Retinoblastoma natural history, complications, and prognosis: Difference between revisions

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==Overview==
==Overview==
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.
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 [[Patient|patients]] die of [[Cranium|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==
==Natural History, Complications, and Prognosis==
*[[Retinoblastoma]] usually first presents with [[leukocoria]].<ref>{{cite book | last = Pizzo | first = Philip | title = Principles and practice of pediatric oncology | publisher = Wolters Kluwer/Lippincott Williams & Wilkins Health | location = Philadelphia, PA | year = 2011 | isbn = 160547682X }}</ref>
 
*Left untreated, [[retinoblastoma]] may be deadly. The [[tumor]] will continue growing and filling the entire [[globe]] with subsequent [[metastasis]].
=== Natural History ===
*The [[tumor]] remains within the [[globe]] and curable within 3 to 6 months of its first presentation ([[leukocoria]]). Any delay in the [[diagnosis]] will decrease the [[survival rate]].<ref name="pmid10574806">{{cite journal |vauthors=Goddard AG, Kingston JE, Hungerford JL |title=Delay in diagnosis of retinoblastoma: risk factors and treatment outcome |journal=Br J Ophthalmol |volume=83 |issue=12 |pages=1320–3 |date=December 1999 |pmid=10574806 |doi= |url=}}</ref>
*Retinoblastoma usually presents with [[leukocoria]].<ref>{{cite book | last = Pizzo | first = Philip | title = Principles and practice of pediatric oncology | publisher = Wolters Kluwer/Lippincott Williams & Wilkins Health | location = Philadelphia, PA | year = 2011 | isbn = 160547682X }}</ref>
*If left untreated, retinoblastoma can be fatal. The [[tumor]] will continue growing and can invade the entire globe of the [[eye]] with subsequent [[metastasis]].
*The [[tumor]] remains within the globe of the [[eye]] and curable within 3 to 6 months of its first presentation (when it presents with [[leukocoria]]). Delay in the [[diagnosis]] will decrease the [[survival rate]].<ref name="pmid10574806">{{cite journal |vauthors=Goddard AG, Kingston JE, Hungerford JL |title=Delay in diagnosis of retinoblastoma: risk factors and treatment outcome |journal=Br J Ophthalmol |volume=83 |issue=12 |pages=1320–3 |date=December 1999 |pmid=10574806 |doi= |url=}}</ref>
*Death may occur within one year of [[metastasis]].
*Death may occur within one year of [[metastasis]].
*[[Metastasis]] may occurs through four possible way:<ref>Singh, Arun D., Carol L. Shields, and Jerry A. Shields. "Prognostic factors in retinoblastoma." Journal of pediatric ophthalmology and strabismus 37.3 (2000): 134.</ref><ref name="pmid8635145">{{cite journal |vauthors=Khelfaoui F, Validire P, Auperin A, Quintana E, Michon J, Pacquement H, Desjardins L, Asselain B, Schlienger P, Vielh P |title=Histopathologic risk factors in retinoblastoma: a retrospective study of 172 patients treated in a single institution |journal=Cancer |volume=77 |issue=6 |pages=1206–13 |date=March 1996 |pmid=8635145 |doi= |url=}}</ref><ref name="pmid18757474">{{cite journal |vauthors=Kim JW, Kathpalia V, Dunkel IJ, Wong RK, Riedel E, Abramson DH |title=Orbital recurrence of retinoblastoma following enucleation |journal=Br J Ophthalmol |volume=93 |issue=4 |pages=463–7 |date=April 2009 |pmid=18757474 |doi=10.1136/bjo.2008.138453 |url=}}</ref><ref name="pmid16632438">{{cite journal |vauthors=Leal-Leal CA, Rivera-Luna R, Flores-Rojo M, Juárez-Echenique JC, Ordaz JC, Amador-Zarco J |title=Survival in extra-orbital metastatic retinoblastoma:treatment results |journal=Clin Transl Oncol |volume=8 |issue=1 |pages=39–44 |date=January 2006 |pmid=16632438 |doi= |url=}}</ref>
*[[Metastasis]] may occur via the following four possible pathways:<ref>Singh, Arun D., Carol L. Shields, and Jerry A. Shields. "Prognostic factors in retinoblastoma." Journal of pediatric ophthalmology and strabismus 37.3 (2000): 134.</ref><ref name="pmid8635145">{{cite journal |vauthors=Khelfaoui F, Validire P, Auperin A, Quintana E, Michon J, Pacquement H, Desjardins L, Asselain B, Schlienger P, Vielh P |title=Histopathologic risk factors in retinoblastoma: a retrospective study of 172 patients treated in a single institution |journal=Cancer |volume=77 |issue=6 |pages=1206–13 |date=March 1996 |pmid=8635145 |doi= |url=}}</ref><ref name="pmid18757474">{{cite journal |vauthors=Kim JW, Kathpalia V, Dunkel IJ, Wong RK, Riedel E, Abramson DH |title=Orbital recurrence of retinoblastoma following enucleation |journal=Br J Ophthalmol |volume=93 |issue=4 |pages=463–7 |date=April 2009 |pmid=18757474 |doi=10.1136/bjo.2008.138453 |url=}}</ref><ref name="pmid16632438">{{cite journal |vauthors=Leal-Leal CA, Rivera-Luna R, Flores-Rojo M, Juárez-Echenique JC, Ordaz JC, Amador-Zarco J |title=Survival in extra-orbital metastatic retinoblastoma:treatment results |journal=Clin Transl Oncol |volume=8 |issue=1 |pages=39–44 |date=January 2006 |pmid=16632438 |doi= |url=}}</ref>
**Direct invasion of the [[central nervous system]] via the [[optic nerve]].
**Direct [[invasion]] of the [[central nervous system]] via the [[optic nerve]]
**Through the [[subarachnoid space]] to the [[contralateral]] [[optic nerve]]
**Through the [[subarachnoid space]] to the [[contralateral]] [[optic nerve]]
**Through the [[cerebrospinal fluid]] to the [[central nervous system]]
**Through the [[cerebrospinal fluid]] to the [[central nervous system]]
**Hematogenously to the [[lungs]], [[bone]], and [[brain]].
**To the [[lungs]], [[bone]], and [[brain]] via the hematogenous route
**The [[tumor]] may also spread via [[lymphatic]] pathway if the [[tumor]] dispers anteriorly into the [[Conjunctiva|conjunctivae]], [[Eyelid|eyelids]], or extraocular tissue.  
**The [[tumor]] may also spread via the [[lymphatic|lymphatics]] if the [[tumor]] invades [[Anatomical terms of location|anteriorly]] into the [[Conjunctiva|conjunctivae]], [[Eyelid|eyelids]], or [[Ocular|extraocular]] [[tissue]].  
*Spontaneous regression of the [[tumor]] is a rare occurrence but may occur in a small number of cases.<ref name="pmid898013">{{cite journal| author=Khodadoust AA, Roozitalab HM, Smith RE, Green WR| title=Spontaneous regression of retinoblastoma. | journal=Surv Ophthalmol | year= 1977 | volume= 21 | issue= 6 | pages= 467-78 | pmid=898013 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=898013  }} </ref>
*Spontaneous regression of the [[tumor]] is a rare occurrence but may occur in a small number of cases.<ref name="pmid898013">{{cite journal| author=Khodadoust AA, Roozitalab HM, Smith RE, Green WR| title=Spontaneous regression of retinoblastoma. | journal=Surv Ophthalmol | year= 1977 | volume= 21 | issue= 6 | pages= 467-78 | pmid=898013 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=898013  }} </ref>
*[[Retinoblastoma]] is bilateral in 25%–35% of cases.<ref name="Chandra1994">{{cite journal|last1=Chandra|first1=Suresh R.|title=Intraocular Tumors: A Text and Atlas|journal=Archives of Ophthalmology|volume=112|issue=2|year=1994|pages=169|issn=0003-9950|doi=10.1001/archopht.1994.01090140045016}}</ref>
===Complications===
* [[Metastasis]]
*Massive [[choroidal]] [[invasion]]
*[[Tumor]] [[invasion]] into the [[anterior chamber]]
*Large [[tumor]] size with [[vitreous]] seeding
*[[Neovascularization]] of the [[iris]]
*[[Glaucoma]]


