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Retinoblastoma Treatment & Management

  • Author: Marichelle Aventura Isidro, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Jul 12, 2016
 

Medical Care

Medical therapy should be directed toward complete control of the tumor and the preservation of as much useful vision as possible. Treatment is usually individualized to the specific patient.

External beam radiation therapy

Incidence of local control is high and retinal late effects are minimal with radiation doses of 4000-4500 cGy used with 200 cGy fractions. However, morbidity and mortality associated with external beam radiation therapy (EBRT) are significant. EBRT results in cessation of bone growth. Therefore, children with retinoblastoma who are treated with EBRT have significant midface hypoplasia. (The younger the child is when EBRT is instituted, the more dramatic the outcome.) More importantly, EBRT has been shown to increase the risk of developing second cancers almost 6-fold during the lifetime of these patients. Today, neoadjuvant chemotherapy (chemoreduction) has superseded EBRT in order to (hopefully) circumvent these terrible adverse effects of EBRT. Nevertheless, EBRT is still indicated in selected circumstances, as follows:

  • For eyes with significant vitreous seeding
  • For children who have progression of disease while undergoing chemoreduction
  • For tumors extending up to or beyond the cut margin of the optic nerve of an enucleated eye (The best method of treatment is being debated in such a case.)

Radioactive isotope plaques

Use of radioactive 60 Co (cobalt); radioactive 125 I (iodine), which is presently the most used; radioactive 192 Ir (iridium); or radioactive 106 Ru (ruthenium)

Radioactive 125 I plaque treatment is recommended for treatment of one larger tumor or a limited number of moderately sized tumors (< 3) present in noncritical areas.

Advantage - Locally directed treatment to the tumor, minimizing radiation to the normal tissue

Disadvantage - Incomplete treatment, high dose to local sclera, significantly less irradiation for anterior lesions, and difficulty placing posterior plaques

Chemotherapy

Primary neoadjuvant chemotherapy or chemoreduction has been the most significant recent advance in the treatment of retinoblastoma. This is typically the principle mode of treatment for eyes in intraocular groups C and D. However, our understanding of dose, duration, and end points are still evolving with this relatively new treatment modality.

Prophylactic chemotherapy is recommended if a tumor is in the optic nerve past the lamina cribrosa because these cases have a poor survival prognosis.

Use of neoadjuvant chemotherapy has the advantage of limiting the necessity for EBRT and reducing the possibility of EBRT-related complications.

Chemotherapy also may be used prior to EBRT, as completed by Kingston and associates in an attempt to improve local control and visual outcome in children with group V tumors, using carboplatin, etoposide, and vincristine, followed by 40-44 Gy of EBRT.[17]

Shields and associates used carboplatin, etoposide, and vincristine chemotherapy, followed by cryotherapy, photocoagulation, and 125 I plaque treatment in an attempt to improve outcome for eyes with more advanced retinoblastoma commonly treated with enucleation.[18]

Current studies completed by the Retinoblastoma Study Group show the promising use of chemotherapy (carboplatin, vincristine sulfate, and etoposide phosphate) as a primary mode of treatment in reducing tumor bulk, followed by various forms of local approaches (radiotherapy [external beam or plaque], cryotherapy, thermotherapy, and photocoagulation) that can be used for final tumor control.

Some reports suggest the addition of cyclosporine in combination with the chemotherapy regimen of carboplatin, etoposide, and vincristine. These reports showed that this addition enhances the efficacy of chemotherapy and eliminates the need for radiation.

Abramson and colleagues have demonstrated successful salvage of eyes typically enucleated for advanced disease.[19] Intra-arterial chemotherapy for advanced retinoblastoma offers another weapon in the arsenal of therapies for retinoblastoma. However, there are still potential complications to consider, and, consequently, this procedure should be performed at tertiary care institutions that specialize in the care of patients with retinoblastoma.

