Retinoblastoma diagnostic study of choice: Difference between revisions
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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=== | ||
[[Ultrasound imaging]] is the gold standard test for the [[diagnosis]] of retinoblastoma. | |||
* | * [[Ultrasound imaging]] is the [[Gold standard (test)|gold standard test]] for the [[diagnosis]] of retinoblastoma. | ||
* [[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> | |||
* [[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> | |||
[[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> | **Confirmation of the [[diagnosis]] | ||
**Evaluation of local [[tumor]] extent | |||
**Assessment of associated developmental [[malformation]] of the [[brain]] | |||
**Detection of trilateral retinoblastoma (pinealoblastoma) | |||
<br /> | |||
===Staging=== | ===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> | |||
'''Extraocular | |||
*This category | '''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 | |||
| 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 | |||
| 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 | |||
| 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 | |||
| colspan="3" style="background: #F5F5F5; padding: 5px;" |Regional extension | | colspan="3" style="background: #F5F5F5; padding: 5px;" |Regional extension | ||
| colspan="3" style="background: #F5F5F5; padding: 5px;" |a.Overt [[orbital disease]] | | colspan="3" style="background: #F5F5F5; padding: 5px;" |a. Overt [[orbital disease]] | ||
b.Preauricular or | b. Preauricular or [[Cervical lymph nodes|cervical lymph node]] extension | ||
|- | |- | ||
! 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;" |[[Metastatic disease]] | ||
| colspan="3" style="background: #F5F5F5; padding: 5px;" |a.Hematogenous [[metastasis]] (without [[CNS]] involvement) | | colspan="3" style="background: #F5F5F5; padding: 5px;" |a. Hematogenous [[metastasis]] (without [[CNS]] involvement) | ||
* Single [[lesion]] | |||
* Multiple [[lesions]] | |||
b. [[CNS]] extension (with or without any other site of regional or [[metastatic disease]]) | |||
* Prechiasmatic [[lesion]] | |||
* [[CNS]] mass | |||
* [[Leptomeningeal]] and [[CSF]] [[disease]] | |||
|} | |||
'''Intraocular 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> | |||
|} | |||
* | *IIRC grouping system sub-[[Classification|classifies]] the [[tumor]] based on the [[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) 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 | ;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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|- | |- | ||
| |cTX | | |cTX | ||
| ||Unknown evidence of intraocular [[tumor]] | | ||Unknown evidence of intraocular [[tumor]] | ||
|- | |- | ||
! cT0 | |||
| ||No evidence of intraocular [[tumor]] | | ||No evidence of intraocular [[tumor]] | ||
|- | |- | ||
! cT1 | |||
| a||Intraocular [[tumor]](s) with sub-retinal fluid ≤ 5mm from the base of any [[tumor]] | | a||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]] | [[Tumors]] ≤ 3mm and further than 1.5 mm from the [[Optic disc|disc]] and [[fovea]] | ||
|- | |- | ||
!cT1 | |||
| b||Intraocular [[tumor]](s) with sub-retinal fluid ≤ 5mm from the base of any [[tumor]] | | b||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]] | [[Tumor|Tumors]] > 3 mm or closer than 1.5 mm to the [[Optic disc|disc]] and [[fovea]] | ||
|- | |- | ||
! cT2 | |||
| a||Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding | | a||Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding | ||
Sub-retinal fluid > 5 mm from the base of any [[tumor]] | Sub-retinal fluid > 5 mm from the base of any [[tumor]] | ||
|- | |- | ||
! cT2 | |||
| b||Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding | | b||Intraocular [[tumor]](s) with [[retinal detachment]], [[vitreous]] seeding or sub-retinal seeding | ||
[[Tumors]] with [[vitreous]] seeding and/or sub-retinal seeding | [[Tumors]] with [[vitreous]] seeding and/or sub-retinal seeding | ||
|- | |- | ||
! cT3 | |||
| a||Advanced intraocular [[tumor]](s) | | a||Advanced intraocular [[tumor]](s) | ||
[[Phthisis bulbi|Phthisis]] or pre-[[phthisis bulbi]] | [[Phthisis bulbi|Phthisis]] or pre-[[phthisis bulbi]] | ||
|- | |- | ||
! cT3 | |||
| b||Advanced intraocular [[tumor]](s) | | b||Advanced intraocular [[tumor]](s) | ||
[[Tumor]] invasion of the [[pars plana]], [[ciliary body]], [[lens]], [[zonules]], [[iris]] or [[Anterior chamber of eyeball|anterior chamber]] | [[Tumor]] invasion of the [[pars plana]], [[ciliary body]], [[lens]], [[zonules]], [[iris]] or [[Anterior chamber of eyeball|anterior chamber]] | ||
