Which of the following tumor is the prototype of hereditary tumors of childhood?
Correct Answer: Retinoblastoma
Description: Retinoblastoma is the prototype of hereditary cancers due to a mutation in the retinoblastoma gene (RB1), which is located on the long arm of chromosome 13 (13q14). This gene is a tumor-suppressor gene that normally controls cellular growth. When the gene is inactivated, as in retinoblastoma, cellular growth is uncontrolled. Uncontrolled cell growth leads to tumor formation. Inactivation of both RB1 alleles within the same cell is required for tumor formation. Ref: Graham D.K., Quinones R.R., Keating A.K., Maloney K., Foreman N.K., Giller R.H., Greffe B.S. (2012). Chapter 31. Neoplastic Disease. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.
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