Retinoblastoma classification: Difference between revisions

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__NOTOC__
__NOTOC__
{{Retinoblastoma}}
{{Retinoblastoma}}
{{CMG}}{{AE}}{{Simrat}}
{{CMG}}; {{AE}} {{Sahar}} {{Simrat}}
==Overview==
==Overview==
Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).<ref name="pmid10388023">{{cite journal| author=Pratt CB, Fontanesi J, Lu X, Parham DM, Elfervig J, Meyer D| title=Proposal for a New Staging Scheme for Intraocular and Extraocular Retinoblastoma Based on an Analysis of 103 Globes. | journal=Oncologist | year= 1997 | volume= 2 | issue= 1 | pages= 1-5 | pmid=10388023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10388023  }} </ref> <ref name="pmid15136321">{{cite journal| author=Shields CL, Mashayekhi A, Demirci H, Meadows AT, Shields JA| title=Practical approach to management of retinoblastoma. | journal=Arch Ophthalmol | year= 2004 | volume= 122 | issue= 5 | pages= 729-35 | pmid=15136321 | doi=10.1001/archopht.122.5.729 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15136321  }} </ref><ref name="pmid15763190">{{cite journal| author=Linn Murphree A| title=Intraocular retinoblastoma: the case for a new group classification. | journal=Ophthalmol Clin North Am | year= 2005 | volume= 18 | issue= 1 | pages= 41-53, viii | pmid=15763190 | doi=10.1016/j.ohc.2004.11.003 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15763190  }} </ref>  
Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).<ref name="pmid10388023">{{cite journal| author=Pratt CB, Fontanesi J, Lu X, Parham DM, Elfervig J, Meyer D| title=Proposal for a New Staging Scheme for Intraocular and Extraocular Retinoblastoma Based on an Analysis of 103 Globes. | journal=Oncologist | year= 1997 | volume= 2 | issue= 1 | pages= 1-5 | pmid=10388023 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10388023  }} </ref> <ref name="pmid15136321">{{cite journal| author=Shields CL, Mashayekhi A, Demirci H, Meadows AT, Shields JA| title=Practical approach to management of retinoblastoma. | journal=Arch Ophthalmol | year= 2004 | volume= 122 | issue= 5 | pages= 729-35 | pmid=15136321 | doi=10.1001/archopht.122.5.729 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15136321  }} </ref><ref name="pmid15763190">{{cite journal| author=Linn Murphree A| title=Intraocular retinoblastoma: the case for a new group classification. | journal=Ophthalmol Clin North Am | year= 2005 | volume= 18 | issue= 1 | pages= 41-53, viii | pmid=15763190 | doi=10.1016/j.ohc.2004.11.003 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15763190  }} </ref>  
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For treatment purposes, retinoblastoma is classified to:
For treatment purposes, retinoblastoma is classified to:
*'''Intra-ocular retinoblastoma''' classification system includes:
*'''Intra-ocular retinoblastoma''' classification system includes:
**International Intraocular Retinoblastoma Classification (IIRC)
**International Intraocular Retinoblastoma Classification (IIRC)<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><ref name="pmid17729249">{{cite journal |vauthors=Zage PE, Reitman AJ, Seshadri R, Weinstein JL, Mets MB, Zeid JL, Greenwald MJ, Strauss LC, Goldman S |title=Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma |journal=Pediatr Blood Cancer |volume=50 |issue=3 |pages=567–72 |date=March 2008 |pmid=17729249 |doi=10.1002/pbc.21301 |url=}}</ref><ref name="pmid19172510">{{cite journal |vauthors=Novetsky DE, Abramson DH, Kim JW, Dunkel IJ |title=Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies--an analysis of impact |journal=Ophthalmic Genet. |volume=30 |issue=1 |pages=40–4 |date=March 2009 |pmid=19172510 |doi=10.1080/13816810802452168 |url=}}</ref>
**Intraocular Classification of Retinoblastoma (ICRB)
**Intraocular Classification of Retinoblastoma (ICRB)
**The cTNMH system of American Joint Committee on Cancer (AJCC)
**The cTNMH system of American Joint Committee on Cancer (AJCC)
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*This classification system was first introduced in 1960 and used to predict the chance of eye salvage after external beam radiotherapy. However, following the introduction of chemotherapy for retinoblastoma treatment, it lost its significance.
*This classification system was first introduced in 1960 and used to predict the chance of eye salvage after external beam radiotherapy. However, following the introduction of chemotherapy for retinoblastoma treatment, it lost its significance.