|- | |- | ||
! cT3 | |||
| c||Advanced intraocular [[tumor]](s) | | c||Advanced intraocular [[tumor]](s) | ||
Raised intraocular pressure with [[neovascularization]] and/or [[buphthalmos]] | Raised intraocular pressure with [[neovascularization]] and/or [[buphthalmos]] | ||
|- | |- | ||
! cT3 | |||
| d||Advanced intraocular [[tumor]](s) | | d||Advanced intraocular [[tumor]](s) | ||
[[Hyphema]] and/or massive [[vitreous hemorrhage]] | [[Hyphema]] and/or massive [[vitreous hemorrhage]] | ||
|- | |- | ||
! cT3 | |||
| e||Advanced intraocular [[tumor]](s) | | e||Advanced intraocular [[tumor]](s) | ||
[[Aseptic]] [[orbital cellulitis]] | [[Aseptic]] [[orbital cellulitis]] | ||
|- | |- | ||
! cT4 | |||
| a||Extraocular [[tumor]](s) involving the orbit, including the [[optic nerve]] | | a||Extraocular [[tumor]](s) involving the 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 | [[Radiological]] evidence of retrobulbar [[optic nerve]] involvement or thickening of the [[optic nerve]] or involvement of the [[Orbit (anatomy)|orbital]] tissues | ||
|- | |- | ||
! cT4 | |||
| b||Extraocular [[tumor]](s) involving the [[orbit]], including the [[optic nerve]] | | b||Extraocular [[tumor]](s) involving the [[orbit]], including the [[optic nerve]] | ||
Extraocular [[tumor]] clinically evident with [[Exophthalmos|proptosis]] and [[Orbital mass causes|orbital mass]] | 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 | |||
| ||Regional [[lymph nodes]] cannot be assessed | | ||Regional [[lymph nodes]] cannot be assessed | ||
|- | |- | ||
! cN0 | |||
| ||No regional [[Lymph node metastases|lymph node involvement]] | | ||No regional [[Lymph node metastases|lymph node involvement]] | ||
|- | |- | ||
! 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 | |||
| ||No [[signs]] or [[symptoms]] of intracranial or distant [[metastasis]] | | ||No [[signs]] or [[symptoms]] of intracranial or distant [[metastasis]] | ||
|- | |- | ||
! cM1 | |||
| a||Distant [[metastasis]] without [[microscopic]] confirmation | | a||Distant [[metastasis]] without [[microscopic]] confirmation | ||
[[Tumor]](s) involving any distant site (e.g. [[bone marrow]], [[liver]]) on [[clinical]] or [[Imaging studies|radiological tests]] | [[Tumor]](s) involving any distant site (e.g. [[bone marrow]], [[liver]]) on [[clinical]] or [[Imaging studies|radiological tests]] | ||
|- | |- | ||
! cM1 | |||
| b||Distant [[metastasis]] without [[microscopic]] confirmation | | b||Distant [[metastasis]] without [[microscopic]] confirmation | ||
Tumour involving the central nervous system on radiological imaging (not including trilateral retinoblastoma) | Tumour involving the central nervous system on radiological imaging (not including trilateral retinoblastoma) | ||
|- | |- | ||
! pM1 | |||
| a||Distant [[metastasis]] with [[microscopic]] confirmation | | a||Distant [[metastasis]] with [[microscopic]] confirmation | ||
[[Histopathological]] confirmation of [[tumor]] at any distant site (e.g. [[bone marrow]], [[liver]], or other) | [[Histopathological]] confirmation of [[tumor]] at any distant site (e.g. [[bone marrow]], [[liver]], or other) | ||
|- | |- | ||
!pM1 | |||
|b | |b | ||
|Distant [[metastasis]] with [[microscopic]] confirmation | |Distant [[metastasis]] with [[microscopic]] confirmation | ||
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding''' | | style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding''' | ||
|- | |- | ||
! HX | |||
| ||Unknown or insufficient evidence of a constitutional [[RB1]] [[gene]] [[mutation]] | | ||Unknown or insufficient evidence of a constitutional [[RB1]] [[gene]] [[mutation]] | ||
|- | |- | ||
!H0 | |||
| | | | ||
|Normal [[RB1]] alleles in blood tested with demonstrated high [[sensitivity]] assays | |Normal [[RB1]] alleles in blood tested with demonstrated high [[sensitivity]] assays | ||
|- | |- | ||
!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]] |
Revision as of 15:44, 21 June 2019
Retinoblastoma Microchapters |
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Retinoblastoma diagnostic study of choice On the Web |
American Roentgen Ray Society Images of Retinoblastoma diagnostic study of choice |
Risk calculators and risk factors for Retinoblastoma diagnostic study of choice |
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
- Ultrasound imaging is the gold standard test for the diagnosis of retinoblastoma.
