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Non-ocular Tumors

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Clinical Ophthalmic Oncology

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Abstract

In the management of retinoblastoma, it is important to remember that there is an entire patient to treat – not just an intraocular tumor. Children with heritable retinoblastoma who have a germ-line mutation in RB1 have an increased risk of developing a second malignancy. All familial cases, all bilateral cases, and approximately 15 % of unilateral cases fall into this category. The most common secondary malignancies are sarcomas, melanoma, and brain tumors. The association of retinoblastoma and an intracranial neuroblastic malignancy, known as “trilateral retinoblastoma,” most often involves the pineal gland (“pineoblastoma/pinealoblastoma”) but may also involve parasellar and suprasellar regions (Chap. 20) [1–3]. The increased risk of developing second, non-ocular malignancies for survivors of heritable retinoblastoma is estimated to be 20 times higher than the general population [4].

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References

  1. Jakobiec FA, Tso MO, Zimmerman LE, et al. Retinoblastoma and intracranial malignancy. Cancer. 1977;39(5):2048–58.

    Article  PubMed  CAS  Google Scholar 

  2. Bader JL, Miller RW, Meadows AT, et al. Trilateral retinoblastoma. Lancet. 1980;2(8194):582–3.

    Article  PubMed  CAS  Google Scholar 

  3. Kivela T. Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. J Clin Oncol. 1999;17(6):1829–37.

    PubMed  CAS  Google Scholar 

  4. Marees T, Moll AC, Imhoff SM, et al. Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up. J Natl Cancer Inst. 2008;100(24):1771–9.

    Article  PubMed  Google Scholar 

  5. Abramson DH, Frank CM. Second nonocular tumors in survivors of bilateral retinoblastoma. A possible age effect on radiation-related risk. Ophthalmology. 1998;105:573–80.

    Article  PubMed  CAS  Google Scholar 

  6. Moll AC, Imhof SM, Schouten-Van Meeteren AY, Kuik DJ, Hofman P, Boers M. Second primary tumors in hereditary retinoblastoma: a register-based study, 1945–1997: is there an age effect on radiation-related risk? Ophthalmology. 2001;108(6):1109–14.

    Article  PubMed  CAS  Google Scholar 

  7. Fletcher O, Easton D, Anderson K, Gilham C, Jay M, Peto J. Lifetime risks of common cancers among retinoblastoma survivors. J Natl Cancer Inst. 2004;96:357–63.

    Article  PubMed  Google Scholar 

  8. Wong FL, Boice JD, Abramson DH, Tarone RE, Kleinerman RA, Stovall M, et al. Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk. JAMA. 1997;278:1262–7.

    Article  PubMed  CAS  Google Scholar 

  9. Eng G, Li FP, Abramson DH, Ellsworth RM, Wong FL, Goldman MB, Seddon J, Tarbell N, Boice JD. Mortality from second tumors among long-term survivors of retinoblastoma. J Natl Cancer Inst. 1993;85:1121–8.

    Article  PubMed  CAS  Google Scholar 

  10. Abramson DH. Second nonocular cancers in retinoblastoma: a unified hypothesis. The Franceschetti lecture. Ophthalmic Genet. 1999;99:193–204.

    Article  Google Scholar 

  11. Kleinerman RA, Tucker MA, Tarone RE, Abramson DH, Seddon JM, Stovall M, Li FP, Fraumeni JK jr. Risk of new cancers after radiotherapy in long-term survivors of retinoblastoma: an extended study. J Clin Onc 2005:23(10);2722–9.

    Google Scholar 

  12. Abramson DH. Retinoblastoma in the 20th century: past success and future challenges. The Weisenfeld lecture. Invest Ophthalmol Vis Sci. 2005;46:2684–91.

    Article  Google Scholar 

  13. Chauveinc L, Mosseri V, Quintana E, Desjardins L, Schlienger P, Doz F, Dutrillaux B. Osteosarcoma following retinoblastoma: age at onset and latency period. Ophthalmic Genet. 2001;22:77–88.

    Article  PubMed  CAS  Google Scholar 

  14. Mohney BG, Robertson DM, Schomberg PJ, Hodge DO. Second nonocular tumors in survivors of heritable retinoblastoma and prior radiation therapy. Am J Ophthalmol. 1998;126:269–77.

    Article  PubMed  CAS  Google Scholar 

  15. Sethi RV, Shih HA, Yeap BY, et al. Second nonocular tumors among survivors of retinoblastoma treated with contemporary photon and proton radiotherapy. Cancer. 2014;120:126–33.

    Article  PubMed  Google Scholar 

  16. Pedersen-Bjergaard J, Andersen MT, Andersen MK. Genetic pathways in the pathogenesis of therapy-related myelodysplasia and acute myeloid leukemia. Hematology Am Soc Hematol Educ Program. 2007;392–397.

    Google Scholar 

  17. Gombos DS, Hungerford J, Ambrason DH, Kingston J, Chantada G, Dunkel IJ, Antoneli CB, Greenwald M, Haik BG, Leal CA, Medina-Sanson A, Schefler AC, Verrakui G, Wieland R, Bornfeld N, Wilson MW, Yu CB. Secondary acute myelogenous leukemia in patients with retinoblastoma: is chemotherapy a factor? Ophthalmology. 2007;114:1378–83.

