• Introduction
  • Total Childhood Cancer
  • Leukemias
  • Lymphomas
  • Kidney Cancers
  • Bone Cancers
  • Soft-Tissue Sarcomas
  • Incidence Rates by County
  • Discussion
  • Childhood Cancer

    Chapter II

    Rates and Risk Factors
    for Specific Childhood Cancers


    OTHER NERVOUS SYSTEM CANCER

    Retinoblastoma

    Retinoblastoma is a cancer which arises in the developing cells of the eye. Histologically, it is similar to other malignant neoplasms of neural origin, such as neuroblastoma and medulloblastoma (the most common type of PNET).

    Incidence Rates

    The incidence of retinoblastoma was 0.4 per 100,000 children in NJ and the US during 1979-1995 (Table 1a). In NJ, the incidence rate was 0.5 per 100,000 black children and 0.4 per 100,000 white children (Table 1b). In the US, the incidence rate was 0.4 per 100,000 among black and white children.

    Demographic Patterns and Survival Rates

    The incidence is highest under age one and declines to a very low rate by age five. The survival rate is very high--about 90 percent of the children survive at least five years. Girls have higher incidence rates than boys, and black children have higher incidence rates than white children.

    Genetic and Behavioral Risk Factors

    Hereditary retinoblastoma accounts for 40 percent of all retinoblastomas. It usually appears in both eyes and is diagnosed earlier than the nonfamilial type. A missing or defective cancer-suppressor gene has been identified which causes predisposition to retinoblastoma. Carriers can be screened so that treatment can be initiated early among those who have a cancer. Since poor vision results in cases with macular involvement, early detection may help retain vision. High consumption of fruits and vegetables and use of multivitamins in pregnancy may reduce the risk of development of retinoblastoma.22


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