==Prognosis==
* Recurrence of [[tumor]]
*Those with [[heritable]] form of the [[disease]] have 50% risk of transmitting the [[mutation]] to their offspring.<ref name="pmid15637391">{{cite journal |vauthors=Garber JE, Offit K |title=Hereditary cancer predisposition syndromes |journal=J. Clin. Oncol. |volume=23 |issue=2 |pages=276–92 |date=January 2005 |pmid=15637391 |doi=10.1200/JCO.2005.10.042 |url=}}</ref>
* Trilateral retinoblastoma<ref name="DunkelJubran2010">{{cite journal|last1=Dunkel|first1=Ira J.|last2=Jubran|first2=Rima F.|last3=Gururangan|first3=Sri|last4=Chantada|first4=Guillermo L.|last5=Finlay|first5=Jonathan L.|last6=Goldman|first6=Stewart|last7=Khakoo|first7=Yasmin|last8=O'Brien|first8=Joan M.|last9=Orjuela|first9=Manuela|last10=Rodriguez-Galindo|first10=Carlos|last11=Souweidane|first11=Mark M.|last12=Abramson|first12=David H.|title=Trilateral retinoblastoma: Potentially curable with intensive chemotherapy|journal=Pediatric Blood & Cancer|volume=54|issue=3|year=2010|pages=384–387|issn=15455009|doi=10.1002/pbc.22336}}</ref><ref name="KimDunkel2015">{{cite journal|last1=Kim|first1=Jonathan W.|last2=Dunkel|first2=Ira|title=Trilateral Retinoblastoma|year=2015|pages=209–213|doi=10.1007/978-3-662-43451-2_20}}</ref>
*The survival rate of the tumor varies according to  different criteria including the stage of the tumor and even the geographic area of the patients.
**Regarding the variable accessibility of patients to the resources, it may range from < 30% in low and middle income societies to > 90% developped contries.<ref name="pmid22414599">{{cite journal |vauthors=Dimaras H, Kimani K, Dimba EA, Gronsdahl P, White A, Chan HS, Gallie BL |title=Retinoblastoma |journal=Lancet |volume=379 |issue=9824 |pages=1436–46 |date=April 2012 |pmid=22414599 |doi=10.1016/S0140-6736(11)61137-9 |url=}}</ref>
*The overall 5-year survival rate increased over the years and was reported 97.3% from 2000 to 2012.<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 5-year survival rate also differs geographically and is higher in the United States than the 5-year survival rates reported in developing countries and regions.
*The survival rate is higher for unilateral involvement than the bilateral form of the tumor.
*It has been observed that survival rate varies depending following factors:
**Laterality of the tumor
**Age at the time of diagnosis
**Decade of diagnosis