Novel therapy

A study by Yi Qu et al used immunohistochemical analysis of normal retina and retinoblastoma tumor specimens acquired from multiple centers in order to evaluate the pathological associations of the nuclear factor-κB (NF-κB) subunits and retinoblastoma. The data point to the fact that therapeutic strategies targeting NF-κB together with other therapies may represent a novel approach to retinoblastoma therapy.[20]

Suzuki et al conducted a study with selective arterial injection involving a balloon catheter and melphalan. It achieved a success rate of 98.8% and had few severe adverse events, including secondary neoplasms. The rate of preservation was more than half for treated eyes, and more than half of the eyes without macular tumors maintained a visual acuity of more than 0.5. No severe eye damage or severe systemic events were detected; secondary neoplasms were observed but no more frequently than otherwise expected.[21]

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Surgical Care

Surgical removal of the tumor has been the standard management of very unfavorable retinoblastoma cases.

Enucleation

Enucleation is performed when there is no chance of preserving useful vision in an eye.

Patients generally requiring enucleation are those who present with total retinal detachments and/or the posterior segment is full of the tumor, in which case it is clear the patient cannot retain any form of useful vision.

Significant orbital growth retardation remains after enucleation for retinoblastoma.[22] The younger the patient is at the time of surgery, the more growth retardation occurs.

Cryotherapy

Cryotherapy can be used primarily for small anteriorly located tumors, remote from the disc and macula but also may be indicated for recurrence after radiation therapy.

Cryotherapy is performed transsclerally. Under direct visualization, freezing is carried out until the ice ball incorporates the entire tumor. A refreeze-thaw cycle is repeated 3-4 times.

Complete disappearance of the tumor with a flat pigmented scar is the sign of successful treatment. This can be repeated if the tumor does not respond initially.

Photocoagulation

Photocoagulation can be used as primary therapy for small posteriorly located tumors.

There is a danger of producing large field defects near the disc and decreased vision resulting from macular pucker by photocoagulation near the macula.

The technique is performed by placing a double row of confluent burns around each tumor using a photocoagulator.

It is important not to do direct treatment on the tumor itself because the light color of the tumor generally precludes absorption of sufficient energy and there is a danger of exploding the tumor with spread of viable tumor debris into the vitreous and other parts of the retina.

Successful treatment with photocoagulation takes weeks to evolve and consists of complete disappearance of the tumor, which is replaced with a flat area.

Photocoagulation can also be used for tumor recurrences after EBRT.

Exenteration 

Exenteration is still performed, especially in most underdeveloped countries, when extension of the tumor into the surrounding areas is considerable.

Status post (S/P) enucleation for retinoblastoma, Status post (S/P) enucleation for retinoblastoma, right eye retinoblastoma, recurrence, right eye. History: IJ, 3-year-old male with chief complaint of right orbital mass. At age 2 months, opacity in right eye is noted. Five months prior to admission (PTA), consultation with an ophthalmologist for proptosis, right eye. Four months PTA, the patient underwent enucleation, right eye, with no alleged tumor involvement of the tumor resection margins on histopathology. One month PTA, gradually enlarging orbital mass, right side, was noted. Examination: Visual acuity right eye, not applicable (S/P enucleation); visual acuity left eye, at least 6/12 (20/40). No masses are seen in left eye on indirect ophthalmoscopy. Diagnostics: Skeletal survey showed lytic lesions on the humerus, femur, and pubic bones.
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Consultations

Patients with retinoblastoma should be evaluated and treated by a team of medical professionals, including an ophthalmologist (preferably an ocular oncologist), pediatrician, oncologist, radiologist, and pathologist. Given that this is a relatively uncommon disease, patients should try to seek attention from physicians with subspecialty training and experience in retinoblastoma, and who are actively participating in organizations that explore up-to-date treatments for retinoblastoma.

The pathologist plays a special role in the treatment of a patient with retinoblastoma. The surgical specimens should be evaluated with care to guide the clinicians with the appropriate postsurgical management.

Appropriate consultations are needed to provide much needed information to each other. In some instances, frozen sections are requested after enucleation or exenteration.

