Abstract
Retinoblastoma is a malignant pediatric eye tumor that is highly curable if the disease remains intraocular; its cure rate and sight preservation rate exceeds 90% in developed countries in part because of early diagnosis and access to adequate treatment. Retinoblastoma accounts for approximately 3% of all pediatric malignancies, and approximately two-thirds of patients present with unilateral disease, while one-third have bilateral involvement. Patients with unilateral disease are usually treated with enucleation, while few also receive adjuvant chemotherapy or radiation therapy. Management of bilateral patients is much more challenging since eye salvage and vision preservation must also be considered. The management of these cases includes chemotherapy and careful, intensive use of focal treatments. Such a regimen therefore requires the cooperation of a highly specialized, multidisciplinary team.
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Selected References
Abramson DH, Beaverson K, Sangani P, Vora R, Lee T, Hochberg H, Kirszrot J, Ranjithan M (2003) Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics 112(6):1248–1255
Balaguer J, Wilson MW, Billups CA, Mancini J, Haik BG, Qaddoumi I, Khoury JD, Rodriguez-Galindo C (2009) Predictive factors of invasion in eyes with retinoblastoma enucleated after eye salvage treatments. Pediatr Blood Cancer 52(3):351–356
Carlos LL, Roberto RL, Victor TG, Carlos HG, Eduardo LP (2002) Risk of dying of retinoblastoma in Mexican children. Med Pediatr Oncol 38(3):211–213
Chantada G, Fandino A, Manzitti J, Urrutia E, Schvartzman E (1999) Late diagnosis of retinoblastoma in a developing country. Arch Dis Child 80(2):171–174
Connolly D (2007) Hamilton institute quickly gaining global reputation. Commercial appeal. http://www.eye.uthsc.edu/news/CommercialApp-Jan07.pdf. Accessed 4 Jan 2007
Cyber-Sight website (2007) Retinoblastoma: one world, one vision. http://cybersight.org/bins/content_page.asp?cid=70–2151. Accessed Jan 2007
Dome Jeffrey S, Rodriguez-Galindo Carlos, Spunt Sheri L, Santana Victor M (2008) Pediatric solid tumors. In: Abeloff Martin D (ed) Abeloff’s clinical oncology, 4th edn. Elsevier, Philadelphia, pp 2075–2129
Dyer MA (2009) In their own words: interviews with cell cycle. The first knockout mouse model of retinoblastoma. Cell Cycle 8(14):2145, Epub 2009 Jul 15. PMID: 19574732
Ellsworth R (1969) The practical management of retinoblastoma. Trans Am Ophthalmol Soc 67:462–534
Finger PT; 7th Edition, AJCC-UICC Ophthalmic Oncology Task Force (committee of 45 members including Haik BG and Wilson MW) (2009) The 7th edition AJCC staging system for eye cancer: an international language for ophthalmic oncology. Arch Pathol Lab Med 133(8):1197–1198. PMID: 19653708
Friend SH, Bernards R, Rogelj S, Weinberg RA, Rapaport JM, Albert DM, Dryja TP (1986) A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma. Nature 323(6089):643–646
Haynie HW (2008) Hamilton eye institute expands reach of telemedicine. West Tennessee Medical News. http://www.westtnmedicalnews.com/news.php?viewStory=1397. Accessed Jul 2008
Helveston EM, Thazhathu MJ, Smallwood LM (2006) ORBIS telemedicine, cyber-sight. In: Yogesan K, Kumar S, Goldschmidt L, Cuadros J (eds) Tele-ophthalmology. Springer, Berlin/Heidelberg, pp 187–193
Knudson AG Jr (1971) Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci U S A 68(4):820–823
Lawrence SD, Hoehn ME, Karcioglu ZA, Haik BG (2009) An unusual case of retinoblastoma. Ophthalmic Surg Lasers Imaging 40(3):296–299, PubMed PMID: 19485296
Leander C, Fu LC, Peña A, Howard SC, Rodriguez-Galindo C, Wilimas JA, Ribeiro RC, Haik B (2007) Impact of an education program on late diagnosis of retinoblastoma in Honduras. Pediatr Blood Cancer 49(6):817–819
