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Latest revision as of 23:59, 29 July 2020

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

Overview

The incidence of retinoblastoma in the United States has been reported to be 1.2 cases per 100,000 children aged 4 years or younger. The median age at diagnosis of retinoblastoma is 18 months. The average age at diagnosis of retinoblastoma for children with unilateral disease and bilateral disease is 24 months and 12 months respectively. Retinoblastoma affects males and females equally. There is no racial predilection to the development of retinoblastoma.

Epidemiology and Demographics

Incidence

The incidence of retinoblastoma in the United States has been reported to be 1.2 cases per 100,000 children aged 4 years or younger.^[1]
The tumor incidence has been reported 0.049 cases per 100,000 child aged 5 to 9.

Prevalence

There is no data on the prevalence of retinoblastoma.

Case-fatality rate/Mortality rate

The mortality rate of retinoblastoma differs according to the stage of the disease as well as the geographic region.^[2]
In extraocular form of this disorder is reported to be greater than 50%.^[3]
However, tumors involving the optic disc superficially, are associated with 10% mortality rate.^[4]

Age

The median age at the time of diagnosis is 18 months.^[5]
The average age at diagnosis of retinoblastoma for children with unilateral disease and bilateral disease is 24 months and 12 months respectively.^[6]
Cases of newly diagnosed retinoblastoma have been reported in children as old as 18 years and even in adults.^[7]^[8]
In adults, retinoblastoma tends to present between 20 to 50 years of age.^[9]
Trilateral retinoblastoma is a well-recognized syndrome that occurs in 5% to 15% of patients with heritable retinoblastoma and is defined by the development of an intracranial midline neuroblastic tumor, which typically develops between the ages of 20 and 36 months.

Gender

Retinoblastoma affects males and females equally.^[5]

Race

There is no racial predilection to the development of retinoblastoma.^[5]

Region

Epidemiological data indicates that retinoblastoma has a higher incidence in some geographic areas.^[10]
The table below provides the highest worldwide incidence rate of retinoblastoma in children aged 0 - 4:


Country	Incidence
Mali	4.25
Uganda	2.4
Zimbabwe	2.33
Hawaii	2.25
India	1.96
Vietnam	1.89
Singapore	1.88
New Zealand	1.78 - 1.86
Spain	1.78
Philippines	1.74
Colombia	1.71
Ecuador	1.66
Nigeria	1.61
Costa Rica	1.57
Peru	1.55

References

↑ 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.
↑ Dimaras, Helen; Kimani, Kahaki; Dimba, Elizabeth AO; Gronsdahl, Peggy; White, Abby; Chan, Helen SL; Gallie, Brenda L (2012). "Retinoblastoma". The Lancet. 379 (9824): 1436–1446. doi:10.1016/S0140-6736(11)61137-9. ISSN 0140-6736.
↑ Kim, J W; Kathpalia, V; Dunkel, I J; Wong, R K; Riedel, E; Abramson, D H (2008). "Orbital recurrence of retinoblastoma following enucleation". British Journal of Ophthalmology. 93 (4): 463–467. doi:10.1136/bjo.2008.138453. ISSN 0007-1161.
↑ Chévez-Barrios, Patricia; Eagle, Ralph C.; Marback, Eduardo F. (2015). "Histopathologic Features and Prognostic Factors": 167–183. doi:10.1007/978-3-662-43451-2_16.
↑ ^5.0 ^5.1 ^5.2 Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW (1998). "Presenting signs of retinoblastoma". J Pediatr. 132 (3 Pt 1): 505–8. PMID 9544909.
↑ Broaddus E, Topham A, Singh AD (2009). "Incidence of retinoblastoma in the USA: 1975-2004". Br J Ophthalmol. 93 (1): 21–3. doi:10.1136/bjo.2008.138750. PMID 18621794.
↑ Binder PS (1974). "Unusual manifestations of retinoblastoma". Am J Ophthalmol. 77 (5): 674–9. PMID 4132770.
↑ Zakka KA, Yee RD, Foos RY (1983). "Retinoblastoma in a 12-year-old girl". Ann Ophthalmol. 15 (1): 88–91. PMID 6830100.
↑ Kaliki S, Shields CL, Gupta A, Mishra DK, Das C, Say EA, Shields JA (December 2015). "NEWLY DIAGNOSED ACTIVE RETINOBLASTOMA IN ADULTS". Retina (Philadelphia, Pa.). 35 (12): 2483–8. doi:10.1097/IAE.0000000000000612. PMID 26035399.
↑ Singh, Arun (2007). Clinical ophthalmic oncology. Edinburgh: Elsevier Saunders. ISBN 978-1-4160-3167-3.

