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Radiation Therapy in the Management of Retinoblastoma

  • T. E. Merchant
Chapter
Part of the Pediatric Oncology book series (PEDIATRICO)

Abstract

Radiation therapy is a primary treatment for retinoblastoma resulting in high rates of disease control and functional organ preservation. Late effects from treatment, including secondary tumor formation in patients with genetic predisposition have, during the past 15 years, led investigators to pursue treatment approaches that delay or omit radiation therapy. Currently, the role of radiation therapy in the management of retinoblastoma remains in a state of uncertainty, and the number of patients irradiated using external beam methods is on the decline even as cure rates continue to increase (Broaddus et al. 2009). Investigators and caregivers await firm evidence that visual outcomes and eye preservation are equivalent with newer approaches that include episcleral plaque brachytherapy as a component of local therapy in the current front-line management for selected patients (Antoneli et al. 2006).

In the treatment of retinoblastoma, radiation therapy provides the benchmark for the evaluation of tumor control, for eye preservation, and for side effects. Its role has been diminished by its known contribution to secondary tumors in a high-risk population and the move toward chemotherapy combined with local ophthalmic therapy (Wilson et al. 2001). Radiation therapy is the most effective nonsurgical treatment for retinoblastoma. It is the only treatment for which long-term data identify attribution of late effects in vulnerable young patients and those with genetic susceptibility to malignancy induction. Radiation therapy has an excellent track record in preservation of the eye. In patients with Reese-Ellsworth group I-II disease, tumor control rates measured at 5 years are in excess of 95%. In patients with more advanced disease (Reese-Ellsworth groups III and IV), 5-year control rates reduce to approximately 50%, partly owing to the greater tumor burden and intraocular extent of disease (Blach et al. 1996). Patients with Reese-Ellsworth group VB disease have 5-year eye-preservation rates of approximately 53% (Abramson et al. 2004a). Poor tumor control in advanced cases is often attributed to vitreous seeding. Although data on visual acuity are relatively limited, most patients are reported to have good visual acuity (20/20-20/40) after radiation therapy; the rest have at least some prospect for functional vision (20/50-20/400) (Egbert et al. 1978; Hall et al. 1999). Visual acuity and field after therapy are affected by tumor location, tumor size, and treatment (Abramson et al. 2004b), which often depend on the patient’s age at the time of diagnosis; younger patients are more likely to have tumors about the macula (Brinkert et al. 1998).

