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==Overview==
==Overview==
A common method of retinoblastoma [[classification]] is critical to plan treatment, evaluate [[prognosis]] and compare outcomes. Available grouping systems include the International Intraocular [[Retinoblastoma]] [[Classification]] (IIRC), Intraocular [[Classification]] of Retinoblastoma (ICRB) and [[TNM|cTNMH systems]] [[diseases]].
[[Ultrasound imaging]] is the [[Gold standard (test)|gold standard test]] for the [[diagnosis]] of retinoblastoma. [[Magnetic resonance imaging|MRI]] can also be helpful in the [[diagnosis]] making. A common method of retinoblastoma [[classification]] is critical to plan treatment, evaluate [[prognosis]] and compare outcomes. Available grouping systems include the International Intraocular [[Retinoblastoma]] [[Classification]] (IIRC), Intraocular [[Classification]] of Retinoblastoma (ICRB) and [[TNM|cTNMH systems]] [[diseases]].
==Diagnostic Study of Choice==
==Diagnostic Study of Choice==
===Study of Choice===
===Study of Choice===


===Staging===
* [[Ultrasound imaging]] is the [[Gold standard (test)|gold standard test]] for the [[diagnosis]] of retinoblastoma.  
A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. There are several [[classification]] system available for [[retinoblastoma]].. For intraocular [[diseases]] the available grouping systems include the International Intraocular [[Retinoblastoma]] [[Classification]] (IIRC), Intraocular [[Classification]] of Retinoblastoma (ICRB) and [[TNM|cTNMH systems]]. For extraocular [[diseases]], the International [[Retinoblastoma]] [[Cancer staging|Staging]] System (IRSS) and [[TNM Staging System|cTNMH]] schemes can be used.
* [[Ultrasound]] biomicroscopy (UBM) has been observed to have 81% [[sensitivity]] and 100% [[specificity]] in the evaluation of the anterior extension of [[tumor]] and assessment of [[posterior chamber]] involvement.<ref name="MoulinGaillard2012">{{cite journal|last1=Moulin|first1=Alexandre P|last2=Gaillard|first2=Marie-Claire|last3=Balmer|first3=Aubin|last4=Munier|first4=Francis L|title=Ultrasound biomicroscopy evaluation of anterior extension in retinoblastoma: a clinicopathological study|journal=British Journal of Ophthalmology|volume=96|issue=3|year=2012|pages=337–340|issn=0007-1161|doi=10.1136/bjophthalmol-2011-300051}}</ref>
Retinoblastoma is classified in two subgroups:<br>
* [[MRI]] is recommended for the following goals:<ref name="SyedRamasubramanian2017">{{cite journal|last1=Syed|first1=Reema|last2=Ramasubramanian|first2=Aparna|title=Advances in Management of Retinoblastoma|journal=Advances in Ophthalmology and Optometry|volume=2|issue=1|year=2017|pages=87–100|issn=24521760|doi=10.1016/j.yaoo.2017.03.005}}</ref>
'''Extraocular disease'''  
 
*This category first subclassified via International Retinoblastoma Staging System different staging system to 0–IV.<ref name="pmid16358310">{{cite journal |vauthors=Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodríguez-Galindo C, Schvartzman E, Popovic MB, Kremens B, Meadows AT, Zucker JM |title=A proposal for an international retinoblastoma staging system |journal=Pediatr Blood Cancer |volume=47 |issue=6 |pages=801–5 |date=November 2006 |pmid=16358310 |doi=10.1002/pbc.20606 |url=}}</ref>
**Confirmation of the [[diagnosis]]
**This sub-classification is established by Chantada and colleagues in 2006.
**Evaluation of local [[tumor]] extent
***This staging system is used in the [[clinical]] setting greater than the AJCC staging system (Please see below).
**Assessment of associated developmental [[malformation]] of the [[brain]]
;International Retinoblastoma Staging System  
**Detection of trilateral retinoblastoma (pinealoblastoma)
 
=== Staging ===
'''Extraocular Disease'''  
*This category is sub-classified via International Retinoblastoma Staging System which is given below:<ref name="pmid16358310">{{cite journal |vauthors=Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodríguez-Galindo C, Schvartzman E, Popovic MB, Kremens B, Meadows AT, Zucker JM |title=A proposal for an international retinoblastoma staging system |journal=Pediatr Blood Cancer |volume=47 |issue=6 |pages=801–5 |date=November 2006 |pmid=16358310 |doi=10.1002/pbc.20606 |url=}}</ref>
 