*[[Prognosis]] is generally good, and the [[survival rate]] of patients with [[retinoblastoma]] with treatment is approximately 95%, in the United States.<ref name="pmid19477707">{{cite journal| author=Lin P, O'Brien JM| title=Frontiers in the management of retinoblastoma. | journal=Am J Ophthalmol | year= 2009 | volume= 148 | issue= 2 | pages= 192-8 | pmid=19477707 | doi=10.1016/j.ajo.2009.04.004 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19477707  }} </ref>
*Elevated [[intracranial pressure|intracranial pressure (ICP)]]
*


*However, the [[prognosis]] is far less in case of eye salvage and depends on the stage of the [[tumor]] at the time of [[diagnosis]].
* Subsequent [[neoplasms]]<ref name="pmid19066271">{{cite journal |vauthors=Marees T, Moll AC, Imhof SM, de Boer MR, Ringens PJ, van Leeuwen FE |title=Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up |journal=J. Natl. Cancer Inst. |volume=100 |issue=24 |pages=1771–9 |date=December 2008 |pmid=19066271 |doi=10.1093/jnci/djn394 |url=}}</ref>
 
*The one year to eighteen month [[survival rates]] in patients with hematogenous [[metastases]] are approximately 50 percent.<ref name="pmid3746592">{{cite journal| author=Abramson DH, Ellsworth RM, Grumbach N, Sturgis-Buckhout L, Haik BG| title=Retinoblastoma: correlation between age at diagnosis and survival. | journal=J Pediatr Ophthalmol Strabismus | year= 1986 | volume= 23 | issue= 4 | pages= 174-7 | pmid=3746592 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3746592  }} </ref><ref name="pmid8622068">{{cite journal| author=Schvartzman E, Chantada G, Fandiño A, de Dávila MT, Raslawski E, Manzitti J| title=Results of a stage-based protocol for the treatment of retinoblastoma. | journal=J Clin Oncol | year= 1996 | volume= 14 | issue= 5 | pages= 1532-6 | pmid=8622068 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8622068  }} </ref><ref name="pmid8060814">{{cite journal| author=Pratt CB, Fontanesi J, Chenaille P, Kun LE, Jenkins JJ, Langston JW et al.| title=Chemotherapy for extraocular retinoblastoma. | journal=Pediatr Hematol Oncol | year= 1994 | volume= 11 | issue= 3 | pages= 301-9 | pmid=8060814 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8060814  }} </ref><ref name="pmid12518344">{{cite journal| author=Chantada G, Fandiño A, Casak S, Manzitti J, Raslawski E, Schvartzman E| title=Treatment of overt extraocular retinoblastoma. | journal=Med Pediatr Oncol | year= 2003 | volume= 40 | issue= 3 | pages= 158-61 | pmid=12518344 | doi=10.1002/mpo.10249 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12518344  }} </ref><ref name="pmid7707117">{{cite journal| author=Doz F, Neuenschwander S, Plantaz D, Courbon B, Gentet JC, Bouffet E et al.| title=Etoposide and carboplatin in extraocular retinoblastoma: a study by the Société Française d'Oncologie Pédiatrique. | journal=J Clin Oncol | year= 1995 | volume= 13 | issue= 4 | pages= 902-9 | pmid=7707117 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7707117  }} </ref>
 