Rodrigues KE, Latorre MR, de Camargo B (2004) Delayed diagnosis in retinoblastoma. J Pediatr 80(6):511–516
Rodriguez-Galindo C, Wilson MW (eds) (2010) Retinoblastoma. Springer, New York
Rodriguez-Galindo C, Wilson MW, Chantada G, Fu L, Qaddoumi I, Antoneli C, Leal-Leal C, Sharma T, Barnoya M, Epelman S, Pizzarello L, Kane JR, Barfield R, Merchant TE, Robison LL, Murphree AL, Chevez-Barrios P, Dyer MA, O’Brien J, Ribeiro RC, Hungerford J, Helveston EM, Haik BG, Wilimas J (2008) Retinoblastoma: one world, one vision. Pediatrics 122(3):e763–e770, Review. PMID: 18762512
Sastre X, Chantada GL, Doz F, Wilson MW, de Davila MT, Rodríguez-Galindo C, Chintagumpala M, Chévez-Barrios P (2009) International Retinoblastoma Staging Working Group. Proceedings of the consensus meetings from the International Retinoblastoma Staging Working Group on the pathology guidelines for the examination of enucleated eyes and evaluation of prognostic risk factors in retinoblastoma. Arch Pathol Lab Med 133(8):1199–1202, PMID: 19653709
Shepard S (2006) Discovery by UT-St. Jude scientific team seen as breakthrough in cancer research. Memphis Bus J http://www.bizjournals.com/memphis/stories/2006/11/13/story8.html?page=all
Shih CS, Laurie N, Holzmacher J, Spence Y, Nathwani AC, Davidoff AM, Dyer MA (2009) AAV-mediated local delivery of interferon-beta for the treatment of retinoblastoma in preclinical models. Neuromolecular Med 11(1):43–52, Epub 2009 Mar 22. PMID: 19306089
St. Jude Children’s Research Hospital (2009) Outreach through Oncopedia. Promise http://www.stjude.org/stjude/v/index.jsp?vgnextoid=55e6cde1a67df110VgnVCM1000001e0215acRCRD. Accessed Winter 2009
Strehlau C (2006) Retinoblastoma symposium with Drs. Barrett Haik, Judith Wilimas, and Eugene Helveston. St. Jude Medical Minute. http://www.eye.uthsc.edu/audio/stjude-medmin-panama.mp3. Accessed 11 Jan 2006
Sutherland J (2006) Mission possible: UTMG ophthalmologists make a difference in Panama. Link UT Medical Group http://www.bizjournals.com/memphis/stories/2006/11/13/story8.html?page=all
UT Health Science Center (2007) University of Tennessee Health Science Center Hamilton Eye Institute uses telemedicine to reach out to Panamanian patients. http://www.uthsc.edu/news/ne.php?HYPER=/news/november/hei_panama_teleconference_release.htm. Accessed 28 Nov 2007
UT Health Science Center (2008) Vietnam Telehealth Conference, Hamilton Eye Institute. The Record. http://www.uthsc.edu/record/pdfs/2008/record_may-jun08.pdf. Accessed May-June 2008
WHBQ-TV Memphis (Fox affiliate) (2006) International symposium aboard ORBIS® flying eye hospital. Fox 13 Eyewitness News. http://www.eye.uthsc.edu/video/Orbis-Fox13news-2006jan13.mpg. Accessed 13 Jan 2006
WHBQ-TV Memphis (Fox affiliate) (2006) Health matters: targeted retinoblastoma therapy. Fox 13 Eyewitness News. http://www.eye.uthsc.edu/video/RB-Ch13FoxNews-Dec5–2006.wmv. Accessed 5 Dec 2006
Wilimas JA, Wilson MW, Haik BG, Barnoya M, Fu L, Castellanos M, Bonilla M, Phillips BX, Helveston EM, Luna-Fineman S, Ribiero R, Rodriguez-Galindo C (2009) Development of retinoblastoma programs in Central America. Pediatr Blood Cancer 53(1):42–46
Wilson MW, Rodriguez-Galindo C, Billups C, Haik BG, Laningham F, Patay Z (2009a) Lack of correlation between the histologic and magnetic resonance imaging results of optic nerve involvement in eyes primarily enucleated for retinoblastoma. Ophthalmology 116(8):1558–1563, Epub 2009 Jul 9. PubMed PMID: 19545904
Wilson MW, Fraga CH, Rodriguez-Galindo C, Hagedorn N, Leggas ML, Stewart C (2009b) Expression of the multi-drug resistance proteins and the pregnane X receptor in treated and untreated retinoblastoma. Curr Eye Res 34(5):386–394
Acknowledgments
United States
Eugene Helveston, M.D.