'''International Retinoblastoma Staging System'''
{|
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Stage
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Stage
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Description
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Description'''
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage 0
! style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage 0
| colspan="6" style="background: #F5F5F5; padding: 5px;" |Eye enucleated and no dissemination of the [[disease]]
| colspan="6" style="background: #F5F5F5; padding: 5px;" |
* [[Eye]] enucleated and no dissemination of the [[disease]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage I
! style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage I
| colspan="6" style="background: #F5F5F5; padding: 5px;" |Eye enucleated, completely resected [[Histology|histologically]]
| colspan="6" style="background: #F5F5F5; padding: 5px;" |
* [[Eye]] enucleated, completely [[Resection|resected]] [[Histology|histologically]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage II
! style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage II
| colspan="6" style="background: #F5F5F5; padding: 5px;" |Eye enucleated, [[microscopic]] residual [[tumor]]
| colspan="6" style="background: #F5F5F5; padding: 5px;" |
* [[Eye]] enucleated, [[microscopic]] residual [[tumor]]
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage III
! style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage III
| colspan="3" style="background: #F5F5F5; padding: 5px;" |Regional extension
| colspan="3" style="background: #F5F5F5; padding: 5px;" |
| colspan="3" style="background: #F5F5F5; padding: 5px;" |a.Overt [[orbital disease]]
* Regional extension
| colspan="3" style="background: #F5F5F5; padding: 5px;" |'''a.''' Overt [[orbital disease]]


b.Preauricular or cervical [[lymph node]] extension
'''b.''' Preauricular or [[Cervical lymph nodes|cervical lymph node]] extension
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage IV
! style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage IV
| colspan="3" style="background: #F5F5F5; padding: 5px;" |Metastatic disease
| colspan="3" style="background: #F5F5F5; padding: 5px;" |
| colspan="3" style="background: #F5F5F5; padding: 5px;" |a.Hematogenous [[metastasis]] (without [[CNS]] involvement)
* [[Metastatic disease]]
| colspan="3" style="background: #F5F5F5; padding: 5px;" |'''a.''' Hematogenous [[metastasis]] (without [[CNS]] involvement)


—Single [[lesion]]
* Single [[lesion]]
* Multiple [[lesions]]


—Multiple [[lesions]]
'''b.''' [[CNS]] extension (with or without any other site of regional or [[metastatic disease]])


b.[[CNS]] extension (with or without any other site of regional or metastatic disease)
* Prechiasmatic [[lesion]]
* [[CNS]] mass
* [[Leptomeningeal]] and [[CSF]] [[disease]]
|}


—Prechiasmatic lesion


—[[CNS]] mass
'''Intraocular Disease'''


—Leptomeningeal and [[CSF]] [[disease]]
*The intraocular [[disease]] can be sub-[[Classification|classified]] by International Intraocular Retinoblastoma Classification (IIRC).<ref name="pmid15763190">{{cite journal |vauthors=Linn Murphree A |title=Intraocular retinoblastoma: the case for a new group classification |journal=Ophthalmol Clin North Am |volume=18 |issue=1 |pages=41–53, viii |date=March 2005 |pmid=15763190 |doi=10.1016/j.ohc.2004.11.003 |url=}}</ref>
|}