[FernandesPollock2018-1] 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.

[DimarasKimani2012-2] Dimaras, Helen; Kimani, Kahaki; Dimba, Elizabeth AO; Gronsdahl, Peggy; White, Abby; Chan, Helen SL; Gallie, Brenda L (2012). "Retinoblastoma". The Lancet. 379 (9824): 1436–1446. doi:10.1016/S0140-6736(11)61137-9. ISSN 0140-6736.

[KimKathpalia2008-3] Kim, J W; Kathpalia, V; Dunkel, I J; Wong, R K; Riedel, E; Abramson, D H (2008). "Orbital recurrence of retinoblastoma following enucleation". British Journal of Ophthalmology. 93 (4): 463–467. doi:10.1136/bjo.2008.138453. ISSN 0007-1161.

[Chévez-BarriosEagle2015-4] Chévez-Barrios, Patricia; Eagle, Ralph C.; Marback, Eduardo F. (2015). "Histopathologic Features and Prognostic Factors": 167–183. doi:10.1007/978-3-662-43451-2_16.

[pmid9544909-5] 5.0 ^5.1 ^5.2 Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW (1998). "Presenting signs of retinoblastoma". J Pediatr. 132 (3 Pt 1): 505–8. PMID 9544909.

[pmid18621794-6] Broaddus E, Topham A, Singh AD (2009). "Incidence of retinoblastoma in the USA: 1975-2004". Br J Ophthalmol. 93 (1): 21–3. doi:10.1136/bjo.2008.138750. PMID 18621794.

[pmid4132770-7] Binder PS (1974). "Unusual manifestations of retinoblastoma". Am J Ophthalmol. 77 (5): 674–9. PMID 4132770.

[pmid6830100-8] Zakka KA, Yee RD, Foos RY (1983). "Retinoblastoma in a 12-year-old girl". Ann Ophthalmol. 15 (1): 88–91. PMID 6830100.

[pmid26035399-9] Kaliki S, Shields CL, Gupta A, Mishra DK, Das C, Say EA, Shields JA (December 2015). "NEWLY DIAGNOSED ACTIVE RETINOBLASTOMA IN ADULTS". Retina (Philadelphia, Pa.). 35 (12): 2483–8. doi:10.1097/IAE.0000000000000612. PMID 26035399.

[10] Singh, Arun (2007). Clinical ophthalmic oncology. Edinburgh: Elsevier Saunders. ISBN 978-1-4160-3167-3.