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Contributor Information and Disclosures
Author

Marichelle Aventura Isidro, MD Consulting Staff, Department of Ophthalmology, Santo Tomas University Hospital of Manila, Philippine Heart Center

Marichelle Aventura Isidro, MD is a member of the following medical societies: American Academy of Ophthalmology, International Society of Refractive Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Thomas M Aaberg, Jr, MD Clinical Assistant Professor, Department of Surgery, Michigan State University College of Human Medicine; Consulting Staff, Department of Ophthalmology, Retina Specialists of Michigan

Thomas M Aaberg, Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Retina Specialists, Retina Society, Michigan Society of Eye Physicians & Surgeons, American Academy of Ophthalmology, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: synergetics, True Vision, B&L<br/>Serve(d) as a speaker or a member of a speakers bureau for: Allergan.

Manolette R Roque, MD, MBA, FPAO Section Chief, Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief, Ocular Immunology and Uveitis, International Eye Institute, St Luke's Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic; Director, AMC Eye Center, Alabang Medical Center

Manolette R Roque, MD, MBA, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Philippine Medical Association, American Uveitis Society, International Ocular Inflammation Society, Philippine Ocular Inflammation Society, American Society of Ophthalmic Administrators, American Academy of Ophthalmic Executives, Philippine Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Barbara L Roque, MD, DPBO, FPAO Senior Partner, Roque Eye Clinic; Chief of Service, Pediatric Ophthalmology and Strabismus Section, Department of Ophthalmology, Asian Hospital and Medical Center; Active Consultant Staff, International Eye Institute, St Luke's Medical Center Global City

Barbara L Roque, MD, DPBO, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Cataract and Refractive Surgery, Philippine Society of Cataract and Refractive Surgery, Philippine Academy of Ophthalmology, Philippine Society of Pediatric Ophthalmolo

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steve Charles, MD Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Macula Society, Retina Society, Club Jules Gonin

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Ophthalmological Society

Disclosure: Nothing to disclose.

References
  1. Boggs W. Good cognitive, social functioning seen decades after retinoblastoma treatment. Medscape Medical News. November 26, 2014. [Full Text].

  2. Brinkman TM, Merchant TE, Li Z, Brennan R, Wilson M, Hoehn ME, et al. Cognitive function and social attainment in adult survivors of retinoblastoma: A report from the St. Jude Lifetime Cohort Study. Cancer. 2014 Nov 24. [Medline].

  3. Pawius P. Observatio XXIII. Tumor oculorum. Observationes Anatomicae Selectiores. Appended to: Bartholinus T. Historiarum Anatomicarum Rariorum, Centuria III & IV. Copenhagen, Denmark: Petrus Morsing; 1657. 38-39.

  4. Hey W. Practical Observations in Surgery. Philadelphia, Pa: James Humphreys; 1805.

  5. Wardrop J. Observations on the fungus haematodes. Edinburgh: Constable; 1809.

  6. Virchow R. Die Drankhaften Geschwulste. Berlin, Germany: Harshwald; 1864.

  7. Flexner S. A peculiar glioma (neuroepithelioma) of the retina. Bull Johns Hopkins Hosp. 1891. 2:

  8. Wintersteiner H. Die Neuroepithelioma Retinae: Eine Anatomiche and Klinische Studie. Leipzig, Germany: Dentisae; 1897.

  9. Verhoeff FH, Jackson E. Minutes of the proceedings of the 62nd annual meeting. Trans Am Ophthalmol Soc. 1926. 24:38-43.

  10. Tso MO, Zimmerman LE, Fine BS. The nature of retinoblastoma, I: photoreceptor differentiation: a clinical and histopathological study. Am J Ophthalmol. 1970. 69:339-49.

  11. Tso MO, Fine BS, Zimmerman LE. The nature of retinoblastoma, II: Photoreceptor differentiation: an electron microscope study. Am J Ophthalmol. 1970. 69:350-9.

  12. ScienceDaily. St. Jude Children's Research Hospital (2007, October 21). Specific Cell That Causes Eye Cancer Identified, Disproving Long-held Theory. sciencedaily.com. Available at http://www.sciencedaily.com­ /releases/. Accessed: Retrieved May 16, 2008.

  13. Castera L, Sabbagh A, Dehainault C, et al. MDM2 as a modifier gene in retinoblastoma. J Natl Cancer Inst. 2010 Dec 1. 102(23):1805-8. [Medline].

