Retinoblastoma natural history, complications, and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2] Sahar Memar Montazerin, M.D.[3]


If left untreated, retinoblastoma may progress to develop seeding in the eye, leading to retinal detachment, necrosis and invasion of the orbit, optic nerve invasion, and central nervous system invasion. The majority of untreated patients die of intracranial extension and disseminated disease within one year. Spontaneous regression of the tumor is a rare occurrence but may occur in a small number of cases. Common complications of retinoblastoma include metastasis, tumor recurrence, trilateral retinoblastoma, and subsequent neoplasms. Prognosis is generally good, and the survival rate of patients with retinoblastoma with treatment is approximately 95% in the United States.

Natural History, Complications, and Prognosis

Natural History



  • Those with heritable form of the disease have 50% risk of transmitting the mutation to their offspring.[11]
  • Regarding the variable accessibility of patients to the resources, the survival rate may range from < 30% in low and middle income societies to > 90% in developed countries.[12]
  • The overall 5-year survival rate increased over the years and was reported 97.3% from 2000 to 2012.[13]
  • Prognosis is generally good, and the survival rate of patients with retinoblastoma with treatment is approximately 95%, in the United States.[14]
  • The survival rate is higher for unilateral involvement than the bilateral form of the tumor.
  • It has been observed that survival rate varies depending upon the following factors:
  • The overall prognosis of trilateral retinoblastoma is poor and patients usually die within the first year of the diagnosis.[15]
  • Intraocular Classification of Retinoblastoma (ICRB) has been observed to have the ability to predict the outcome of chemotherapy:[16]
    • Category A - C is associated with ≥ 90% chance to salvage the eye.
    • Category D is associated with a 47% chance to salvage the eye.
    • Category E is excluded due to eye enucleation.
  • Prognosis is usually poor with non-ocular tumor and it usually occurs in individuals who have received radiation therapy for their primary retinoblastoma tumors.[17]


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  2. Goddard AG, Kingston JE, Hungerford JL (December 1999). "Delay in diagnosis of retinoblastoma: risk factors and treatment outcome". Br J Ophthalmol. 83 (12): 1320–3. PMID 10574806.
  3. Singh, Arun D., Carol L. Shields, and Jerry A. Shields. "Prognostic factors in retinoblastoma." Journal of pediatric ophthalmology and strabismus 37.3 (2000): 134.
  4. Khelfaoui F, Validire P, Auperin A, Quintana E, Michon J, Pacquement H, Desjardins L, Asselain B, Schlienger P, Vielh P (March 1996). "Histopathologic risk factors in retinoblastoma: a retrospective study of 172 patients treated in a single institution". Cancer. 77 (6): 1206–13. PMID 8635145.
  5. Kim JW, Kathpalia V, Dunkel IJ, Wong RK, Riedel E, Abramson DH (April 2009). "Orbital recurrence of retinoblastoma following enucleation". Br J Ophthalmol. 93 (4): 463–7. doi:10.1136/bjo.2008.138453. PMID 18757474.
  6. Leal-Leal CA, Rivera-Luna R, Flores-Rojo M, Juárez-Echenique JC, Ordaz JC, Amador-Zarco J (January 2006). "Survival in extra-orbital metastatic retinoblastoma:treatment results". Clin Transl Oncol. 8 (1): 39–44. PMID 16632438.
  7. Khodadoust AA, Roozitalab HM, Smith RE, Green WR (1977). "Spontaneous regression of retinoblastoma". Surv Ophthalmol. 21 (6): 467–78. PMID 898013.
  8. Dunkel, Ira J.; Jubran, Rima F.; Gururangan, Sri; Chantada, Guillermo L.; Finlay, Jonathan L.; Goldman, Stewart; Khakoo, Yasmin; O'Brien, Joan M.; Orjuela, Manuela; Rodriguez-Galindo, Carlos; Souweidane, Mark M.; Abramson, David H. (2010). "Trilateral retinoblastoma: Potentially curable with intensive chemotherapy". Pediatric Blood & Cancer. 54 (3): 384–387. doi:10.1002/pbc.22336. ISSN 1545-5009.
  9. Kim, Jonathan W.; Dunkel, Ira (2015). "Trilateral Retinoblastoma": 209–213. doi:10.1007/978-3-662-43451-2_20.
  10. Marees T, Moll AC, Imhof SM, de Boer MR, Ringens PJ, van Leeuwen FE (December 2008). "Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up". J. Natl. Cancer Inst. 100 (24): 1771–9. doi:10.1093/jnci/djn394. PMID 19066271.
  11. Garber JE, Offit K (January 2005). "Hereditary cancer predisposition syndromes". J. Clin. Oncol. 23 (2): 276–92. doi:10.1200/JCO.2005.10.042. PMID 15637391.
  12. Dimaras H, Kimani K, Dimba EA, Gronsdahl P, White A, Chan HS, Gallie BL (April 2012). "Retinoblastoma". Lancet. 379 (9824): 1436–46. doi:10.1016/S0140-6736(11)61137-9. PMID 22414599.
  13. Fernandes, Arthur Gustavo; Pollock, Benjamin D.; Rabito, Felicia A. (2018). "Retinoblastoma in the United States: A 40-Year Incidence and Survival Analysis". Journal of Pediatric Ophthalmology & Strabismus. 55 (3): 182–188. doi:10.3928/01913913-20171116-03. ISSN 0191-3913.
  14. Lin P, O'Brien JM (2009). "Frontiers in the management of retinoblastoma". Am J Ophthalmol. 148 (2): 192–8. doi:10.1016/j.ajo.2009.04.004. PMID 19477707.
  15. Blach LE, McCormick B, Abramson DH, Ellsworth RM (July 1994). "Trilateral retinoblastoma--incidence and outcome: a decade of experience". Int. J. Radiat. Oncol. Biol. Phys. 29 (4): 729–33. PMID 8040018.
  16. Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT, Shields JA (December 2006). "The International Classification of Retinoblastoma predicts chemoreduction success". Ophthalmology. 113 (12): 2276–80. doi:10.1016/j.ophtha.2006.06.018. PMID 16996605.
  17. Aerts I, Pacquement H, Doz F, Mosseri V, Desjardins L, Sastre X, Michon J, Rodriguez J, Schlienger P, Zucker JM, Quintana E (July 2004). "Outcome of second malignancies after retinoblastoma: a retrospective analysis of 25 patients treated at the Institut Curie". Eur. J. Cancer. 40 (10): 1522–9. doi:10.1016/j.ejca.2004.03.023. PMID 15196536.