A patient with hereditary retinoblastoma is at risk for secondary malignant tumors other than sarcomas. A survivor of hereditary retinoblastoma should be periodically evaluated for the emergence of secondary malignancies, especially as the patient ages.
Gene affected: RB1 (RB transcriptional corepressor 1), at 13q14.2
Clinical features of hereditary retinoblastoma:
(1) unilateral or bilateral retinoblastoma
(2) family history of retinoblastoma
(3) germline mutation in RB1
Nonsarcomatous malignancies with an increased risk include:
(1) nasal cavity
(2) pineoblastoma
(3) central nervous system tumors
(4) oral cavity
(5) breast
(6) melanoma
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