  14. Abdel Razek AA, Elkhamary S, Al-Mesfer S, Alkatan HM. Correlation of Apparent Diffusion Coefficient at 3T with Prognostic Parameters of Retinoblastoma. AJNR Am J Neuroradiol. 2012 May. 33(5):944-8. [Medline].

  15. Felberg NT, Donoso LA. Surface and cytoplasmic antigens in retinoblastoma. Invest Ophthalmol Vis Sci. 1980 Oct. 19(10):1242-5. [Medline].

  16. Moscinski LC, Pendergrass TW, Weiss A, Hvizdala E, Buckley KS, Kalina RE. Recommendations for the use of routine bone marrow aspiration and lumbar punctures in the follow-up of patients with retinoblastoma. J Pediatr Hematol Oncol. 1996 May. 18(2):130-4. [Medline].

  17. Kingston JE, Hungerford JL, Madreperla SA, et al. Results of combined chemotherapy and radiotherapy for advanced intraocular retinoblastoma. Arch Ophthalmol. 1996 Nov. 114(11):1339-43. [Medline].

  18. Shields CL, Shields JA, Needle M, et al. Combined chemoreduction and adjuvant treatment for intraocular retinoblastoma. Ophthalmology. 1997 Dec. 104(12):2101-11. [Medline].

  19. Abramson DH, Dunkel IJ, Brodie SE, Marr B, Gobin YP. Superselective ophthalmic artery chemotherapy as primary treatment for retinoblastoma (chemosurgery). Ophthalmology. 2010 Aug. 117(8):1623-9. [Medline].

  20. Qu Y, Zhou F, Dai X, et al. Clinicopathologic significances of nuclear expression of nuclear factor-?B transcription factors in retinoblastoma. J Clin Pathol. 2011 Aug. 64(8):695-700. [Medline].

  21. Suzuki S, Yamane T, Mohri M, Kaneko A. Selective ophthalmic arterial injection therapy for intraocular retinoblastoma: the long-term prognosis. Ophthalmology. 2011 Oct. 118(10):2081-7. [Medline].

  22. Lin HY, Liao SL. Orbital development in survivors of retinoblastoma treated by enucleation with hydroxyapatite implant. Br J Ophthalmol. 2011 May. 95(5):630-3. [Medline].

  23. 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 Sep. 122(9):1316-23. [Medline].

  24. Abramson DH. Second nonocular cancers in retinoblastoma: a unified hypothesis. The Franceschetti Lecture. Ophthalmic Genet. 1999 Sep. 20(3):193-204. [Medline].

  25. Abramson DH, Frank CM, Susman M, et al. Presenting signs of retinoblastoma. J Pediatr. 1998 Mar. 132(3 Pt 1):505-8. [Medline].

  26. Albert DM. Historic review of retinoblastoma. Ophthalmology. 1987 Jun. 94(6):654-62. [Medline].

  27. Apushkin MA, Apushkin MA, Shapiro MJ, et al. Retinoblastoma and simulating lesions: role of imaging. Neuroimaging Clin N Am. 2005 Feb. 15(1):49-67. [Medline].

  28. Badhu B, Sah SP, Thakur SK, et al. Clinical presentation of retinoblastoma in Eastern Nepal. Clin Experiment Ophthalmol. 2005 Aug. 33(4):386-9. [Medline].

  29. Beck MN, Balmer A, Dessing C, et al. First-line chemotherapy with local treatment can prevent external-beam irradiation and enucleation in low-stage intraocular retinoblastoma. J Clin Oncol. 2000 Aug. 18(15):2881-7. [Medline].

  30. Biswas J, Mani B, Shanmugam MP, et al. Retinoblastoma in adults. Report of three cases and review of the literature. Surv Ophthalmol. 2000 Mar-Apr. 44(5):409-14. [Medline].

  31. Bunin GR, Petrakova A, Meadows AT, et al. Occupations of parents of children with retinoblastoma: a report from the Children's Cancer Study Group. Cancer Res. 1990 Nov 15. 50(22):7129-33. [Medline].

  32. Chan HS, Gallie BL, Munier FL. Chemotherapy for retinoblastoma. Ophthalmol Clin North Am. 2005 Mar. 18(1):55-63, viii. [Medline].