*The [[Five year survival rate|five-year survival]] for patients with [[pineal]] trilateral retinoblastoma is 44 percent and five-year.<ref name="pmid25126964">{{cite journal| author=de Jong MC, Kors WA, de Graaf P, Castelijns JA, Kivelä T, Moll AC| title=Trilateral retinoblastoma: a systematic review and meta-analysis. | journal=Lancet Oncol | year= 2014 | volume= 15 | issue= 10 | pages= 1157-67 | pmid=25126964 | doi=10.1016/S1470-2045(14)70336-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25126964  }} </ref>
 
*The [[Survival rate|survival]] for nonpineal trilateral retinoblastoma is 57 percent.
 
*The [[prognosis]] for vision in children with [[bilateral]] retinoblastoma depends upon the effectiveness of therapy and extent of [[tumor]] involvement. <ref name="pmid24671926">{{cite journal| author=Berry JL, Jubran R, Wong K, Lee TC, Murphree AL, Kim JW| title=Factors predictive of long-term visual outcomes of Group D eyes treated with chemoreduction and low-dose IMRT salvage: the Children's Hospital Los Angeles experience. | journal=Br J Ophthalmol | year= 2014 | volume= 98 | issue= 8 | pages= 1061-5 | pmid=24671926 | doi=10.1136/bjophthalmol-2013-304411 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24671926  }} </ref>
 
*The [[prognosis]] is better if the [[Tumor|tumors]] do not involve the [[fovea]], are small, and there is a limited  seeding and [[retinal detachment]].
 
*The [[prognosis]] for vision in the unaffected eye of children with unilateral retinoblastoma is excellent.
 
*There is greater than 50 percent [[mortality rate]] for extra-orbital [[metastatic]] disease.<ref name="pmid18757474">{{cite journal| author=Kim JW, Kathpalia V, Dunkel IJ, Wong RK, Riedel E, Abramson DH| title=Orbital recurrence of retinoblastoma following enucleation. | journal=Br J Ophthalmol | year= 2009 | volume= 93 | issue= 4 | pages= 463-7 | pmid=18757474 | doi=10.1136/bjo.2008.138453 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18757474  }} </ref><ref name="pmid16632438">{{cite journal| author=Leal-Leal CA, Rivera-Luna R, Flores-Rojo M, Juárez-Echenique JC, Ordaz JC, Amador-Zarco J| title=Survival in extra-orbital metastatic retinoblastoma:treatment results. | journal=Clin Transl Oncol | year= 2006 | volume= 8 | issue= 1 | pages= 39-44 | pmid=16632438 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16632438  }} </ref>
 
*For patients with [[germline]] retinoblastoma, the most common cause of death is a secondary [[malignancy]], due to ''[[RB1]]'' deletion, and not the initial primary [[malignancy]].<ref name="pmid10610188">{{cite journal| author=Abramson DH| title=Second nonocular cancers in retinoblastoma: a unified hypothesis. The Franceschetti Lecture. | journal=Ophthalmic Genet | year= 1999 | volume= 20 | issue= 3 | pages= 193-204 | pmid=10610188 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10610188  }} </ref>
 
