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

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{{CMG}}
__NOTOC__
{{Retinoblastoma}}
{{Retinoblastoma}}
 
{{CMG}};{{AE}} {{Simrat}} {{Sahar}}
==Overview==
==Overview==
If left untreated, the tumor fills the eye and completely destroys the globe in six months .Retinoblastoma is a rapidly growing tumor. Metastatic spread begins after six months and metastasized tumor is very rare at presentation. The tumor may spread through the subarachnoid space to the contralateral optic nerve or through the cerebrospinal fluid to the central nervous system or hematogenously to the lung, bone, or brain or by lymphatics if the tumor spreads anteriorly into the conjunctivae and eyelids, or extends into extraocular tissue. The most common routes of metastatic spread are direct infiltration via the optic nerve to the central nervous system, or spread via the choroid to the orbit.
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.
If untreated, death usually occurs in few years.
==Natural History, Complications, and Prognosis==


==Natural History==
=== Natural History ===
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. Generally, metastasis may begin within 4 months of diagnosis. Metastasis usually occur through direct invasion of the central nervous system via the optic nerve. Retinoblastoma may spread through the subarachnoid space to the contralateral optic nerve or through the cerebrospinal fluid to the central nervous system and may spread hematogenously to the lungs, bone, and brain.<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> The majority of untreated patients die of intracranial extension and disseminated disease within two one year. Spontaneous regression of the tumor is a rare occurrence but may occur in a small number of cases.<ref name="pmid7126513">{{cite journal| author=Sanborn GE, Augsburger JJ, Shields JA| title=Spontaneous regression of bilateral retinoblastoma. | journal=Br J Ophthalmol | year= 1982 | volume= 66 | issue= 11 | pages= 685-90 | pmid=7126513 | doi= | pmc=PMC1039901 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7126513  }} </ref><ref name="pmid16121553">{{cite journal| author=Kao LY, Yang ML| title=Spontaneous regression of retinoblastoma in a Taiwan series. | journal=J Pediatr Ophthalmol Strabismus | year= 2005 | volume= 42 | issue= 4 | pages= 228-32 | pmid=16121553 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16121553  }} </ref><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 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>
==Prognosis==
*If left untreated, retinoblastoma can be fatal. The [[tumor]] will continue growing and can invade the entire globe of the [[eye]] with subsequent [[metastasis]].
*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> However, the prognosis for eye salvage is far less and  usually depends on the stage of retinoblastoma at diagnosis.
*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>
*In children with retinoblastoma that has invaded into the choroid, optic nerve, sclera, orbit or anterior chamber require chemotherapy as they are at greatest risk for metastasis and death. Those children without evidence of invasion do not require chemotherapy.
*Death may occur within one year of [[metastasis]].
*The goal of treatment in retinoblastoma is to preserve life and to prevent the loss of an eye, blindness, and other serious effects of treatment that reduce the patient's life span or the quality of life. With improvements in the diagnosis and management of retinoblastoma over the past several decades, metastatic retinoblastoma is observed less frequently in the United States and other developed nations.
*[[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>
* As a result, other causes of retinoblastoma-related mortality in the first and subsequent decades of life, such as trilateral retinoblastoma and subsequent neoplasms (SNs), have become significant contributors to retinoblastoma-related mortality. Death from a subsequent neoplasms is the most common cause of death and contributes to more than 50% of deaths for patients with bilateral disease. In the United States, before the advent of chemoreduction as a means of treating heritable or bilateral disease, trilateral retinoblastoma contributed to more than 50% of retinoblastoma-related mortality in the first decade after diagnosis.<ref name="NIH">  Retinoblastoma related mortality. National Cancer Institute(2015) http://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#link/_488_toc Accessed on October 10 2015</ref>
**Direct [[invasion]] of the [[central nervous system]] via the [[optic nerve]]
**Through the [[subarachnoid space]] to the [[contralateral]] [[optic nerve]]
**Through the [[cerebrospinal fluid]] to the [[central nervous system]]
**To the [[lungs]], [[bone]], and [[brain]] via the hematogenous route
**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>
===Complications===
* [[Metastasis]]
*Massive [[choroidal]] [[invasion]]
*[[Tumor]] [[invasion]] into the [[anterior chamber]]
*Large [[tumor]] size with [[vitreous]] seeding
*[[Neovascularization]] of the [[iris]]
*[[Glaucoma]]


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>
* Recurrence of [[tumor]]
*Cataract
* 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>
*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 cancers, 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="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><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>
*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>
*Larger tumor
*Greater age
*Evidence of optic nerve involvement


*Elevated [[intracranial pressure|intracranial pressure (ICP)]]


==Complications==
* 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 complications of retinoblastoma may be
 
*Local spread of tumor
*Development of other tumors
*Cataract from radiotherapy
*Failure of tooth eruption after radiotherapy
*Bony deformities


===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==
<references/>
{{reflist|2}}
 
==See also==
*[[Eye cancer]]
*[[Eye examination]]
 
{{Nervous tissue tumors}}
 
[[Category:Ophthalmology]]
[[Category:Types of cancer]]
[[Category:hereditary cancers]]
[[Category:Oncology stub]]
 


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Latest revision as of 23:59, 29 July 2020

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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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