  33. Chantada GL, de Davila MT, Fandino A, et al. Retinoblastoma with low risk for extraocular relapse. Ophthalmic Genet. 1999 Sep. 20(3):133-40. [Medline].

  34. Cowell JK. The genetics of retinoblastoma. Br J Cancer. 1991 Mar. 63(3):333-6. [Medline].

  35. de Graaf P, Barkhof F, Moll AC. Retinoblastoma: MR imaging parameters in detection of tumor extent. Radiology. 2005 Apr. 235(1):197-207. [Medline].

  36. DiCiommo D, Gallie BL, Bremner R. Retinoblastoma: the disease, gene and protein provide critical leads to understand cancer. Semin Cancer Biol. 2000 Aug. 10(4):255-69. [Medline].

  37. Dudgeon J. Retinoblastoma--trends in conservative management. Br J Ophthalmol. 1995 Feb. 79(2):104. [Medline].

  38. Dudgeon J. Unilateral retinoblastoma--genetic implications. Br J Ophthalmol. 1996 Mar. 80(3):193. [Medline].

  39. Ferris FL 3rd, Chew EY. A new era for the treatment of retinoblastoma. Arch Ophthalmol. 1996 Nov. 114(11):1412. [Medline].

  40. Finger PT, Czechonska G, Demirci H, Rausen A. Chemotherapy for retinoblastoma: a current topic. Drugs. 1999 Dec. 58(6):983-96. [Medline].

  41. Friedman DL, Himelstein B, Shields CL, et al. Chemoreduction and local ophthalmic therapy for intraocular retinoblastoma. J Clin Oncol. 2000 Jan. 18(1):12-7. [Medline].

  42. Gombos DS, Diba R. Estimating the incidence of retinoblastoma in Texas. Tex Med. 2005 Jul. 101(7):70-2. [Medline].

  43. Hamel P, Heon E, Gallie BL, et al. Focal therapy in the management of retinoblastoma: when to start and when to stop. J AAPOS. 2000 Dec. 4(6):334-7. [Medline].

  44. Harbour JW. Overview of RB gene mutations in patients with retinoblastoma. Implications for clinical genetic screening. Ophthalmology. 1998 Aug. 105(8):1442-7. [Medline].

  45. Harini R, Ata-ur-Rasheed M, Shanmugam MP. Genetic profile of 81 retinoblastoma patients from a referral hospital in southern India. Indian J Ophthalmol. 2001 Mar. 49(1):37-42. [Medline].

  46. Heimann H, Bechrakis NE, Zepeda LC. Exposure of orbital implants wrapped with polyester-urethane after enucleation for advanced retinoblastoma. Ophthal Plast Reconstr Surg. 2005 Mar. 21(2):123-8. [Medline].

  47. Honavar SG, Singh AD. Management of advanced retinoblastoma. Ophthalmol Clin North Am. 2005 Mar. 18(1):65-73, viii. [Medline].

  48. Hungerford J. Factors influencing metastasis in retinoblastoma. Br J Ophthalmol. 1993 Sep. 77(9):541. [Medline].

  49. Joseph B, Shanmugam MP, Srinivasan MK. Retinoblastoma: genetic testing versus conventional clinical screening in India. Mol Diagn. 2004. 8(4):237-43. [Medline].

  50. Journee-de Korver HG, Midena E, Singh AD. Infrared thermotherapy: from laboratory to clinic. Ophthalmol Clin North Am. 2005 Mar. 18(1):99-110, viii-ix. [Medline].

  51. Kaelin WG. Functions of the retinoblastoma protein. Bioessays. 1999 Nov. 21(11):950-8. [Medline].

  52. Kao LY, Yang ML. Spontaneous regression of retinoblastoma in a Taiwan series. J Pediatr Ophthalmol Strabismus. 2005 Jul-Aug. 42(4):228-32. [Medline].

  53. Khan AO, Al-Mesfer S. Lack of efficacy of dilated screening for retinoblastoma. J Pediatr Ophthalmol Strabismus. 2005 Jul-Aug. 42(4):205-10; quiz 233-4. [Medline].