*'''Intraocular disease''' which first classified by Reese-Ellsworth Classification method in the 1960s and used to predict the [[Survival rate|survival]] chance of salvaging eye following [[external beam radiotherapy]].
*IIRC grouping system sub-[[Classification|classifies]] the [[tumor]] based on the [[tumor]] characteristics, such as:
**This classification replaced by International Intraocular Retinoblastoma Classification (IIRC) scheme following the introduction of [[chemotherapy]] for the treatment of retinoblastoma in 1990.<ref name="pmid15763190">{{cite journal |vauthors=Linn Murphree A |title=Intraocular retinoblastoma: the case for a new group classification |journal=Ophthalmol Clin North Am |volume=18 |issue=1 |pages=41–53, viii |date=March 2005 |pmid=15763190 |doi=10.1016/j.ohc.2004.11.003 |url=}}</ref>
*IIRC grouping system sub-classifies the [[tumor]] according to from A to E according to [[tumor]] characteristics such as:
**Size
**Size
**Location
**Location
**Presence of retinoblastoma ‘seeds’ (small colonies of cancerous cells in the [[vitreous]])
**Presence of retinoblastoma ‘seeds’ (small colonies of [[Cancer cell|cancerous cells]] in the [[vitreous]])
**[[Retinal detachment]]
**[[Retinal detachment]]
In 2006, Intraocular Classification of Retinoblastoma (ICRB), has been developed by Dr. Shields and colleagues.<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>
*This classification is modified version of IIRC and the main difference is in advanced categories of the [[tumor]] (D and E).
*This classification method has been observed to be successful at predicting the outcome of [[chemotherapy]].
*IIRC and ICRB are now the most commonly used classification system for retinoblastoma.<ref name="pmid28730089">{{cite journal |vauthors=Scelfo C, Francis JH, Khetan V, Jenkins T, Marr B, Abramson DH, Shields CL, Pe'er J, Munier F, Berry J, Harbour JW, Yarovoy A, Lucena E, Murray TG, Bhagia P, Paysse E, Tuncer S, Chantada GL, Moll AC, Ushakova T, Plager DA, Ziyovuddin I, Leal CA, Materin MA, Ji XD, Cursino JW, Polania R, Kiratli H, All-Ericsson C, Kebudi R, Honavar SG, Vishnevskia-Dai V, Epelman S, Daniels AB, Ling JD, Traore F, Ramirez-Ortiz MA |title=An international survey of classification and treatment choices for group D retinoblastoma |journal=Int J Ophthalmol |volume=10 |issue=6 |pages=961–967 |date=2017 |pmid=28730089 |pmc=5515152 |doi=10.18240/ijo.2017.06.20 |url=}}</ref>
*American Joint Committee on Cancer (AJCC) staging system is another suggested grouping system for the classification of the [[tumor]] using [[TNM system]].
**This system is not frequently used for [[pediatrics]] and is not grouped according to the [[prognostic]] values.


;American Joint Committee on Cancer (AJCC) staging system
* In 2006, Intraocular Classification of Retinoblastoma (ICRB) was developed by Dr. Shields and colleagues.<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>
*IIRC and ICRB are now the most commonly used [[classification]] systems for retinoblastoma.<ref name="pmid28730089">{{cite journal |vauthors=Scelfo C, Francis JH, Khetan V, Jenkins T, Marr B, Abramson DH, Shields CL, Pe'er J, Munier F, Berry J, Harbour JW, Yarovoy A, Lucena E, Murray TG, Bhagia P, Paysse E, Tuncer S, Chantada GL, Moll AC, Ushakova T, Plager DA, Ziyovuddin I, Leal CA, Materin MA, Ji XD, Cursino JW, Polania R, Kiratli H, All-Ericsson C, Kebudi R, Honavar SG, Vishnevskia-Dai V, Epelman S, Daniels AB, Ling JD, Traore F, Ramirez-Ortiz MA |title=An international survey of classification and treatment choices for group D retinoblastoma |journal=Int J Ophthalmol |volume=10 |issue=6 |pages=961–967 |date=2017 |pmid=28730089 |pmc=5515152 |doi=10.18240/ijo.2017.06.20 |url=}}</ref>
 
;
;
;
;
;American Joint Committee on Cancer (AJCC) Staging System
;


{|
{|
! colspan="2" style="background:#DCDCDC;" align="center" + |The table below adopted from Community Eye Health Journal <ref name="pmid29915471">{{cite journal |vauthors= |title=TNM8: The updated TNM classification for retinoblastoma |journal=Community Eye Health |volume=31 |issue=101 |pages=34 |date=2018 |pmid=29915471 |pmc=5998398 |doi= |url=}}</ref>
! colspan="2" style="background:#DCDCDC;" align="center" + |The table below is adopted from Community Eye Health Journal <ref name="pmid29915471">{{cite journal |vauthors= |title=TNM8: The updated TNM classification for retinoblastoma |journal=Community Eye Health |volume=31 |issue=101 |pages=34 |date=2018 |pmid=29915471 |pmc=5998398 |doi= |url=}}</ref>
|-  
|-  
|}
|}
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
|-
|-
| |cTX
! cTX
| ||Unknown evidence of intraocular [[tumor]]
! |
|
* Unknown evidence of intraocular [[tumor]]
|-
|-
|  cT0
! cT0
| ||No evidence of intraocular [[tumor]]
!  |
|
* No evidence of intraocular [[tumor]]
|-
|-
| cT1
! cT1
| a||Intraocular [[tumor]](s) with sub-retinal fluid ≤ 5mm from the base of any [[tumor]]
! a
[[Tumors]] ≤ 3mm and further than 1.5 mm from the [[Optic disc|disc]] and [[fovea]]
|
* Intraocular [[tumor]](s) with sub-retinal fluid ≤ 5mm from the base of any [[tumor]]
 