 
{| border="3"
;The International Classification of Retinoblastoma staging system <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><ref name="pmid17729249">{{cite journal |vauthors=Zage PE, Reitman AJ, Seshadri R, Weinstein JL, Mets MB, Zeid JL, Greenwald MJ, Strauss LC, Goldman S |title=Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma |journal=Pediatr Blood Cancer |volume=50 |issue=3 |pages=567–72 |date=March 2008 |pmid=17729249 |doi=10.1002/pbc.21301 |url=}}</ref><ref name="pmid19172510">{{cite journal |vauthors=Novetsky DE, Abramson DH, Kim JW, Dunkel IJ |title=Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies--an analysis of impact |journal=Ophthalmic Genet. |volume=30 |issue=1 |pages=40–4 |date=March 2009 |pmid=19172510 |doi=10.1080/13816810802452168 |url=}}</ref>
|+ Intraocular classifications of retinoblastoma and their features
{| style="border: 0px; font-size: 90%; margin: 3px; width: 800px" align=center
!  !! International Intraocular Retinoblastoma Classification (IIRC) !! Intraocular Classification of Retinoblastoma (ICRB)
|valign=top|
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Groups}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Features}}
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
! Group A
:Group A
(very low
| style="padding: 5px 5px; background: #F5F5F5;" |
risk)
*Small intraretinal tumors away from foveola and optic nerve
|Small intraretinal tumors away from foveola and optic nerve<br>3mm or smaller in the greatest dimension, confined to the retina<br>Located further than 3 mm from the foveola and 1.5 mm from the optic disc
**3mm or smaller in the greatest dimension, confined to retina
| Tumors ≤ 3 mm (in basal dimension or thickness)
**Located further than 3 mm from the foveola and 1.5 mm from the optic disc.
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
!Group B
:Group B
(low risk)
| style="padding: 5px 5px; background: #F5F5F5;" |
|Tumors confined to the retina<br>Not in the group A<br>Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
*Tumors confined to the retina.
|Tumors > 3 mm (in basal dimension or thickness) or<br>Macular location (≤ 3 mm to foveola)<br>Juxtapapillary location (≤ 1.5 mm to disc)<br>Additional subretinal fluid (≤3 mm from margin)
**Not in the group A
**Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-All remaining discrete tumors confined to the retina.weight: bold" |
! Group C
:Group C
(moderate
| style="padding: 5px 5px; background: #F5F5F5;" |
risk)
*Local disease with minimal subretinal or vitreous seeding with following caracteristics:
|Local disease with minimal subretinal or vitreous seeding with following characteristics:<br>Discrete<br>Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina<br>Local fine vitreous seeding may be present close to the discrete tumor<br>Local subretinal seeding less than 3 mm (2 DD) from the tumor
**Discrete
|Tumor with:<br>Subretinal seeds ≤ 3 mm from tumor<br>Vitreous seeds ≤ 3 mm from tumor<br>Both subretinal and vitreous seeds ≤ 3 mm from retinoblastoma
**Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina
**Local fine vitreous seeding may be present close to the discrete tumor
**Local subretinal seeding less than 3 mm (2 DD) from the tumor
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
!Group D
:Group D
(high risk)
| style="padding: 5px 5px; background: #F5F5F5;" |
|Diffuse tumor with significant vitreous or subretinal seeding<br>Maybe massive or diffuse<br>Subretinal fluid present or past without seeding, involving up to total retinal detachment<br>The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses<br>Diffuse subretinal seeding may include subretinal plaques or tumor nodules
*Diffuse tumor with significant vitreous or subretinal seeding
|Tumor with:<br>Subretinal seeds > 3 mm from tumor<br>Vitreous seeds > 3 mm from tumor<br>Both subretinal and vitreous seeds > 3 mm from retinoblastoma
**May be massive or diffuse
**Subretinal fluid present or past without seeding, involving up to total retinal detachment
**The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses
**Diffuse subretinal seeding may include subretinal plaques or tumor nodules
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
!Group E
:Group E
(very high
| style="padding: 5px 5px; background: #F5F5F5;" |
risk)
*Presence of any one or more of the following poor prognosis features
|Presence of any one or more of the following poor prognosis features<br>Tumor touching the lens<br>Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment<br>Diffuse infiltrating retinoblastoma<br>Neovascular glaucoma<br>Opaque media from hemorrhage<br>Tumor necrosis with aseptic orbital cellulitis<br>Phthisis bulbi
**Tumor touching the lens
|Extensive tumor filling >50% globe or with<br>Neovascular glaucoma<br>Opaque media from hemorrhage in the anterior chamber, vitreous or subretinal space<br>Invasion of the post-laminar optic nerve<br>choroid (>2 mm), sclera, orbit, anterior chamber
**Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment
**Diffuse infiltrating retinoblastoma
**Neovascular glaucoma
**Opaque media from hemorrhage
**Tumor necrosis with aseptic orbital cellulitis
**Phthisis bulbi
|}
|}


==References==
==References==

Revision as of 15:27, 29 April 2019

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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

Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).[1] [2][3]

Classification

Retinoblastoma has been classified according to different classification system for variable purpuses.[4]
Each classification system has been developed depending on the change in the management of tumor.
For treatment purposes, retinoblastoma is classified to:

