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| {{CMG}};{{AE}} {{Simrat}} {{Sahar}} | | {{CMG}};{{AE}} {{Simrat}} {{Sahar}} |
| ==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.<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> 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.<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> | | 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, Complications, and Prognosis== |
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| ==Natural History== | | === Natural History === |
| *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> | | *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> |
| *Left untreated, retinoblastoma may be deadly. The tumor will continue growing and filling the entire globe with subsequent metastasis. | | *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 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> | | *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]] |
| **Hematogenouslyto 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 conjunctivae, 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]] |
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| ==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> |
| *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 is far less in case of eye salvage and depends on the stage of the tumor at the time of diagnosis.
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| *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>
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| *The 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>
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| *The survival for nonpineal trilateral retinoblastoma is 57 percent.
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| *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>
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| *The prognosis is better if the tumors do not involve the [[fovea]], are small, and there is a limited seeding and [[retinal detachment]].
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| *The prognosis for vision in the unaffected eye of children with unilateral retinoblastoma is excellent.
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| *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>
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| *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>
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| *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. | | *Elevated [[intracranial pressure|intracranial pressure (ICP)]] |
| *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 favourable prognosis than children with a higher grade or poorly differentiated tumor.
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| {| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
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| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|MRI component}}
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| ! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Features}}
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| :Grade 1
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Well differentiated or low grade – slow growing, less likely to spread
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
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| :Grade 2
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Moderately well differentiated or moderate grade
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
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| :Grade 3
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Poorly differentiated or high grade – tend to grow quickly, more likely to spread
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
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| :Grade 4
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Undifferentiated or high grade – tend to grow very quickly, likely to spread
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| |}
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| 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>
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| *[[Cataract]]
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| *Larger [[tumor]]
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| *Greater age
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| *[[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>
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| *Evidence of [[optic nerve]] involvement
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| *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>
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| *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>
| | * 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> |
| **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]]
| |
| *Recurrence of [[tumor]]
| |
| *Trilateral retinnoblastoma
| |
| **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.<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> Approximately 5% to 10% of children with [[heritable]] retinoblastoma develop [[pineal gland]] [[cysts]] detected by [[MRI]]; these cyst abnormalities must be distinguished from the pineoblastoma that typically defines trilateral retinoblastoma.
| |
| *Subsequent neoplasms | |