* [[Tumors]] ≤ 3mm and further than 1.5 mm from the [[Optic disc|disc]] and [[fovea]]
|-
|-
|cT1
!cT1
| b||Intraocular [[tumor]](s) with sub-retinal fluid ≤ 5mm from the base of any [[tumor]]
! b
[[Tumor|Tumors]] > 3 mm or closer than 1.5 mm to the [[Optic disc|disc]] and [[fovea]]
|
* Intraocular [[tumor]](s) with sub-retinal fluid ≤ 5mm from the base of any [[tumor]]
 
* [[Tumor|Tumors]] > 3 mm or closer than 1.5 mm to the [[Optic disc|disc]] and [[fovea]]
|-
|-
| cT2
! cT2
| a||Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding
! a
Sub-retinal fluid > 5 mm from the base of any [[tumor]]
|
* Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding
 
* Sub-retinal fluid > 5 mm from the base of any [[tumor]]
|-
|-
| cT2
! cT2
| b||Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding
! b
[[Tumors]] with [[vitreous]] seeding and/or sub-retinal seeding
|
* Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding
 
* [[Tumors]] with [[vitreous]] seeding and/or sub-retinal seeding
|-
|-
| cT3
! cT3
| a||Advanced intraocular [[tumor]](s)
! a
[[Phthisis bulbi|Phthisis]] or pre-[[phthisis bulbi]]
|
* Advanced intraocular [[tumor]](s)
 
* [[Phthisis bulbi|Phthisis]] or pre-[[phthisis bulbi]]
|-
|-
| cT3
! cT3
| b||Advanced intraocular [[tumor]](s)
! b
[[Tumor]] invasion of the [[pars plana]], [[ciliary body]], [[lens]], [[zonules]], [[iris]] or [[Anterior chamber of eyeball|anterior chamber]]
|
* Advanced intraocular [[tumor]](s)
 
* [[Tumor]] invasion of the [[pars plana]], [[ciliary body]], [[lens]], [[zonules]], [[iris]] or [[Anterior chamber of eyeball|anterior chamber]]
|-
|-
| cT3
! cT3
| c||Advanced intraocular [[tumor]](s)
! c
Raised intraocular pressure with [[neovascularization]] and/or [[buphthalmos]]
|
* Advanced intraocular [[tumor]](s)
 
* Raised [[intraocular pressure]] with [[neovascularization]] and/or [[buphthalmos]]
|-
|-
| cT3
! cT3
| d||Advanced intraocular [[tumor]](s)
! d
[[Hyphema]] and/or massive [[vitreous hemorrhage]]
|
* Advanced intraocular [[tumor]](s)
 
* [[Hyphema]] and/or massive [[vitreous hemorrhage]]
|-
|-
| cT3
! cT3
| e||Advanced intraocular [[tumor]](s)
! e
[[Aseptic]] [[orbital cellulitis]]
|
* Advanced intraocular [[tumor]](s)
 
* [[Aseptic]] [[orbital cellulitis]]
|-
|-
| cT4
! cT4
| a||Extraocular [[tumor]](s) involving the orbit, including the [[optic nerve]]
! a
[[Radiological]] evidence of retrobulbar [[optic nerve]] involvement or thickening of the [[optic nerve]] or involvement of the [[Orbit (anatomy)|orbital]] tissues
|
* Extraocular [[tumor]](s) involving the [[Orbit (anatomy)|orbit]], including the [[optic nerve]]
 
* [[Radiological]] evidence of retrobulbar [[optic nerve]] involvement or thickening of the [[optic nerve]] or involvement of the [[Orbit (anatomy)|orbital]] tissues
|-
|-
| cT4
! cT4
| b||Extraocular [[tumor]](s) involving the [[orbit]], including the [[optic nerve]]
! b
Extraocular [[tumor]] clinically evident with [[Exophthalmos|proptosis]] and [[Orbital mass causes|orbital mass]]
|
* Extraocular [[tumor]](s) involving the [[orbit]], including the [[optic nerve]]
 