*Given the poor [[prognosis]] of trilateral retinoblastoma and the short interval between the [[diagnosis]] of retinoblastoma and the occurrence of trilateral [[disease]], routine neuroimaging could potentially detect most cases within 2 years of first [[diagnosis]].
*[[Prognosis]] depends on stage and grade of retinoblastoma.<ref name="CCS">Grades of Retinoblastoma. Canadian Cancer Society(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#cit/section_1.45 Accessed on October 13 2015</ref> Children who have a lower grade of retinoblastoma or a well-differentiated tumor have a more favorable prognosis than children with a higher grade or poorly differentiated [[tumor]].
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align="center"
| valign="top" |
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|MRI component}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Features}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
:Grade 1
| style="padding: 5px 5px; background: #F5F5F5;" |
*Well differentiated or low grade – slow growing, less likely to spread
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Grade 2
| style="padding: 5px 5px; background: #F5F5F5;" |
*Moderately well differentiated or moderate grade
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Grade 3
| style="padding: 5px 5px; background: #F5F5F5;" |
*Poorly differentiated or high grade – tend to grow quickly, more likely to spread
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Grade 4
| style="padding: 5px 5px; background: #F5F5F5;" |
*Undifferentiated or high grade – tend to grow very quickly, likely to spread
|-
|}
The features associated with poor prognosis in retinoblastoma include:<ref name="pmid10845413">{{cite journal| author=Singh AD, Shields CL, Shields JA| title=Prognostic factors in retinoblastoma. | journal=J Pediatr Ophthalmol Strabismus | year= 2000 | volume= 37 | issue= 3 | pages= 134-41; quiz 168-9 | pmid=10845413 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10845413  }} </ref>
*[[Cataract]]
*Larger [[tumor]]
*Greater age
 
*[[Tumor]] [[anaplasia]]<ref name="pmid25528954">{{cite journal| author=Mendoza PR, Specht CS, Hubbard GB, Wells JR, Lynn MJ, Zhang Q et al.| title=Histopathologic grading of anaplasia in retinoblastoma. | journal=Am J Ophthalmol | year= 2015 | volume= 159 | issue= 4 | pages= 764-76 | pmid=25528954 | doi=10.1016/j.ajo.2014.12.014 | pmc=PMC4361305 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25528954  }} </ref>
*Evidence of [[optic nerve]] involvement
 
*Delay in diagnosis of more than six months<ref name="pmid2008269">{{cite journal| author=Messmer EP, Heinrich T, Höpping W, de Sutter E, Havers W, Sauerwein W| title=Risk factors for metastases in patients with retinoblastoma. | journal=Ophthalmology | year= 1991 | volume= 98 | issue= 2 | pages= 136-41 | pmid=2008269 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2008269  }} </ref><ref name="pmid10574806">{{cite journal| author=Goddard AG, Kingston JE, Hungerford JL| title=Delay in diagnosis of retinoblastoma: risk factors and treatment outcome. | journal=Br J Ophthalmol | year= 1999 | volume= 83 | issue= 12 | pages= 1320-3 | pmid=10574806 | doi= | pmc=PMC1722906 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10574806  }} </ref><ref name="pmid2587030">{{cite journal| author=Erwenne CM, Franco EL| title=Age and lateness of referral as determinants of extra-ocular retinoblastoma. | journal=Ophthalmic Paediatr Genet | year= 1989 | volume= 10 | issue= 3 | pages= 179-84 | pmid=2587030 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2587030  }} </ref><ref name="pmid25841975">{{cite journal| author=Kaliki S, Srinivasan V, Gupta A, Mishra DK, Naik MN| title=Clinical features predictive of high-risk retinoblastoma in 403 Asian Indian patients: a case-control study. | journal=Ophthalmology | year= 2015 | volume= 122 | issue= 6 | pages= 1165-72 | pmid=25841975 | doi=10.1016/j.ophtha.2015.01.018 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25841975  }} </ref>
 
*History of intraocular surgery, which may inadvertently contribute to [[vitreous]] seeding or, more importantly, to extraocular spread<ref name="pmid2804029">{{cite journal| author=Stevenson KE, Hungerford J, Garner A| title=Local extraocular extension of retinoblastoma following intraocular surgery. | journal=Br J Ophthalmol | year= 1989 | volume= 73 | issue= 9 | pages= 739-42 | pmid=2804029 | doi= | pmc=PMC1041868 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2804029  }} </ref><ref name="pmid11097606">{{cite journal| author=Shields CL, Honavar S, Shields JA, Demirci H, Meadows AT| title=Vitrectomy in eyes with unsuspected retinoblastoma. | journal=Ophthalmology | year= 2000 | volume= 107 | issue= 12 | pages= 2250-5 | pmid=11097606 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11097606  }} </ref>
 
