

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