<prompt> Second most common malignancy in a patient with retinoblastoma is:NephroblastomaOsteosarcomaNeuroblastomaCraniopharyngioma  <response>: Retinoblastoma: It is the most common primary intraocular malignancy of childhood. The mutation occurs in the retinoblastoma gene Rb at chromosome 13q14. This is a tumor suppressor gene. One mutated copy may be inherited in an autosomal dominant fashion germline mutation. If a second mutation spontaneously occurs in any cell, tumorigenesis is likely. Individuals with a germinal mutation are at risk for the development of tumors other than retinoblastoma pineal tumors, osteosarcoma, and other soft tissue sarcomas. Ref: Braverman R.S. 2012. Chapter 16. Eye. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer Eds, CURRENT Diagnosis Treatment: Pediatrics, 21e. 