*Use of external beam [[radiotherapy]], which contributes to the subsequent development of secondary malignancies, particularly in patients with the heritable form of the disease<ref name="pmid9333268">{{cite journal| author=Wong FL, Boice JD, Abramson DH, Tarone RE, Kleinerman RA, Stovall M et al.| title=Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk. | journal=JAMA | year= 1997 | volume= 278 | issue= 15 | pages= 1262-7 | pmid=9333268 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9333268  }} </ref><ref name="pmid9544627">{{cite journal| author=Abramson DH, Frank CM| title=Second nonocular tumors in survivors of bilateral retinoblastoma: a possible age effect on radiation-related risk. | journal=Ophthalmology | year= 1998 | volume= 105 | issue= 4 | pages= 573-9; discussion 579-80 | pmid=9544627 | doi=10.1016/S0161-6420(98)94006-4 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9544627  }} </ref><ref name="pmid9134547">{{cite journal| author=Moll AC, Imhof SM, Bouter LM, Tan KE| title=Second primary tumors in patients with retinoblastoma. A review of the literature. | journal=Ophthalmic Genet | year= 1997 | volume= 18 | issue= 1 | pages= 27-34 | pmid=9134547 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9134547  }} </ref>
 
*[[Choroidal]], [[optic nerve]], scleral, or orbital invasion, which increases the risk of [[metastatic]] disease.<ref name="pmid25528954">{{cite journal| author=Mendoza PR, Specht CS, Hubbard GB, Wells JR, Lynn MJ, Zhang Q et al.| title=Histopathologic grading of anaplasia in retinoblastoma. | journal=Am J Ophthalmol | year= 2015 | volume= 159 | issue= 4 | pages= 764-76 | pmid=25528954 | doi=10.1016/j.ajo.2014.12.014 | pmc=PMC4361305 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25528954  }} </ref><ref name="pmid11801265">{{cite journal| author=Finger PT, Harbour JW, Karcioglu ZA| title=Risk factors for metastasis in retinoblastoma. | journal=Surv Ophthalmol | year= 2002 | volume= 47 | issue= 1 | pages= 1-16 | pmid=11801265 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11801265  }} </ref><ref name="pmid2008269">{{cite journal| author=Messmer EP, Heinrich T, Höpping W, de Sutter E, Havers W, Sauerwein W| title=Risk factors for metastases in patients with retinoblastoma. | journal=Ophthalmology | year= 1991 | volume= 98 | issue= 2 | pages= 136-41 | pmid=2008269 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2008269  }} </ref><ref name="pmid8299091">{{cite journal| author=Shields CL, Shields JA, Baez K, Cater JR, De Potter P| title=Optic nerve invasion of retinoblastoma. Metastatic potential and clinical risk factors. | journal=Cancer | year= 1994 | volume= 73 | issue= 3 | pages= 692-8 | pmid=8299091 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8299091  }} </ref><ref name="pmid8218048">{{cite journal| author=Shields CL, Shields JA, Baez KA, Cater J, De Potter PV| title=Choroidal invasion of retinoblastoma: metastatic potential and clinical risk factors. | journal=Br J Ophthalmol | year= 1993 | volume= 77 | issue= 9 | pages= 544-8 | pmid=8218048 | doi= | pmc=PMC513947 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8218048  }} </ref>
 