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@@ Line 1: / Line 1: @@
-{{CMG}}
+__NOTOC__
 {{Retinoblastoma}}
+{{CMG}}; {{AE}} {{Sahar}} {{Simrat}}
 ==Overview==
+The [[incidence]] of retinoblastoma in the United States has been reported to be 1.2 cases per 100,000 [[Child|children]] aged 4 years or younger. The [[median]] age at [[diagnosis]] of retinoblastoma is 18 months. The average age at [[diagnosis]] of retinoblastoma for children with unilateral [[disease]] and [[bilateral]] [[disease]] is 24 months and 12 months respectively. Retinoblastoma affects [[Male|males]] and [[Female|females]] equally. There is no [[racial]] predilection to the development of retinoblastoma.
+==Epidemiology and Demographics==
+===Incidence===
+*The [[incidence]] of retinoblastoma in the United States has been reported to be 1.2 cases per 100,000 children aged 4 years or younger.<ref name="FernandesPollock2018">{{cite journal|last1=Fernandes|first1=Arthur Gustavo|last2=Pollock|first2=Benjamin D.|last3=Rabito|first3=Felicia A.|title=Retinoblastoma in the United States: A 40-Year Incidence and Survival Analysis|journal=Journal of Pediatric Ophthalmology & Strabismus|volume=55|issue=3|year=2018|pages=182–188|issn=0191-3913|doi=10.3928/01913913-20171116-03}}</ref>
+*The [[tumor]] [[incidence]] has been reported 0.049 cases per 100,000 child aged 5 to 9.
+===Prevalence===
+*There is no data on the [[prevalence]] of retinoblastoma.
+===Case-fatality rate/Mortality rate===
+*The [[mortality rate]] of retinoblastoma differs according to the stage of the [[disease]] as well as the geographic region.<ref name="DimarasKimani2012">{{cite journal|last1=Dimaras|first1=Helen|last2=Kimani|first2=Kahaki|last3=Dimba|first3=Elizabeth AO|last4=Gronsdahl|first4=Peggy|last5=White|first5=Abby|last6=Chan|first6=Helen SL|last7=Gallie|first7=Brenda L|title=Retinoblastoma|journal=The Lancet|volume=379|issue=9824|year=2012|pages=1436–1446|issn=01406736|doi=10.1016/S0140-6736(11)61137-9}}</ref>
+*In extraocular form of this [[disorder]] is reported to be greater than 50%.<ref name="KimKathpalia2008">{{cite journal|last1=Kim|first1=J W|last2=Kathpalia|first2=V|last3=Dunkel|first3=I J|last4=Wong|first4=R K|last5=Riedel|first5=E|last6=Abramson|first6=D H|title=Orbital recurrence of retinoblastoma following enucleation|journal=British Journal of Ophthalmology|volume=93|issue=4|year=2008|pages=463–467|issn=0007-1161|doi=10.1136/bjo.2008.138453}}</ref>
+*However, [[tumors]] involving the [[optic disc]] superficially, are associated with 10% [[mortality rate]].<ref name="Chévez-BarriosEagle2015">{{cite journal|last1=Chévez-Barrios|first1=Patricia|last2=Eagle|first2=Ralph C.|last3=Marback|first3=Eduardo F.|title=Histopathologic Features and Prognostic Factors|year=2015|pages=167–183|doi=10.1007/978-3-662-43451-2_16}}</ref>
-==Incidence==
+===Age===
-The incidence of retinoblastoma in US is approximately 4.3 cases per 1000,000 children under 15 years of age.<ref name="SEER">  Retinoblastoma. SEER(2015) http://seer.cancer.gov/csr/1975_2012/results_merged/sect_29_childhood_cancer_iccc.pdf#search=retinoblastoma Accessed on October 2 2015</ref>  The age-adjusted annual incidence in children aged 0 to 4 years is 10 to 14 cases per 1 million (approximately one in 14,000–18,000 live births). Retinoblastoma presents with cumulative lifetime incidence rate of 1 case of retinoblastoma per 18000 to 30000 live births worldwide.<ref name="pmid15579980">{{cite journal| author=Abramson DH, Schefler AC| title=Update on retinoblastoma. | journal=Retina | year= 2004 | volume= 24 | issue= 6 | pages= 828-48 | pmid=15579980 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15579980  }} </ref>
+*The [[median]] age at the time of [[diagnosis]] is 18 months.<ref name="pmid9544909">{{cite journal| author=Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW| title=Presenting signs of retinoblastoma. | journal=J Pediatr | year= 1998 | volume= 132 | issue= 3 Pt 1 | pages= 505-8 | pmid=9544909 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9544909  }} </ref>
+*The average age at [[diagnosis]] of retinoblastoma for children with unilateral [[disease]] and [[bilateral]] [[disease]] is 24 months and 12 months respectively.<ref name="pmid18621794">{{cite journal| author=Broaddus E, Topham A, Singh AD| title=Incidence of retinoblastoma in the USA: 1975-2004. | journal=Br J Ophthalmol | year= 2009 | volume= 93 | issue= 1 | pages= 21-3 | pmid=18621794 | doi=10.1136/bjo.2008.138750 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18621794  }} </ref>
+*Cases of newly [[Diagnose|diagnosed]] retinoblastoma have been reported in children as old as 18 years and even in [[Adult|adults]].<ref name="pmid4132770">{{cite journal| author=Binder PS| title=Unusual manifestations of retinoblastoma. | journal=Am J Ophthalmol | year= 1974 | volume= 77 | issue= 5 | pages= 674-9 | pmid=4132770 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4132770  }} </ref><ref name="pmid6830100">{{cite journal| author=Zakka KA, Yee RD, Foos RY| title=Retinoblastoma in a 12-year-old girl. | journal=Ann Ophthalmol | year= 1983 | volume= 15 | issue= 1 | pages= 88-91 | pmid=6830100 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6830100  }} </ref>
+*In [[Adult|adults]], retinoblastoma tends to present between 20 to 50 years of age.<ref name="pmid26035399">{{cite journal |vauthors=Kaliki S, Shields CL, Gupta A, Mishra DK, Das C, Say EA, Shields JA |title=NEWLY DIAGNOSED ACTIVE RETINOBLASTOMA IN ADULTS |journal=Retina (Philadelphia, Pa.) |volume=35 |issue=12 |pages=2483–8 |date=December 2015 |pmid=26035399 |doi=10.1097/IAE.0000000000000612 |url=}}</ref>
+*Trilateral retinoblastoma is a well-recognized [[syndrome]] that occurs in 5% to 15% of [[Patient|patients]] with [[heritable]] retinoblastoma and is defined by the [[development]] of an [[Cranial|intracranial]] midline [[Neuroblast|neuroblastic]] [[tumor]], which typically develops between the ages of 20 and 36 months.
-Thus, while the estimated annual incidence in the United States is approximately four cases per 1 million children younger than 15 years,
+===Gender===