- 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.[1]
- MRI is recommended for the following goals:[2]
- 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 | Eye enucleated, completely resected histologically | |||||
Stage II | Eye enucleated, microscopic residual tumor | |||||
Stage III | Regional extension | a. Overt orbital disease
b. Preauricular or cervical lymph node extension | ||||
Stage IV | Metastatic disease | 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]
- IIRC grouping system sub-classifies the tumor based on the tumor characteristics, such as:
- Size
- Location
- Presence of retinoblastoma ‘seeds’ (small colonies of cancerous cells in the vitreous)
- Retinal detachment
- 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
Tumors ≤ 3mm and further than 1.5 mm from the disc and fovea |
cT1 | b | Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor |
cT2 | a | Intraocular tumor(s) with retinal detachment, vitreous seeding or sub-retinal seeding
Sub-retinal fluid > 5 mm from the base of any tumor |
cT2 | b | Intraocular tumor(s) with retinal detachment, vitreous seeding or sub-retinal seeding |
cT3 | a | Advanced intraocular tumor(s)
Phthisis or pre-phthisis bulbi |
cT3 | b | Advanced intraocular tumor(s)
Tumor invasion of the pars plana, ciliary body, lens, zonules, iris or anterior chamber |
cT3 | c | Advanced intraocular tumor(s)
Raised intraocular pressure with neovascularization and/or buphthalmos |
cT3 | d | Advanced intraocular tumor(s)
Hyphema and/or massive vitreous hemorrhage |
cT3 | e | Advanced intraocular tumor(s) |
cT4 | a | Extraocular tumor(s) involving the orbit, including the optic nerve
Radiological evidence of retrobulbar optic nerve involvement or thickening of the optic nerve or involvement of the orbital tissues |
cT4 | b | Extraocular tumor(s) involving the orbit, including the optic nerve
Extraocular tumor clinically evident with proptosis and orbital mass |
Regional lymph nodes (cN) | ||
Stage | Sub-stage | Finding |
cNX | Regional lymph nodes cannot be assessed | |
cN0 | No regional lymph node involvement | |
cN1 | Evidence of preauricular, submandibular, and cervical lymph node involvement | |
Distant metastasis | ||
Stage | Sub-stage | Finding |
cM0 | No signs or symptoms of intracranial or distant metastasis | |
cM1 | a | Distant metastasis without microscopic confirmation
Tumor(s) involving any distant site (e.g. bone marrow, liver) on clinical or radiological tests |
cM1 | b | Distant metastasis without microscopic confirmation
Tumour involving the central nervous system on radiological imaging (not including trilateral retinoblastoma) |
pM1 | a | Distant metastasis with microscopic confirmation
Histopathological confirmation of tumor at any distant site (e.g. bone marrow, liver, or other) |
pM1 | b | Distant metastasis with microscopic confirmation
Histopathological confirmation of tumor in the cerebrospinal fluid or CNS parenchyma |
Heritable trait (H) | ||
Stage | Sub-stage | Finding |
HX | Unknown or insufficient evidence of a constitutional RB1 gene mutation | |
H0 | Normal RB1 alleles in blood tested with demonstrated high sensitivity assays | |
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.