* Extraocular [[tumor]] clinically evident with [[Exophthalmos|proptosis]] and [[Orbital mass causes|orbital mass]]
|-
|-
|+
|+
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
|-
|-
| cNX
! cNX
| ||Regional [[lymph nodes]] cannot be assessed
!  |
|
* Regional [[lymph nodes]] cannot be assessed
|-
|-
| cN0
! cN0
| ||No regional [[Lymph node metastases|lymph node involvement]]
!  |
|
* No regional [[Lymph node metastases|lymph node involvement]]
|-
|-
| cN1
! cN1
| ||Evidence of preauricular, [[submandibular]]<nowiki/>, and [[cervical]] [[Lymph node metastases|lymph node involvement]]
!  |
|
* Evidence of preauricular, [[submandibular]]<nowiki/>, and [[cervical]] [[Lymph node metastases|lymph node involvement]]
|-
|-
|+
|+
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
|-
|-
| cM0
! cM0
| ||No [[signs]] or [[symptoms]] of intracranial or distant [[metastasis]]
!  |
|
* No [[signs]] or [[symptoms]] of intracranial or distant [[metastasis]]
|-
|-
| cM1
! cM1
| a||Distant [[metastasis]] without [[microscopic]] confirmation
! a
[[Tumor]](s) involving any distant site (e.g. [[bone marrow]], [[liver]]) on [[clinical]] or [[Imaging studies|radiological tests]]
|
* Distant [[metastasis]] without [[microscopic]] confirmation
 
* [[Tumor]](s) involving any distant site (e.g. [[bone marrow]], [[liver]]) on [[clinical]] or [[Imaging studies|radiological tests]]
|-
|-
| cM1
! cM1
| b||Distant [[metastasis]] without [[microscopic]] confirmation
! b
Tumour involving the central nervous system on radiological imaging (not including trilateral retinoblastoma)
|
* Distant [[metastasis]] without [[microscopic]] confirmation
 
* [[Tumor]] involving the central nervous system on radiological imaging (not including trilateral retinoblastoma)
|-
|-
| pM1
! pM1
| a||Distant [[metastasis]] with [[microscopic]] confirmation
! a
[[Histopathological]] confirmation of [[tumor]] at any distant site (e.g. [[bone marrow]], [[liver]], or other)
|
* Distant [[metastasis]] with [[microscopic]] confirmation
 
* [[Histopathological]] confirmation of [[tumor]] at any distant site (e.g. [[bone marrow]], [[liver]], or other)
|-
|-
|pM1
!pM1
|b
!b
|Distant [[metastasis]] with [[microscopic]] confirmation
|
* Distant [[metastasis]] with [[microscopic]] confirmation


[[Histopathological]] confirmation of [[tumor]] in the [[cerebrospinal fluid]] or [[CNS]] [[parenchyma]]
* [[Histopathological]] confirmation of [[tumor]] in the [[cerebrospinal fluid]] or [[CNS]] [[parenchyma]]
|-
|-
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Heritable trait (H)
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Heritable trait (H)
Line 183: Line 251:
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding'''
|-
|-
| HX
! HX
| ||Unknown or insufficient evidence of a constitutional [[RB1]] [[gene]] [[mutation]]
!  |
|
* Unknown or insufficient evidence of a constitutional [[RB1]] [[gene]] [[mutation]]
|-
|-
|H0
!H0
!
|
|
|Normal [[RB1]] alleles in blood tested with demonstrated high [[sensitivity]] assays
* Normal [[RB1]] [[Allele|alleles]] in [[blood]] tested with demonstrated high [[sensitivity]] [[Assay|assays]]
|-
|-
|H1
!H1
!
|
|
|Bilateral retinoblastoma, retinoblastoma with an intracranial [[CNS]] midline [[embryonic]] [[tumor]] (i.e. trilateral retinoblastoma), patient with a [[family history]] of retinoblastoma, or molecular definition of constitutional [[RB1]] [[gene]] [[mutation]]
* Bilateral retinoblastoma, retinoblastoma with an intracranial [[CNS]] midline [[embryonic]] [[tumor]] (i.e. trilateral retinoblastoma), patient with a [[family history]] of retinoblastoma, or molecular definition of constitutional [[RB1]] [[gene]] [[mutation]]
|-
|-
|-
|-
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==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

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

Overview

Ultrasound imaging is the gold standard test for the diagnosis of retinoblastoma. MRI can also be helpful in the diagnosis making. A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. Available grouping systems include the International Intraocular Retinoblastoma Classification (IIRC), Intraocular Classification of Retinoblastoma (ICRB) and cTNMH systems diseases.