  • Intra-ocular retinoblastoma classification system includes:
    • International Intraocular Retinoblastoma Classification (IIRC)[5][6][7]
    • Intraocular Classification of Retinoblastoma (ICRB)
    • The cTNMH system of American Joint Committee on Cancer (AJCC)
  • Extra-ocular retinoblastoma classification system are such as:
    • International retinoblastoma staging system
    • cTNMH system of American Joint Committee on Cancer (AJCC)

the cTNMH system has been developed by the American Joint Committee on Cancer (AJCC).[8]

  • The tumor classified according to the size of the primary tumor (T), lymph node involvement (N) and presence of systemic metastasis (M).
  • The recently edited guideline added H for retinoblastoma as the hereditary component for Rb gene.
  • This category also includes a pathological subgroup (pTNM) which is frequently used by the ophthalmic pathologist.
  • To see the full stageing systm click here.

Reese-Ellsworth Classification for Intraocular Tumors is another staging system for retinoblastoma.[3]

  • This classification system was first introduced in 1960 and used to predict the chance of eye salvage after external beam radiotherapy. However, following the introduction of chemotherapy for retinoblastoma treatment, it lost its significance.
Intraocular classifications of retinoblastoma and their features
International Intraocular Retinoblastoma Classification (IIRC) Intraocular Classification of Retinoblastoma (ICRB)
Group A

(very low risk)

Small intraretinal tumors away from foveola and optic nerve
3mm or smaller in the greatest dimension, confined to the retina
Located further than 3 mm from the foveola and 1.5 mm from the optic disc
Tumors ≤ 3 mm (in basal dimension or thickness)
Group B

(low risk)

Tumors confined to the retina
Not in the group A
Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
Tumors > 3 mm (in basal dimension or thickness) or
Macular location (≤ 3 mm to foveola)
Juxtapapillary location (≤ 1.5 mm to disc)
Additional subretinal fluid (≤3 mm from margin)
Group C

(moderate risk)

Local disease with minimal subretinal or vitreous seeding with following characteristics:
Discrete
Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina
Local fine vitreous seeding may be present close to the discrete tumor
Local subretinal seeding less than 3 mm (2 DD) from the tumor
Tumor with:
Subretinal seeds ≤ 3 mm from tumor
Vitreous seeds ≤ 3 mm from tumor
Both subretinal and vitreous seeds ≤ 3 mm from retinoblastoma
Group D

(high risk)

Diffuse tumor with significant vitreous or subretinal seeding
Maybe massive or diffuse
Subretinal fluid present or past without seeding, involving up to total retinal detachment
The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses
Diffuse subretinal seeding may include subretinal plaques or tumor nodules
Tumor with:
Subretinal seeds > 3 mm from tumor
Vitreous seeds > 3 mm from tumor
Both subretinal and vitreous seeds > 3 mm from retinoblastoma
Group E

(very high risk)

Presence of any one or more of the following poor prognosis features
Tumor touching the lens
Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment
Diffuse infiltrating retinoblastoma
Neovascular glaucoma
Opaque media from hemorrhage
Tumor necrosis with aseptic orbital cellulitis
Phthisis bulbi
Extensive tumor filling >50% globe or with
Neovascular glaucoma
Opaque media from hemorrhage in the anterior chamber, vitreous or subretinal space
Invasion of the post-laminar optic nerve
choroid (>2 mm), sclera, orbit, anterior chamber

References

  1. Pratt CB, Fontanesi J, Lu X, Parham DM, Elfervig J, Meyer D (1997). "Proposal for a New Staging Scheme for Intraocular and Extraocular Retinoblastoma Based on an Analysis of 103 Globes". Oncologist. 2 (1): 1–5. PMID 10388023.
  2. Shields CL, Mashayekhi A, Demirci H, Meadows AT, Shields JA (2004). "Practical approach to management of retinoblastoma". Arch Ophthalmol. 122 (5): 729–35. doi:10.1001/archopht.122.5.729. PMID 15136321.
  3. 3.0 3.1 Linn Murphree A (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.
  4. Chantada GL, Sampor C, Bosaleh A, Solernou V, Fandiño A, de Dávila MT (September 2013). "Comparison of staging systems for extraocular retinoblastoma: analysis of 533 patients". JAMA Ophthalmol. 131 (9): 1127–34. doi:10.1001/jamaophthalmol.2013.260. PMID 23787805.
  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. Zage PE, Reitman AJ, Seshadri R, Weinstein JL, Mets MB, Zeid JL, Greenwald MJ, Strauss LC, Goldman S (March 2008). "Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma". Pediatr Blood Cancer. 50 (3): 567–72. doi:10.1002/pbc.21301. PMID 17729249.
  7. Novetsky DE, Abramson DH, Kim JW, Dunkel IJ (March 2009). "Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies--an analysis of impact". Ophthalmic Genet. 30 (1): 40–4. doi:10.1080/13816810802452168. PMID 19172510.
  8. "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.

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