*Intraocular Classification of Retinoblastoma (ICRB) has been observed to have the ability to predict the outcome of chemotherapy:<ref name="pmid16996605">{{cite journal |vauthors=Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT, Shields JA |title=The International Classification of Retinoblastoma predicts chemoreduction success |journal=Ophthalmology |volume=113 |issue=12 |pages=2276–80 |date=December 2006 |pmid=16996605 |doi=10.1016/j.ophtha.2006.06.018 |url=}}</ref>
**Category A-C is associated with ≥90% chance to salvage the eye.
**Category D is associated with a 47% chance of eye salvage.
**Category E is excluded due to eye enucleation.
==Complications==
===Complications of retinoblastoma===
[[Metastasis]]
*[[Metastatic]] [[disease]] associates with [[poor prognosis]].
*Several [[histopathologic]] factors has been associated with increased risk of [[metastasis]]. these include:<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>
**Presence of [[Orbital cavity|orbital]] [[invasion]]
**Retrolaminar invasion of [[optic nerve]]
**Scleral and extrascleral [[invasion]]
**Massive [[choroidal]] [[invasion]]
**[[Anterior segment of eye|Anterior segment]] [[Invasion|involvement]]
*The most important [[Prognostic|prognostic factor]] are the involvement of [[optic nerve]] posterior to the [[Lamina cribrosa sclerae|lamina cribrosa]] and extrascleral spread of [[tumor]] into the [[orbit]].
*Other elements with probable risk of [[tumor]] [[metastasis]] include:
**Massive [[choroidal]] [[invasion]]
**[[Tumor]] invasion into the [[anterior chamber]]
**Large [[tumor]] size with [[vitreous]] seeding
**[[Neovascularization]] of the [[iris]]
**[[Glaucoma]]
Recurrence of [[tumor]]<br>
Trilateral retinnoblastoma
*Trilateral retinoblasroma is a rare complication and more commonly affects those with bilateral disease who are less than 1 year of age.<ref name="DunkelJubran2010">{{cite journal|last1=Dunkel|first1=Ira J.|last2=Jubran|first2=Rima F.|last3=Gururangan|first3=Sri|last4=Chantada|first4=Guillermo L.|last5=Finlay|first5=Jonathan L.|last6=Goldman|first6=Stewart|last7=Khakoo|first7=Yasmin|last8=O'Brien|first8=Joan M.|last9=Orjuela|first9=Manuela|last10=Rodriguez-Galindo|first10=Carlos|last11=Souweidane|first11=Mark M.|last12=Abramson|first12=David H.|title=Trilateral retinoblastoma: Potentially curable with intensive chemotherapy|journal=Pediatric Blood & Cancer|volume=54|issue=3|year=2010|pages=384–387|issn=15455009|doi=10.1002/pbc.22336}}</ref>
*Trilateral retinoblastoma is characterized by the combination of bilateral intraocular retinoblastoma and pineoblastoma, a primitive tumor of pineal gland.<ref name="KimDunkel2015">{{cite journal|last1=Kim|first1=Jonathan W.|last2=Dunkel|first2=Ira|title=Trilateral Retinoblastoma|year=2015|pages=209–213|doi=10.1007/978-3-662-43451-2_20}}</ref>
*Patients with this complication may remain asymptomatic and will be discovered incidentally by imaging studies or they may indicate signs of elevated intracranial pressure (ICP).
Subsequent neoplasms
===Complications of retinoblastoma therapy===
**[[Radiation]] and [[chemotherapy]] therapy for retinoblastoma has the potential to induce secondary [[malignancies]] such as [[lymphoma]] and [[leukemia]]. In pediatric cancer patients, the [[epipodophyllotoxin]] [[etoposide]] is known to induce secondary leukemias characterized by site-specific [[DNA]] rearrangements.<ref name="pmid8070034">{{cite journal| author=Rivera GK, Pui CH, Santana VM, Pratt CB, Crist WM| title=Epipodophyllotoxins in the treatment of childhood cancer. | journal=Cancer Chemother Pharmacol | year= 1994 | volume= 34 Suppl | issue=  | pages= S89-95 | pmid=8070034 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8070034  }} </ref>
*[[Visual field defects]]
**Patients with retinoblastoma demonstrate a variety of long-term [[visual field defects]] after treatment for their intraocular disease.<ref name="pmid15364711">{{cite journal| author=Abramson DH, Melson MR, Servodidio C| title=Visual fields in retinoblastoma survivors. | journal=Arch Ophthalmol | year= 2004 | volume= 122 | issue= 9 | pages= 1324-30 | pmid=15364711 | doi=10.1001/archopht.122.9.1324 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15364711  }} </ref>
*[[Hearing loss]]
**Age younger than 6 months at the time of treatment and higher [[carboplatin]] systemic exposures appear to correlate with an increased risk of otologic toxic effects.<ref name="pmid22370329">{{cite journal| author=Qaddoumi I, Bass JK, Wu J, Billups CA, Wozniak AW, Merchant TE et al.| title=Carboplatin-associated ototoxicity in children with retinoblastoma. | journal=J Clin Oncol | year= 2012 | volume= 30 | issue= 10 | pages= 1034-41 | pmid=22370329 | doi=10.1200/JCO.2011.36.9744 | pmc=PMC3341147 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22370329  }} </ref><ref name="pmid22370322">{{cite journal| author=Leahey A| title=A cautionary tale: dosing chemotherapy in infants with retinoblastoma. | journal=J Clin Oncol | year= 2012 | volume= 30 | issue= 10 | pages= 1023-4 | pmid=22370322 | doi=10.1200/JCO.2011.39.4254 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22370322  }} </ref>
*[[Temporal bone]] [[hypoplasia]] after external beam [[radiation]]
*[[Cataract]] formation after external beam radiation
*[[Optic nerve]] atrophy after caboplatin therapy<ref name="pmid12912689">{{cite journal| author=Mulvihill A, Budning A, Jay V, Vandenhoven C, Heon E, Gallie BL et al.| title=Ocular motility changes after subtenon carboplatin chemotherapy for retinoblastoma. | journal=Arch Ophthalmol | year= 2003 | volume= 121 | issue= 8 | pages= 1120-4 | pmid=12912689 | doi=10.1001/archopht.121.8.1120 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12912689  }} </ref>
*[[Fibrosis]] of [[extraocular muscles]] after carboplatin therapy<ref name="pmid16876514">{{cite journal| author=Schmack I, Hubbard GB, Kang SJ, Aaberg TM, Grossniklaus HE| title=Ischemic necrosis and atrophy of the optic nerve after periocular carboplatin injection for intraocular retinoblastoma. | journal=Am J Ophthalmol | year= 2006 | volume= 142 | issue= 2 | pages= 310-5 | pmid=16876514 | doi=10.1016/j.ajo.2006.02.044 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16876514  }} </ref>
*Potential complications of [[enucleation]] include scleral perforation with seeding of [[tumor]] cells into the orbit.