Keywords

Cataract Formation Normal Tissue Complication Probability Vitreous Seeding Delay Radiation Therapy Intraocular Retinoblastoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Abramson DH, Frank CM (1998) Second nonocular tumors in survivors of bilateral retinoblastoma: a possible age effect on radiation-related risk. Ophthalmology 105:573-9PubMedCrossRefGoogle Scholar
  2. Abramson DH, Beaverson KL, Chang ST et al (2004a) Outcome following initial external beam radiotherapy in patients with Reese-Ellsworth group Vb retinoblastoma. Arch Ophthalmol 122:1316-23PubMedCrossRefGoogle Scholar
  3. Abramson DH, Melson MR, Servodidio C (2004b) Visual fields in retinoblastoma survivors. Arch Ophthalmol 122(9):1324-30PubMedCrossRefGoogle Scholar
  4. Antoneli CB, Ribeiro KC, Steinhorst F, Novaes PE, Chojniak MM, Malogolowkin M (2006) Treatment of retinoblastoma patients with chemoreduction plus local therapy: experience of the AC Camargo Hospital, Brazil. J Pediatr Hematol Oncol 28(6):342-5PubMedCrossRefGoogle Scholar
  5. Blach LE, McCormick B, Abramson DH (1996) External beam radiation therapy and retinoblastoma: long-term results in the comparison of two techniques. Int J Radiat Oncol Biol Phys 35:45-51PubMedCrossRefGoogle Scholar
  6. Brinkert AW, Moll AC, Jager MJ et al (1998) Distribution of tumors in the retina in hereditary retinoblastoma patients. Ophthalmic Genet 19:63-7PubMedCrossRefGoogle Scholar
  7. Broaddus E, Topham A, Singh AD (2009) Survival with retinoblastoma in the USA: 1975-2004. Br J Ophthalmol 93(1):24-7 Epub 2008 Aug 21PubMedCrossRefGoogle Scholar
  8. Egbert PR, Donaldson SS, Moazed K, Rosenthal AR (1978) Visual results and ocular complications following radiotherapy for retinoblastoma. Arch Ophthalmol 96:1826-30PubMedCrossRefGoogle Scholar
  9. Gass DM (1963) Retinoblastoma obscured by recent trauma. Arch Ophthalmol 69(5):583-584PubMedCrossRefGoogle Scholar
  10. Gombos DS, Hungerford J, Abramson DH, Kingston J, Chantada G, Dunkel IJ, Antoneli CB, Greenwald M, Haik BG, Leal CA, Medina-Sanson A, Schefler AC, Veerakul G, Wieland R, Bornfeld N, Wilson MW, Yu CB (2007) Secondary acute myelogenous leukemia in patients with retinoblastoma: is chemotherapy a factor? Ophthalmology 114(7):1378-83PubMedCrossRefGoogle Scholar
  11. Guyuron B (1990) The hourglass facial deformity. J Craniomaxillofac Surg 18(5):187-91PubMedCrossRefGoogle Scholar
  12. Hall LS, Ceisler E, Abramson DH (1999) Visual outcomes in children with bilateral retinoblastoma. J AAPOS 3:138-42PubMedCrossRefGoogle Scholar
  13. Higgins PD, Gerbi BJ, Macedon M, Dusenbery KE (2006) Fractionated stereotactic radiotherapy for pediatric patients with retinoblastoma. J Appl Clin Med Phys 7(2):9-17PubMedCrossRefGoogle Scholar
  14. Karcioglu ZA (2002) Fine needle aspiration biopsy (FNAB) for retinoblastoma. Retina 22(6):707-10PubMedCrossRefGoogle Scholar
  15. Kase S, Parikh JG, Youssef PN, Murphree AL, Rao NA (2008) Transforming growth factor beta in retinoblastoma-related cataract. Arch Ophthalmol 126(11):1539-42PubMedCrossRefGoogle Scholar
  16. Kleinerman RA, Tucker MA, Tarone RE, Abramson DH, Seddon JM, Stovall M, Li FP, Fraumeni JF Jr (2005) Risk of new cancers after radiotherapy in long-term survivors of retinoblastoma: an extended follow-up. J Clin Oncol 23(10):2272-9PubMedCrossRefGoogle Scholar
  17. Kleinerman RA, Tucker MA, Abramson DH, Seddon JM, Tarone RE, Fraumeni JF Jr (2007) Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma. J Natl Cancer Inst 99(1):24-31PubMedCrossRefGoogle Scholar
  18. Krasin MJ, Crawford BT, Zhu Y et al (2004) Intensity-modulated radiation therapy for children with intraocular retinoblastoma: potential sparing of the bony orbit. Clin Oncol (R Coll Radiol) 16:215-22CrossRefGoogle Scholar
  19. Krengli M, Hug EB, Adams JA et al (2005) Proton radiation therapy for retinoblastoma: comparison of various intraocular tumor locations and beam arrangements. Int J Radiat Oncol Biol Phys 61:583-93PubMedCrossRefGoogle Scholar
  20. Leal-Leal CA, Rivera-Luna R, Flores-Rojo M, Juárez-Echenique JC, Ordaz JC, Amador-Zarco J (2006) Survival in extra-orbital metastatic retinoblastoma:treatment results. Clin Transl Oncol 8(1):39-44PubMedCrossRefGoogle Scholar
