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

Jump to navigation Jump to search
VisualWikitext
m (Bot: Removing from Primary care)
 
(4 intermediate revisions by 2 users not shown)
Line 3: Line 3:
{{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. 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===
==Prognosis==
* [[Metastasis]]
*Massive [[choroidal]] [[invasion]]
*[[Tumor]] [[invasion]] into the [[anterior chamber]]
*Large [[tumor]] size with [[vitreous]] seeding
*[[Neovascularization]] of the [[iris]]
*[[Glaucoma]]
 
* Recurrence of [[tumor]]
* 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>
 
*Elevated [[intracranial pressure|intracranial pressure (ICP)]]
 
* 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>
 
===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>
*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>
*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, 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>
**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 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 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]].
*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:
*It has been observed that survival rate varies depending following factors:
**Laterality of the [[tumor]]
**Laterality of the tumor
**Age at the time of [[diagnosis]]
**Age at the time of diagnosis
**Decade 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>
*[[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>
*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>
*The overall prognosis of trilateral retinoblastoma is poor and 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>
**Category A - C is associated with ≥ 90% chance to salvage the [[eye]].
*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 D is associated with a 47% chance to salvage the [[eye]].
**Category A-C is associated with ≥90% chance to salvage the eye.
**Category E is excluded due to eye [[enucleation]].
**Category D is associated with a 47% chance of eye salvage.
*[[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>
**Category E is excluded due to eye enucleation.
*Prognosis is usually poor with non-ocular tumor and it usually occurs in those who 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>
==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
*Children with germline mutation of RB1 gene are at elevated risk of secondary malignant tumors.<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 most common secondary tumors include:
**Sarcoma
**Melanoma
**Brain tumors
*The estimated risk of developing a secondary tumor is 20 times greater than the general population.
===Complications of retinoblastoma therapy===
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Medicine]]
[[Category:Medicine]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date]]
[[Category:Primary care]]
[[Category:Surgery]]
[[Category:Surgery]]

Latest revision as of 23:59, 29 July 2020

Retinoblastoma Microchapters

Home

Patient Information

Overview

Historical perspective

Classification

Pathophysiology

Causes

Differentiating Retinoblastoma from other Diseases

Epidemiology & Demographics

Risk Factors

Screening

Natural history, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History & Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Retinoblastoma natural history, complications, and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Retinoblastoma natural history, complications, and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Retinoblastoma natural history, complications, and prognosis

CDC on Retinoblastoma natural history, complications, and prognosis

Retinoblastoma natural history, complications, and prognosis in the news

Blogs on Retinoblastoma natural history, complications, and prognosis

Directions to Hospitals Treating Retinoblastoma

Risk calculators and risk factors for Retinoblastoma natural history, complications, and prognosis

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.
Retrieved from "https://www.wikidoc.org/index.php?title=Retinoblastoma_natural_history,_complications,_and_prognosis&oldid=1642857"