===Prognosis===
*Those with [[heritable]] form of the [[disease]] have 50% risk of transmitting the [[mutation]] to their offspring.<ref name="pmid15637391">{{cite journal |vauthors=Garber JE, Offit K |title=Hereditary cancer predisposition syndromes |journal=J. Clin. Oncol. |volume=23 |issue=2 |pages=276–92 |date=January 2005 |pmid=15637391 |doi=10.1200/JCO.2005.10.042 |url=}}</ref>
*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.<ref name="pmid22414599">{{cite journal |vauthors=Dimaras H, Kimani K, Dimba EA, Gronsdahl P, White A, Chan HS, Gallie BL |title=Retinoblastoma |journal=Lancet |volume=379 |issue=9824 |pages=1436–46 |date=April 2012 |pmid=22414599 |doi=10.1016/S0140-6736(11)61137-9 |url=}}</ref>
*The overall 5-year [[survival rate]] increased over the years and was reported 97.3% from 2000 to 2012.<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>
*[[Prognosis]] is generally good, and the [[survival rate]] of patients with retinoblastoma with treatment is approximately 95%, in the United States.<ref name="pmid19477707">{{cite journal| author=Lin P, O'Brien JM| title=Frontiers in the management of retinoblastoma. | journal=Am J Ophthalmol | year= 2009 | volume= 148 | issue= 2 | pages= 192-8 | pmid=19477707 | doi=10.1016/j.ajo.2009.04.004 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19477707  }} </ref>
*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:
**Laterality of the [[tumor]]
**Age at the time of [[diagnosis]]
**Decade of [[diagnosis]]
*The overall [[prognosis]] of trilateral retinoblastoma is poor and [[Patient|patients]] usually die within the first year of the [[diagnosis]].<ref name="pmid8040018">{{cite journal |vauthors=Blach LE, McCormick B, Abramson DH, Ellsworth RM |title=Trilateral retinoblastoma--incidence and outcome: a decade of experience |journal=Int. J. Radiat. Oncol. Biol. Phys. |volume=29 |issue=4 |pages=729–33 |date=July 1994 |pmid=8040018 |doi= |url=}}</ref>
*Intraocular Classification of Retinoblastoma (ICRB) has been observed to have the ability to predict the outcome of [[chemotherapy]]:<ref name="pmid16996605">{{cite journal |vauthors=Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT, Shields JA |title=The International Classification of Retinoblastoma predicts chemoreduction success |journal=Ophthalmology |volume=113 |issue=12 |pages=2276–80 |date=December 2006 |pmid=16996605 |doi=10.1016/j.ophtha.2006.06.018 |url=}}</ref>
**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]].<ref name="pmid15196536">{{cite journal |vauthors=Aerts I, Pacquement H, Doz F, Mosseri V, Desjardins L, Sastre X, Michon J, Rodriguez J, Schlienger P, Zucker JM, Quintana E |title=Outcome of second malignancies after retinoblastoma: a retrospective analysis of 25 patients treated at the Institut Curie |journal=Eur. J. Cancer |volume=40 |issue=10 |pages=1522–9 |date=July 2004 |pmid=15196536 |doi=10.1016/j.ejca.2004.03.023 |url=}}</ref>
==References==
==References==
{{reflist|2}}
{{reflist|2}}
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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]

Overview

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

Complications

Prognosis

  • 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]

References

  1. ↑ Pizzo, Philip (2011). Principles and practice of pediatric oncology. Philadelphia, PA: Wolters Kluwer/Lippincott Williams & Wilkins Health. ISBN 160547682X.
  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.
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