  21. Lee CT, Bilton SD, Famiglietti RM, Riley BA, Mahajan A, Chang EL, Maor MH, Woo SY, Cox JD, Smith AR (2005) Treatment planning with protons for pediatric retinoblastoma, medulloblastoma, and pelvic sarcoma: how do protons compare with other conformal techniques? Int J Radiat Oncol Biol Phys 63(2):362-72PubMedCrossRefGoogle Scholar
  22. Mahajan S, Juneja M, George T (2008) Osteosarcoma as a second neoplasm after chemotherapeutic treatment of hereditary retinoblastoma: a case report. 1. Quintessence Int 39(5):439-452PubMedGoogle Scholar
  23. Marees T, Moll AC, Imhof SM, de Boer MR, Ringens PJ, van Leeuwen FE (2008) Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up. J Natl Cancer Inst 100(24):1771-9 Epub 2008 Dec 9PubMedCrossRefGoogle Scholar
  24. Merchant TE, Gould CJ, Wilson MW et al (2004) Episcleral plaque brachytherapy for retinoblastoma. Pediatr Blood Cancer 43:134-9PubMedCrossRefGoogle Scholar
  25. Miller DM, Murray TG, Cicciarelli NL, Capo H, Markoe AM (2005) Pars plana lensectomy and intraocular lens implantation in pediatric radiation-induced cataracts in retinoblastoma. Ophthalmology 112(9):1620-1624PubMedCrossRefGoogle Scholar
  26. Moll AC, Imhof SM, Schouten-Van Meeteren AY et al (2001) Second primary tumors in hereditary retinoblastoma: a register-based study, 1945-1997: is there an age effect on radiation-related risk? Ophthalmology 108:1109-14PubMedCrossRefGoogle Scholar
  27. Munier FL, Verwey J, Pica A, Balmer A, Zografos L, Abouzeid H, Timmerman B, Goitein G, Moeckli R (2008) New developments in external beam radiotherapy for retinoblastoma: from lens to normal tissue-sparing techniques. Clin Experiment Ophthalmol 36(1):78-89PubMedCrossRefGoogle Scholar
  28. Murthy R, Honavar SG, Vemuganti GK, Naik MN, Reddy VP (2007) Systemic metastasis following hyphema drainage in an unsuspected retinoblastoma. J Pediatr Ophthalmol Strabismus 44(2):120-3PubMedGoogle Scholar
  29. O’Doherty M, Lanigan B, Breathnach F, O’Meara A, Gallie B, Chan H, O’Keefe M (2005) A retrospective review of visual outcome and complications in the treatment of retinoblastoma. Ir Med J 98(1):17-20PubMedGoogle Scholar
  30. Pomarede R, Czernichow P, Zucker JM, Schlienger P, Haye C, Rosenwald JC, Labib A, Rappaport R (1984) Incidence of anterior pituitary deficiency after radiotherapy at an early age: study in retinoblastoma. Acta Paediatr Scand 73(1):115-9PubMedCrossRefGoogle Scholar
  31. Sahgal A, Millar BA, Michaels H, Jaywant S, Chan HS, Heon E, Gallie B, Laperriere N (2006) Focal stereotactic external beam radiotherapy as a vision-sparing method for the treatment of peripapillary and perimacular retinoblastoma: preliminary results. Clin Oncol (R Coll Radiol) 18(8):628-34CrossRefGoogle Scholar
  32. Shields CL, Honavar S, Shields JA, Demirci H, Meadows AT (2000) Vitrectomy in eyes with unsuspected retinoblastoma. Ophthalmology 107(12):2250-5PubMedCrossRefGoogle Scholar
  33. Shields CL, Shields JA, Cater J et al (2001) Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors. Ophthalmology 108:2116-21PubMedCrossRefGoogle Scholar
  34. Srithavaj T, Thaweboon S (2006) Determination of oral microflora in irradiated ocular deformed children. Southeast Asian J Trop Med Public Health 37(5):991-5PubMedGoogle Scholar
  35. Stannard C, Lipper S, Sealy R, Sevel D (1979) Retinoblastoma: correlation of invasion of the optic nerve and choroid with prognosis and metastasis. Br J Ophthalmol 63:560-570PubMedCrossRefGoogle Scholar
  36. Stevenson KE, Hungerford J, Garner A (1989) Local extraocular extension of retinoblastoma following intraocular surgery. Br J Ophthalmol 73(9):739-42PubMedCrossRefGoogle Scholar
  37. Tawansy KA, Samuel MA, Shammas M, Murphree AL (2006) Vitreoretinal complications of retinoblastoma treatment. Retina 26(7 Suppl):S47-52PubMedCrossRefGoogle Scholar
  38. Wilson MW, Rodriguez-Galindo C, Haik BG et al (2001) Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma. Ophthalmology 108:2106-14 discussion 2114-5PubMedCrossRefGoogle Scholar
  39. Wong FL, Boice JD Jr, Abramson DH et al (1997) Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk. JAMA 278:1262-7PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Division of Radiation OncologySt. Jude Children’s Research HospitalMemphisUSA

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