Diagnostic Study of Choice

Study of Choice

    • Confirmation of the diagnosis
    • Evaluation of local tumor extent
    • Assessment of associated developmental malformation of the brain
    • Detection of trilateral retinoblastoma (pinealoblastoma)

Staging

Extraocular Disease

  • This category is sub-classified via International Retinoblastoma Staging System which is given below:[3]

International Retinoblastoma Staging System

Stage Description
Stage 0
  • Eye enucleated and no dissemination of the disease
Stage I
Stage II
Stage III
  • Regional extension
 a. Overt orbital disease

b. Preauricular or cervical lymph node extension

Stage IV a. Hematogenous metastasis (without CNS involvement)

b. CNS extension (with or without any other site of regional or metastatic disease)


Intraocular Disease

  • The intraocular disease can be sub-classified by International Intraocular Retinoblastoma Classification (IIRC).[4]
  • In 2006, Intraocular Classification of Retinoblastoma (ICRB) was developed by Dr. Shields and colleagues.[5]
  • IIRC and ICRB are now the most commonly used classification systems for retinoblastoma.[6]
American Joint Committee on Cancer (AJCC) Staging System
The table below is adopted from Community Eye Health Journal [7]
Primary tumour (cT)*
Stage Sub-stage Finding
cTX
  • Unknown evidence of intraocular tumor
cT0
  • No evidence of intraocular tumor
cT1 a
  • Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor
cT1 b
  • Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor
cT2 a
  • Sub-retinal fluid > 5 mm from the base of any tumor
cT2 b
cT3 a
  • Advanced intraocular tumor(s)
cT3 b
  • Advanced intraocular tumor(s)
cT3 c
  • Advanced intraocular tumor(s)
cT3 d
  • Advanced intraocular tumor(s)
cT3 e
  • Advanced intraocular tumor(s)
cT4 a
cT4 b
Regional lymph nodes (cN)
Stage Sub-stage Finding
cNX
cN0
cN1
Distant metastasis
Stage Sub-stage Finding
cM0
cM1 a
cM1 b
  • Tumor involving the central nervous system on radiological imaging (not including trilateral retinoblastoma)
pM1 a
pM1 b
Heritable trait (H)
Stage Sub-stage Finding
HX
H0
H1
  • Bilateral retinoblastoma, retinoblastoma with an intracranial CNS midline embryonic tumor (i.e. trilateral retinoblastoma), patient with a family history of retinoblastoma, or molecular definition of constitutional RB1 gene mutation

References

  1. ↑ Moulin, Alexandre P; Gaillard, Marie-Claire; Balmer, Aubin; Munier, Francis L (2012). "Ultrasound biomicroscopy evaluation of anterior extension in retinoblastoma: a clinicopathological study". British Journal of Ophthalmology. 96 (3): 337–340. doi:10.1136/bjophthalmol-2011-300051. ISSN 0007-1161.
  2. ↑ Syed, Reema; Ramasubramanian, Aparna (2017). "Advances in Management of Retinoblastoma". Advances in Ophthalmology and Optometry. 2 (1): 87–100. doi:10.1016/j.yaoo.2017.03.005. ISSN 2452-1760.
  3. ↑ Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodríguez-Galindo C, Schvartzman E, Popovic MB, Kremens B, Meadows AT, Zucker JM (November 2006). "A proposal for an international retinoblastoma staging system". Pediatr Blood Cancer. 47 (6): 801–5. doi:10.1002/pbc.20606. PMID 16358310.
  4. ↑ Linn Murphree A (March 2005). "Intraocular retinoblastoma: the case for a new group classification". Ophthalmol Clin North Am. 18 (1): 41–53, viii. doi:10.1016/j.ohc.2004.11.003. PMID 15763190.
  5. ↑ 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.
  6. ↑ Scelfo C, Francis JH, Khetan V, Jenkins T, Marr B, Abramson DH, Shields CL, Pe'er J, Munier F, Berry J, Harbour JW, Yarovoy A, Lucena E, Murray TG, Bhagia P, Paysse E, Tuncer S, Chantada GL, Moll AC, Ushakova T, Plager DA, Ziyovuddin I, Leal CA, Materin MA, Ji XD, Cursino JW, Polania R, Kiratli H, All-Ericsson C, Kebudi R, Honavar SG, Vishnevskia-Dai V, Epelman S, Daniels AB, Ling JD, Traore F, Ramirez-Ortiz MA (2017). "An international survey of classification and treatment choices for group D retinoblastoma". Int J Ophthalmol. 10 (6): 961–967. doi:10.18240/ijo.2017.06.20. PMC 5515152. PMID 28730089.
  7. ↑ "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.

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