  54. Kim NJ, Choung HK, Khwarg SI. Free orbital fat graft to prevent porous polyethylene orbital implant exposure in patients with retinoblastoma. Ophthal Plast Reconstr Surg. 2005 Jul. 21(4):253-8. [Medline].

  55. Kivela T. Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. J Clin Oncol. 1999 Jun. 17(6):1829-37. [Medline].

  56. Lee CT, Bilton SD, Famiglietti RM. 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. 2005 Oct 1. 63(2):362-72. [Medline].

  57. Linn Murphree A. Intraocular retinoblastoma: the case for a new group classification. Ophthalmol Clin North Am. 2005 Mar. 18(1):41-53, viii. [Medline].

  58. Lipinski MM, Jacks T. The retinoblastoma gene family in differentiation and development. Oncogene. 1999 Dec 20. 18(55):7873-82. [Medline].

  59. Lohmann DR. RB1 gene mutations in retinoblastoma. Hum Mutat. 1999. 14(4):283-8. [Medline].

  60. MacDonald DJ, Lessick M. Hereditary cancers in children and ethical and psychosocial implications. J Pediatr Nurs. 2000 Aug. 15(4):217-25. [Medline].

  61. Margo C, Hidayat A, Kopelman J, et al. Retinocytoma. A benign variant of retinoblastoma. Arch Ophthalmol. 1983 Oct. 101(10):1519-31. [Medline].

  62. Mashayekhi A, Shields CL, Eagle RC. Cavitary changes in retinoblastoma: relationship to chemoresistance. Ophthalmology. 2005 Jun. 112(6):1145-50. [Medline].

  63. Matsubara H, Makimoto A, Higa T. A multidisciplinary treatment strategy that includes high-dose chemotherapy for metastatic retinoblastoma without CNS involvement. Bone Marrow Transplant. 2005 Apr. 35(8):763-6. [Medline].

  64. Mietz H, Hutton WL, Font RL. Unilateral retinoblastoma in an adult: report of a case and review of the literature. Ophthalmology. 1997 Jan. 104(1):43-7. [Medline].

  65. Miller DM, Murray TG, Cicciarelli NL. Pars plana lensectomy and intraocular lens implantation in pediatric radiation-induced cataracts in retinoblastoma. Ophthalmology. 2005 Sep. 112(9):1620-4. [Medline].

  66. Mittnacht S. The retinoblastoma protein--from bench to bedside. Eur J Cell Biol. 2005 Mar. 84(2-3):97-107. [Medline].

  67. Moll AC, Imhof SM, Meeteren AY, et al. At what age could screening for familial retinoblastoma be stopped? A register based study 1945-98. Br J Ophthalmol. 2000 Oct. 84(10):1170-2. [Medline].

  68. Nork TM, Millecchia LL, de Venecia GB, et al. Immunocytochemical features of retinoblastoma in an adult. Arch Ophthalmol. 1996 Nov. 114(11):1402-6. [Medline].

  69. O'Doherty M, Lanigan B, Breathnach F, et al. A retrospective review of visual outcome and complications in the treatment of retinoblastoma. Ir Med J. 2005 Jan. 98(1):17-20. [Medline].

  70. Pendergrass TW, Davis S. Incidence of retinoblastoma in the United States. Arch Ophthalmol. 1980 Jul. 98(7):1204-10. [Medline].

  71. Pochanugool L, Hathirat P, Kunavisarut S, et al. Radiation treatment of retinoblastoma: experience in the past two decades. J Med Assoc Thai. 1994 Jun. 77(6):308-13. [Medline].

  72. Schuler A, Weber S, Neuhauser M. Age at diagnosis of isolated unilateral retinoblastoma does not distinguish patients with and without a constitutional RB1 gene mutation but is influenced by a parent-of-origin effect. Eur J Cancer. 2005 Mar. 41(5):735-40. [Medline].

  73. Schvartzman E, Chantada G, Fandino A, et al. Results of a stage-based protocol for the treatment of retinoblastoma. J Clin Oncol. 1996 May. 14(5):1532-6. [Medline].

  74. Shanmugam MP, Biswas J, Gopal L. The clinical spectrum and treatment outcome of retinoblastoma in Indian children. J Pediatr Ophthalmol Strabismus. 2005 Mar-Apr. 42(2):75-81; quiz 112-3. [Medline].