| **Patients with retinoblastoma have a high risk of developing subsequent neoplasms. Osteogenic or [[soft tissue]] [[sarcomas]] are the common subsequent neoplasms that develop in children.<ref name="pmid8320741">{{cite journal| author=Eng C, Li FP, Abramson DH, Ellsworth RM, Wong FL, Goldman MB et al.| title=Mortality from second tumors among long-term survivors of retinoblastoma. | journal=J Natl Cancer Inst | year= 1993 | volume= 85 | issue= 14 | pages= 1121-8 | pmid=8320741 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8320741 }} </ref><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="pmid11581064">{{cite journal| author=Abramson DH, Melson MR, Dunkel IJ, Frank CM| title=Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma. | journal=Ophthalmology | year= 2001 | volume= 108 | issue= 10 | pages= 1868-76 | pmid=11581064 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11581064 }} </ref><ref name="pmid17202110">{{cite journal| author=Kleinerman RA, Tucker MA, Abramson DH, Seddon JM, Tarone RE, Fraumeni JF| title=Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma. | journal=J Natl Cancer Inst | year= 2007 | volume= 99 | issue= 1 | pages= 24-31 | pmid=17202110 | doi=10.1093/jnci/djk002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17202110 }} </ref><ref name="pmid15800318">{{cite journal| author=Kleinerman RA, Tucker MA, Tarone RE, Abramson DH, Seddon JM, Stovall M et al.| title=Risk of new cancers after radiotherapy in long-term survivors of retinoblastoma: an extended follow-up. | journal=J Clin Oncol | year= 2005 | volume= 23 | issue= 10 | pages= 2272-9 | pmid=15800318 | doi=10.1200/JCO.2005.05.054 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15800318 }} </ref><ref name="pmid23674091">{{cite journal| author=MacCarthy A, Bayne AM, Brownbill PA, Bunch KJ, Diggens NL, Draper GJ et al.| title=Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004. | journal=Br J Cancer | year= 2013 | volume= 108 | issue= 12 | pages= 2455-63 | pmid=23674091 | doi=10.1038/bjc.2013.228 | pmc=PMC3694232 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23674091 }} </ref> Factors that influence the risk of having subsequent neoplasms include:
| |
| ***Heritable retinoblastoma- There is increased risk of developing secondary primary tumors, including osteogenic [[sarcoma]], [[soft tissue]] [[sarcomas]] (particularly [[leiomyosarcoma]]) and [[malignant melanoma]] in patients with the genetic abnormality associated with heritable forms of retinoblastoma.<ref name="pmid23036192">{{cite journal| author=Kleinerman RA, Schonfeld SJ, Tucker MA| title=Sarcomas in hereditary retinoblastoma. | journal=Clin Sarcoma Res | year= 2012 | volume= 2 | issue= 1 | pages= 15 | pmid=23036192 | doi=10.1186/2045-3329-2-15 | pmc=PMC3499233 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23036192 }} </ref><ref name="pmid8320741">{{cite journal| author=Eng C, Li FP, Abramson DH, Ellsworth RM, Wong FL, Goldman MB et al.| title=Mortality from second tumors among long-term survivors of retinoblastoma. | journal=J Natl Cancer Inst | year= 1993 | volume= 85 | issue= 14 | pages= 1121-8 | pmid=8320741 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8320741 }} </ref><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="pmid3356485">{{cite journal| author=DerKinderen DJ, Koten JW, Nagelkerke NJ, Tan KE, Beemer FA, Den Otter W| title=Non-ocular cancer in patients with hereditary retinoblastoma and their relatives. | journal=Int J Cancer | year= 1988 | volume= 41 | issue= 4 | pages= 499-504 | pmid=3356485 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3356485 }} </ref><ref name="pmid11581064">{{cite journal| author=Abramson DH, Melson MR, Dunkel IJ, Frank CM| title=Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma. | journal=Ophthalmology | year= 2001 | volume= 108 | issue= 10 | pages= 1868-76 | pmid=11581064 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11581064 }} </ref><ref name="pmid17202110">{{cite journal| author=Kleinerman RA, Tucker MA, Abramson DH, Seddon JM, Tarone RE, Fraumeni JF| title=Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma. | journal=J Natl Cancer Inst | year= 2007 | volume= 99 | issue= 1 | pages= 24-31 | pmid=17202110 | doi=10.1093/jnci/djk002 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17202110 }} </ref>
| |
| ***Past treatment of retinoblastoma with [[radiation therapy]].<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>
| |
| ***Age at time of radiation therapy- The risk of subsequent neoplasms appears to be dependent on the patient's age at the time that external-beam radiation therapy is administered, especially in children younger than 12 months, and the [[histopathologic ]]types of subsequent neoplasms may be influenced by age.<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>
| |
| ***Previous subsequent neoplasms- Those who survive subsequent neoplasms are at a sevenfold increased risk for developing another subsequent neoplasm.<ref name="pmid11581064">{{cite journal| author=Abramson DH, Melson MR, Dunkel IJ, Frank CM| title=Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma. | journal=Ophthalmology | year= 2001 | volume= 108 | issue= 10 | pages= 1868-76 | pmid=11581064 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11581064 }} </ref> An additional threefold increase in risk is seen for patients treated with radiation therapy.<ref name="pmid20400293">{{cite journal| author=Marees T, van Leeuwen FE, Schaapveld M, Imhof SM, de Boer MR, Kors WA et al.| title=Risk of third malignancies and death after a second malignancy in retinoblastoma survivors. | journal=Eur J Cancer | year= 2010 | volume= 46 | issue= 11 | pages= 2052-8 | pmid=20400293 | doi=10.1016/j.ejca.2010.03.029 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20400293 }} </ref>
| |
| ===Complications of retinoblastoma therapy===
| |
| *Secondary malignancies
| |
| **[[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== |
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