    Article  PubMed  Google Scholar 

  18. Qaddoumi I, Billups CA, Tagen M, et al. Topotecan and vincristine combination is effective against bilateral intraocular retinoblastoma and has manageable toxicity. Cancer. 2012;118(22):5663–70.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  19. Dunkel IJ, Gerald WL, Rosenfield NS, Strong EW, Abramson DH, Ghavimi F. Outcome of patients with a history of bilateral retinoblastoma treated for a second malignancy: the memorial Sloan-Kettering experience. Med Ped Onc. 1998;30:59–62.

    Article  CAS  Google Scholar 

  20. Fontanesi J, Parham DM, Pratt C, Meyer D. Second malignant neoplasms in children with retinoblastoma: the St. Jude Children’s Research Hospital experience. Ophthalmic Genet. 1996;16:105–8.

    Article  Google Scholar 

  21. Kleinerman RA, Yu CL, Little MP, Abramson DH, Seddon J, Tucker MA. Variation of second cancer risk by family history of retinoblastoma among long-term survivors. J Clin Oncol. 2012;30:950–7.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Shinohara ET, DeWees T, Perkins SM. Subsequent malignancies and their effect on survival in patients with retinoblastoma. Pediatri Blood Cancer. 2014;61:116–9.

    Article  Google Scholar 

  23. Abramson DH, Melson MR, Dunkel IJ, Frank CM. Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma. Ophthalmology. 2001;108(10):1868–76.

    Article  PubMed  CAS  Google Scholar 

  24. Kleinerman RA, Tarone RE, Abramson DH, Seddon JM, Li FP, Tucker MA. Hereditary retinoblastoma and risk of lung cancer. J Natl Cancer Inst. 2000;92:2037–9.

    Article  PubMed  CAS  Google Scholar 

  25. Harbour JW, Lai SL, Whang-Peng J, Gazdar AF, Minna JD, Kaye FJ. Abnormalities in structure and expression of the human retinoblastoma gene in SCLC. Science. 1988;241:353–7.

    Article  PubMed  CAS  Google Scholar 

  26. Li FP, Abramson DH, Tarone RE, Kleinerman RA, Fraumeni Jr JF, Boice JD. Hereditary retinoblastoma, lipoma, and second primary cancers. J Natl Cancer Inst. 1997;89:83–4, 109.

    Article  PubMed  CAS  Google Scholar 

  27. Aerts I, Pacquement H, Doz F, Mosseri V, Deschardins L, Sastre X, Michon J, Rodriguez J, Schlienger P, Zucker JM, Quintana E. Outcome of second malignancies after retinoblastoma: a retrospective analysis of 25 patients treated at the Institut Curie. Eur J Cancer. 2004;40:1522–9.

    Article  PubMed  Google Scholar 

  28. Shields CL, Honavar SG, Meadows AT, Shields JA, Demirci H, Singh A, Friedman DL, Naduvilath TJ. Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation. Am J Ophthalmo. 2002;133:657–64.

    Article  Google Scholar 

  29. Abramson DH, Beaverson KL, Chang ST, Dunkel IJ, McCormick B. Outcome following initial external beam radiotherapy in patients with Reese-Ellsworth group Vb retinoblastoma. Arch Ophthalmol. 2004;122(9):1316–23.

    Article  PubMed  Google Scholar 

  30. Yue NC, Benson ML. The hourglass deformity as a consequence of orbital irradiation for bilateral retinoblastoma. Pediatr Radiol. 1996;26:421–3.

    Article  PubMed  CAS  Google Scholar 

  31. Merchant TE, Gould CJ, Hilton NE, Kun LE, Rodriguez-Galindo C, Pratt CB, Wilson MW, Haik BG. Ocular preservation after 36 Gy external beam radiation therapy for retinoblastoma. J Pediatr Hematol Oncol. 2002;24:246–9.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Ophthalmology, University of Tennessee Health Sciences Center, 930 Madsion, 4th Floor, Memphis, TN, 38163, USA

    Brian C. Tse MD & Matthew W. Wilson MD, FACS

  2. Division of Solid Tumors, Department Oncology, St Jude Children’s Research Hospital, 332 N Lauderdale, Memphis, TN, 38105, USA

    Rachel C. Brennan MD

  3. Department of Pediatrics, Dana-Faber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA, 02215, USA

    Carlos Rodriguez-Galindo MD & Matthew W. Wilson MD, FACS

Authors
  1. Brian C. Tse MD
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  2. Rachel C. Brennan MD
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  3. Carlos Rodriguez-Galindo MD
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  4. Matthew W. Wilson MD, FACS
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Corresponding author

Correspondence to Matthew W. Wilson MD, FACS .

Editor information

Editors and Affiliations

  1. Dept. of Ophthalmic Oncology, Cole Eye Institute Cleveland Clinic Foundation, Cleveland, Ohio, USA

    Arun D. Singh

  2. Department of Ophthalmology, Children's Hospital Los Angeles, Los Angeles, USA

    A. Linn Murphree

  3. University of California, San Francisco, California, USA

    Bertil E. Damato

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Tse, B.C., Brennan, R.C., Rodriguez-Galindo, C., Wilson, M.W. (2015). Non-ocular Tumors. In: Singh, A., Murphree, A., Damato, B. (eds) Clinical Ophthalmic Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43451-2_19

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  • DOI: https://doi.org/10.1007/978-3-662-43451-2_19

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