  75. Shields CL, De Potter P, Himelstein BP, et al. Chemoreduction in the initial management of intraocular retinoblastoma. Arch Ophthalmol. 1996 Nov. 114(11):1330-8. [Medline].

  76. Shields CL, Mashayekhi A, Cater J. Chemoreduction for retinoblastoma: analysis of tumor control and risks for recurrence in 457 tumors. Trans Am Ophthalmol Soc. 2004. 102:35-44; discussion 44-5. [Medline].

  77. Shields CL, Mashayekhi A, Cater J. Macular retinoblastoma managed with chemoreduction: analysis of tumor control with or without adjuvant thermotherapy in 68 tumors. Arch Ophthalmol. 2005 Jun. 123(6):765-73. [Medline].

  78. Shields CL, Materin MA, Shields JA. Review of optical coherence tomography for intraocular tumors. Curr Opin Ophthalmol. 2005 Jun. 16(3):141-54. [Medline].

  79. Shields CL, Shields JA, Meadows AT. Chemoreduction for retinoblastoma may prevent trilateral retinoblastoma. J Clin Oncol. 2000 Jan. 18(1):236-7. [Medline].

  80. Shields JA. Importance of early diagnosis of retinoblastoma. Br J Ophthalmol. 1999 Dec. 83(12):1315-6. [Medline].

  81. Singh AD, Shields CL, Shields JA. New insights into trilateral retinoblastoma. Cancer. 1999 Jul 1. 86(1):3-5. [Medline].

  82. Singh AD, Shields CL, Shields JA. Prognostic factors in retinoblastoma. J Pediatr Ophthalmol Strabismus. 2000 May-Jun. 37(3):134-41; quiz 168-9. [Medline].

  83. Stiller CA. Retinoblastoma and low level radiation. BMJ. 1993 Aug 21. 307(6902):461-2. [Medline].

  84. Tong CT, Howard SA, Shah HR. Effects of celecoxib in human retinoblastoma cell lines and in a transgenic murine model of retinoblastoma. Br J Ophthalmol. 2005 Sep. 89(9):1217-20. [Medline].

  85. Wilson MW, Haik BG, Liu T. Effect on ocular survival of adding early intensive focal treatments to a two-drug chemotherapy regimen in patients with retinoblastoma. Am J Ophthalmol. 2005 Sep. 140(3):397-406. [Medline].

  86. Wycliffe ND, Mafee MF. Magnetic resonance imaging in ocular pathology. Top Magn Reson Imaging. 1999 Dec. 10(6):384-400. [Medline].

  87. Retinoblastoma. Cancer.org. Available at http://www.cancer.org/cancer/retinoblastoma/detailedguide/retinoblastoma-staging. 12 March 2015; Accessed: 22 June 2016.

  88. H Jenkinson. AAPOS 2011 San Diego Workshop: Retinoblastoma- 2011 and beyond. AAPOS.org. Available at http://www.aapos.org/client_data/files/2011/311_parulekar_handouts_all.pdf. 2011; Accessed: 22 June 2016.