-The incidence of retinoblastoma is 1 in 12,000 to 30,000 live births. [6] In the US, there are 250 to 350 new cases per year. The incidence in the US is relatively low at 3.58 cases per year per million children under 15 years of age. For many years, the reported median age at diagnosis has been 18 months, with the median age of diagnosis of bilateral cases occurring at 12 months and of unilateral cases at 24 months. [7] Recently, however, European investigators have questioned the basis on which these epidemiologic assumptions are made and have reported that the age at diagnosis of unilateral cases may be equal to that of bilateral cases.
+*Retinoblastoma affects [[Male|males]] and [[Female|females]] equally.<ref name="pmid9544909">{{cite journal |vauthors=Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW |title=Presenting signs of retinoblastoma |journal=J. Pediatr. |volume=132 |issue=3 Pt 1 |pages=505–8 |date=March 1998 |pmid=9544909 |doi= |url=}}</ref>
-Here are some highlights listed by the SEER (Surveillance, Epidemiology and End Results) about the incidence of retinoblastoma among children and adolescents.<ref> http://seer.cancer.gov/publications/childhood/retinoblastoma.pdf </ref>
+===Race===
+*There is no [[racial]] predilection to the development of retinoblastoma.<ref name="pmid9544909">{{cite journal |vauthors=Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW |title=Presenting signs of retinoblastoma |journal=J. Pediatr. |volume=132 |issue=3 Pt 1 |pages=505–8 |date=March 1998 |pmid=9544909 |doi= |url=}}</ref>
+===Region===
+*[[Epidemiological]] data indicates that retinoblastoma has a higher [[incidence]] in some geographic areas.<ref>{{cite book | last = Singh | first = Arun | title = Clinical ophthalmic oncology | publisher = Elsevier Saunders | location = Edinburgh | year = 2007 | isbn = 978-1-4160-3167-3 }}</ref>
+*The table below provides the highest worldwide [[incidence rate]] of retinoblastoma in children aged 0 - 4:
-* Retinoblastoma accounted for approximately 11% of cancers developing in the first year of life,but for only 3% of the cancers developing among children younger than 15 years of age.
+{| border="3"
-* In the US, approximately 300 children and adolescents younger than 20 years of age are diagnosed with retinoblastomas each year.
+|+
-* The vast majority of cases of retinoblastoma occur among young children with almost two-thirds(63%) of retinoblastomas occuring before the age of two years and 95% occuring before the age of 5 years.
+! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Country}} !! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Incidence}}
-* The incidence of bilateral tumors was strongly age dependent with 42% of the retinoblastomas occuring in children less than one year of age being bilateral compared to 21% of those among children aged one year, and only 9% among older children.
+|-
-* Rates of retinoblastoma were the same among males (3.7 per million) and females (4.0 per million) and also among whites (3.7 per million) and blacks (4.0 per million).
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Mali
-* There was no subsantial change in the retinoblastoma incidence during the 21 year period, 1975-95.
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" | 4.25
-* It is estimated that 38.2% of patients with hereditary retinoblastoma will develop a secondary malignancy with an associated long-term mortality rate of 26%.
+|-
-* Retinoblastoma presents with cumulative lifetime incidence rate of 1 case of retinoblastoma per 18000 to 30000 live births worldwide. A higher incidence is noted in developing countries, this has been implicated to lower socioeconomic status and the presence of human papilloma virus sequences in the retinoblastoma tissue.
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | Uganda
-* Almost 80% of children with retinoblastoma are diagnosed before 3 years of age and diagnosis in children above 6 years of age is extremely rare. In the UK, bilateral cases usually present within 14–16 months, while diagnosis of unilateral cases peaks between 24 and 30 months.
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |2.4
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | Zimbabwe
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |2.33
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | Hawaii
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |2.25
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | India
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.96
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | Vietnam
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.89
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Singapore
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.88
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |New Zealand
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.78 - 1.86
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Spain
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.78
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Philippines
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.74
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Colombia
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.71
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Ecuador
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.66
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Nigeria
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.61
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Costa Rica
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.57
+|-
+! style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |Peru
+| style="padding: 5px 5px; background: #F5F5F5;" align="center" |1.55
+|}
 ==References==
-<references/>
+{{reflist|2}}
-==See also==
+[[Category:Medicine]]
-*[[Eye cancer]]
+[[Category:Oncology]]
-*[[Eye examination]]
+[[Category:Up-To-Date]]
+[[Category:Surgery]]
-{{Nervous tissue tumors}}
-[[Category:Ophthalmology]]
-[[Category:Types of cancer]]
-[[Category:hereditary cancers]]
-[[Category:Oncology stub]]
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