Ophthalmologist-in-Chief
ORBIS International
Al L. Ueltschi
Chairman
ORBIS International & FlightSafety International, Inc.
Lynda Smallwood
Senior Manager, Cyber-Sight
ORBIS International
Matthew W. Wilson, M.D., FACS
Professor of Ophthalmology
UT Hamilton Eye Institute
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
James C. Fleming, M.D., FACS
Philip M. Lewis Professor of Ophthalmology
Director of the Orbit Center
Hamilton Eye Institute
UT Health Science Center
Memphis, TN
Raul C. Ribeiro, M.D.
Director, Leukemia/Lymphoma
Director, International Outreach
St. Jude Children’s Research Hospital, Memphis, TN
Judith A. Wilimas, M.D.
Medical Director
Referring Physicians Office and Domestic Affiliate Program
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
Carlos Rodriguez-Galindo, M.D.
Pediatric Oncologist
Dana-Farber Cancer Institute/
Children’s Hospital Boston
Boston, MA
Ibrahim Qaddoumi, M.D., M.S.
Director of Telemedicine
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
George Vélez, DHAc, M.B.A., CFAAMA
Administrative Director
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
Gaston K. Rivera, M.D.
Medical Director, Chile Program
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
Yuri Quintana, Ph.D.
Director, Education and Informatics
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
Richard O’Brien
Educational Technologies Administrator
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
Jorge I. Calzada, M.D.
Ophthalmologist
Hamilton Eye Institute
UT Health Science Center
Memphis, TN
Sandra Luna-Fineman, M.D.
Pediatric Hematologist/Oncologist
California Pacific Med Center-Davies
San Francisco, CA
The Richard T. and Josephine Arkwright Foundation
New York, NY
Robert B. Carter, M.B.A.
Executive Vice President & Chief Information Officer
FedEx Corporation
Memphis, TN
Blanca Phillips
Coordinator and Ophthalmic Photographer
UT Hamilton Eye Institute
International Outreach Program
St. Jude Children’s Research Hospital
Memphis, TN
Lee A. Thompson, M.A.
Publication Specialist
Hamilton Eye Institute
UT Health Science Center
Memphis, TN
Guatemala
Federico Antillon-Klussman, M.D.
Pediatric Hematologist/Oncologist & Medical Director
Hospital de Oncología Pediátrica
Guatemala City, Guatemala
Margarita Barnoya, M.D.
Ophthalmologist
Hospital de Oncología Pediátrica
Guatemala City, Guatemala
Mauricio Castellanos, M.D.
Pediatric Hematologist/Oncologist
Hospital de Oncología Pediátrica
Guatemala City, Guatemala
Guillermo Chantada, M.D.
Pediatric Hematologist/Oncologist
Department of Hemato-Oncology, Hospital JP Garrahan
Buenos Aires, Argentina
Panama
Ernesto Calvo, M.D.
Ophthalmologist
Clinica de Ojos Orillac-Calvo
Panama, Republic of Panama
Maria Sabina Ahchu, M.D.
Pediatric Hematologist/Oncologist
Hospital del Niño de Panamá
Panama, Republic of Panama
J. Thomas Ford
Trustee, Board Member
Director, and Past President Programa Niños Sanos: Niños Felices
Panama, Republic of Panama
Honduras
Ligia Fu, M.D.
Pediatric Hematologist/Oncologist & Medical Director
Hospital Escuela Materno Infantil
Tegucigalpa, Honduras
Carlos Maldonado, M.D.
Ophthalmologist
Hospital Escuela Materno Infantil
Tegucigalpa, Honduras
Lilia Lopez, M.D.
Ophthalmologist
Hospital Escuela Materno Infantil
Tegucigalpa, Honduras
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Haik, B.G. (2012). Retinoblastoma Management: Connecting Institutions with Telemedicine. In: Yogesan, K., Goldschmidt, L., Cuadros, J. (eds) Digital Teleretinal Screening. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-25810-7_19
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