 
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Classic histologic finding of retinoblastoma (Flexner-Wintersteiner rosettes)
Retinoblastoma, intraocular stage (leukocoria). History: NB, 1-year-old male from Quezon Province, Philippines, with chief complaint of opacity, left eye. Born full-term spontaneous vaginal delivery (FTSVD) to a 27-year-old gravida 3, para 2 (2002) at home. Four months prior to admission (PTA), opacity was noted in the left eye (no consultation/medications). Five days PTA, consultation with an ophthalmologist. Examination: (+) leukocoria with visual acuity of central, steady, and maintained fixation on right eye, (-) dazzle on left eye; (+) Marcus Gunn (MG) reflex. Diagnostics: Ocular ultrasound was performed, revealing intraocular retinoblastoma. Management: Patient underwent enucleation of left eye. Examination under anesthesia of right eye: E/N retina. Histopathology: Retinoblastoma, intraocular stage left eye.
Retinoblastoma, glaucomatous stage. History: AB, 2-year-old female from Marikina City, Philippines, with chief complaint of proptosis, right eye. The patient is an adopted child. Prior to admission (PTA), with child aged 6 months (time of adoption), surrogate mother noted an opacity in the right eye. No medical consultation. One year PTA, physician consultation; told AB had an "eye mass" and needed to see an ophthalmologist. No compliance. One month PTA, proptosis was noted in the right eye. Examination: Visual acuity (VA) of right eye is no light perception; VA of left eye is central, steady, and maintained fixation. Sensorium: Awake but irritable. Diagnostics: Intracranial extension on CT scan. Skeletal survey: E/N. Management: The patient underwent exenteration (right side).
Patient with retinoblastoma, glaucomatous stage. Intracranial extension on CT scan.
Patient with retinoblastoma, glaucomatous stage. Another CT scan slice, showing the intracranial extension of the tumor.
Retinoblastoma, extraocular stage (neglected with necrosis). History: RC, 2-year-old male with chief complaint of left orbital mass. Born full-term spontaneous vaginal delivery (FTSVD) to a gravida 3, para 2 (2001) at home. Three months prior to admission (PTA), an inward deviation of the left eye was noted. No consultation. Six months PTA, opacity in the left eye was noted. Five months PTA, proptosis of the left eye with pain and bleeding was noted. Family/Social History: Indigent family. Youngest of 3 siblings; eldest sibling had no retinoblastoma; second sibling had retinoblastoma and underwent enucleation, dying after 2 sessions of chemotherapy. A cousin passed away with retinoblastoma. Examination: Indirect ophthalmoscopy of right eye revealed a large intraocular mass occupying the inferior half of the retina. Mass on left side. Management: The patient was scheduled for exenteration, left side. The mother and child went home against medical advice; what happened to the patient is not known.
Status post (S/P) enucleation for retinoblastoma, right eye retinoblastoma, recurrence, right eye. History: IJ, 3-year-old male with chief complaint of right orbital mass. At age 2 months, opacity in right eye is noted. Five months prior to admission (PTA), consultation with an ophthalmologist for proptosis, right eye. Four months PTA, the patient underwent enucleation, right eye, with no alleged tumor involvement of the tumor resection margins on histopathology. One month PTA, gradually enlarging orbital mass, right side, was noted. Examination: Visual acuity right eye, not applicable (S/P enucleation); visual acuity left eye, at least 6/12 (20/40). No masses are seen in left eye on indirect ophthalmoscopy. Diagnostics: Skeletal survey showed lytic lesions on the humerus, femur, and pubic bones.
Retinoblastoma, intraocular stage (CT scan findings). History: 5-month-old female with chief complaint of "cat's eye reflex." Two months prior to admission (PTA), cat's eye reflex noted with outward deviation of left eye. The patient's 29-year-old mother had bilateral retinoblastoma and underwent enucleation, left eye, at age 2 years. Examination: Regressed type stage III, left eye visual acuity (+) dazzle right eye; indirect ophthalmoscopy (+) mass nasal retina with seeding, multiple tumors in peripheral retina, left eye. E/N Retina: Right eye. Management: The patient underwent enucleation, left eye. Examination under anesthesia of right eye: E/N. Histopathology: Retinoblastoma, intraocular stage, well-differentiated left eye.
Flexner-Wintersteiner rosettes in retinoblastoma
Presenting signs or symptoms in retinoblastoma. (This table is modified from Abramson DH, Frank CM, Susman M, et al. Presenting signs of retinoblastoma. J Pediatr 1998 Mar; 132(3 Pt 1): 505-8.)
Reese-Ellsworth classification of retinoblastoma
Classic regression patterns of retinoblastoma
Genetic counseling for retinoblastoma. (This table is modified from Vogel F. Genetics of retinoblastoma. Hum Genet 1979; 52:1.)
Vitreous seeding (intraocular retinoblastoma). Courtesy of Manolette Roque, MD, Ophthalmic Consultants Philippines Co, EYE REPUBLIC Ophthalmology Clinic.
Reese-Ellsworth Stage V: vitreous seeding. Courtesy of Manolette Roque, MD, Ophthalmic Consultants Philippines Co, EYE REPUBLIC Ophthalmology Clinic.
 
 
 
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