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Second Tumors in Retinoblastoma Survivors

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Part of the Essentials in Ophthalmology book series (ESSENTIALS)

Abstract

Improvements in treatment over the past century have greatly increased survival for retinoblastoma patients. However, as survival has improved, second cancers have emerged as a major cause of morbidity and mortality in retinoblastoma survivors. Several large cohort studies of long-term survivors have consistently reported that hereditary survivors have a greater risk of second cancers compared to non-hereditary survivors, with elevated risks persisting into adulthood. The cumulative incidence of second cancers 50 years after retinoblastoma diagnosis is 36 % in hereditary survivors compared to only 6 % in non-hereditary survivors. The most common second cancers diagnosed in hereditary survivors are bone sarcomas, soft tissue sarcomas, and melanoma, which together account for 82 % of second cancers in these survivors. Increasing doses of radiation significantly increase the risk of bone and soft tissue sarcomas, but not melanoma. Chemotherapy in combination with radiotherapy increases the risk of bone sarcomas and soft tissue sarcomas, particularly leiomyosarcoma. Although radiotherapy is used less frequently in current treatment approaches for retinoblastoma, survivors previously treated with radiotherapy should be screened for the risk of second cancers into adulthood and encouraged to follow cancer prevention strategies. Further research is needed to evaluate second cancer risks associated with chemotherapies in current treatment approaches and identify potential genetic markers of risk.

Keywords

Second primary cancer Retinoblastoma Sarcomas Radiotherapy 

Notes

Informed Consent and Conflict of Interest

Informed consent and animal studies disclosures are not applicable to this review.

Ruth Kleinerman, Lindsay Morton, Margaret Tucker, and Jeannette Wong declare that they have no conflict of interest.

This work was supported by the Intramural Research Program of the National Institutes of Health, National Cancer Institute.

This work was presented at the conference “1914–2014: Celebrating 100 years of Our Retinoblastoma Center in New York,” September 18–19, 2014.

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  1. 1.Radiation Epidemiology Branch, Division of Cancer Epidemiology and GeneticsNational Cancer Institute, National Institutes of HealthRockvilleUSA
  2. 2.Human Genetics Program, Division of Cancer Epidemiology and GeneticsNational Cancer Institute, National Institutes of HealthRockvilleUSA

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