Retinoblastoma

Oncology

Survivors

Worldwide, the population of RB survivors has grown thanks to curative therapies developed through decades of research1-3,13. However, with these treatments come toxicities, causing survivors to face later-life threats to their health such as new cancers (especially those patients with bilateral RB or unilateral RB with a germline mutation in the gene Rb1) and therapy-related late side effects9-11,14,15. This risk is magnified by exposure to EBRT and chemotherapy exposures. For example, RB survivors have a cumulative incidence rate of secondary cancers reported as high as 36% at 50 years from diagnosis in patients with hereditary/bilateral RB and 5.7% for nonhereditary/unilateral patients11. Some chemotherapy drugs are toxic to the ear. Children receiving systemic chemotherapy need audiological assessments.

Therefore, it is imperative that all RB survivors have adequate surveillance follow-up for life, tailored to the therapies they received and their genetic makeup. Multiple survivorship guidelines and screening strategies have been developed over the past years16-18. In addition to this, survivorship programs are being developed in most paediatric cancer centres that can continue to provide care to survivors when they reach adulthood. Once your treatment is complete, you should ask your oncologist for a recommendation and referral for such survivorship programs or find a primary care physician willing to follow survivorship surveillance guidelines for RB survivors.


1Dimaras H, Kimani K, Dimba EAO, et al. Retinoblastoma. The Lancet.379(9824):1436-1446.
2Shields CL, Shields JA. Basic understanding of current classification and management of retinoblastoma. Curr Opin Ophthalmol. 2006;17(3):228-234.
3Rodriguez-Galindo C, Orbach DB, VanderVeen D. Retinoblastoma. Pediatric clinics of North America. 2015;62(1):201-223.
9Temming P, Viehmann A, Arendt M, et al. Pediatric second primary malignancies after retinoblastoma treatment. Pediatric blood & cancer. 2015;62(10):1799-1804.
10Temming P, Arendt M, Viehmann A, et al. Incidence of second cancers after radiotherapy and systemic chemotherapy in heritable retinoblastoma survivors: A report from the German reference center. Pediatric blood & cancer. 2017;64(1):71-80.
11Kleinerman RA, Tucker MA, Tarone RE, et al. Risk of new cancers after radiotherapy in long-term survivors of retinoblastoma: an extended follow-up. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2005;23(10):2272-2279.
13Chantada G, Schaiquevich P. Intra-arterial chemotherapy for retinoblastoma. JAMA Ophthalmology. 2016;134(10):1202-1203.
14Friedman DN, Chou JF, Oeffinger KC, et al. Chronic medical conditions in adult survivors of retinoblastoma: Results of the Retinoblastoma Survivor Study. Cancer. 2016;122(5):773-781.
15Ford JS, Chou JF, Sklar CA, et al. Psychosocial Outcomes in Adult Survivors of Retinoblastoma. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2015;33(31):3608-3614.
16Landier W, Bhatia S, Eshelman DA, et al. Development of risk-based guidelines for pediatric cancer survivors: the Children's Oncology Group Long-Term Follow-Up Guidelines from the Children's Oncology Group Late Effects Committee and Nursing Discipline. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2004;22(24):4979-4990.
17Nathan PC, Ness KK, Mahoney MC, et al. Screening and surveillance for second malignant neoplasms in adult survivors of childhood cancer: a report from the childhood cancer survivor study. Annals of internal medicine. 2010;153(7):442-451.
18Poplack DG, Fordis M, Landier W, Bhatia S, Hudson MM, Horowitz ME. Childhood cancer survivor care: development of the Passport for Care. Nature reviews Clinical oncology. 2014;11(12):740-750.
Last modified
03 February 2020