A patient with hereditary retinoblastoma is at increased risk for a soft tissue sarcoma as well as other tumors. Monitoring for subsequent tumors should be part of patient follow-up.
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
Soft tissue sarcomas include:
(1) leiomyosarcoma, including blood vessels
(2) fibrosarcoma
(3) pleomorphic sarcoma
(4) rhabdomyosarcoma
(5) liposarcoma
(6) other
Risk factors, especially if these were given before 1 year of age:
(1) radiation therapy
(2) alkylating agent
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