Retinoblastoma diagnostic study of choice: Difference between revisions
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**Detection of trilateral retinoblastoma (pinealoblastoma) | **Detection of trilateral retinoblastoma (pinealoblastoma) | ||
=== Staging === | |||
===Staging=== | |||
'''Extraocular Disease''' | '''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> | *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> | ||
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|- | |- | ||
! 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 [[Resection|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 nodes|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]] | * Prechiasmatic [[lesion]] | ||
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; | ; | ||
;American Joint Committee on Cancer (AJCC) | ; | ||
; | |||
; | |||
;American Joint Committee on Cancer (AJCC) Staging System | |||
; | ; | ||
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| style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding''' | | style="background: #DCDCDC; padding: 5px; text-align: center;" |'''Finding''' | ||
|- | |- | ||
! cTX | |||
| | ! | | ||
| | |||
* Unknown evidence of intraocular [[tumor]] | |||
|- | |- | ||
! cT0 | ! cT0 | ||
! | | |||
| | |||
* No evidence of intraocular [[tumor]] | |||
|- | |- | ||
! cT1 | ! cT1 | ||
! 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 | |||
[[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 | |||
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 | |||
[[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 | |||
[[Phthisis bulbi|Phthisis]] or pre-[[phthisis bulbi]] | | | ||
* Advanced intraocular [[tumor]](s) | |||
* [[Phthisis bulbi|Phthisis]] or pre-[[phthisis bulbi]] | |||
|- | |- | ||
! cT3 | ! cT3 | ||
! 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 | |||
Raised intraocular pressure with [[neovascularization]] and/or [[buphthalmos]] | | | ||
* Advanced intraocular [[tumor]](s) | |||
* Raised [[intraocular pressure]] with [[neovascularization]] and/or [[buphthalmos]] | |||
|- | |- | ||
! cT3 | ! cT3 | ||
! d | |||
[[Hyphema]] and/or massive [[vitreous hemorrhage]] | | | ||
* Advanced intraocular [[tumor]](s) | |||
* [[Hyphema]] and/or massive [[vitreous hemorrhage]] | |||
|- | |- | ||
! cT3 | ! cT3 | ||
! e | |||
[[Aseptic]] [[orbital cellulitis]] | | | ||
* Advanced intraocular [[tumor]](s) | |||
* [[Aseptic]] [[orbital cellulitis]] | |||
|- | |- | ||
! cT4 | ! cT4 | ||
! 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]] 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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|- | |- | ||
! cNX | ! cNX | ||
! | | |||
| | |||
* Regional [[lymph nodes]] cannot be assessed | |||
|- | |- | ||
! cN0 | ! cN0 | ||
! | | |||
| | |||
* No regional [[Lymph node metastases|lymph node involvement]] | |||
|- | |- | ||
! cN1 | ! cN1 | ||
! | | |||
| | |||
* Evidence of preauricular, [[submandibular]]<nowiki/>, and [[cervical]] [[Lymph node metastases|lymph node involvement]] | |||
|- | |- | ||
|+ | |+ | ||
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|- | |- | ||
! cM0 | ! cM0 | ||
! | | |||
| | |||
* No [[signs]] or [[symptoms]] of intracranial or distant [[metastasis]] | |||
|- | |- | ||
! cM1 | ! cM1 | ||
! 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 | |||
* [[Tumor]] involving the central nervous system on radiological imaging (not including trilateral retinoblastoma) | |||
|- | |- | ||
! pM1 | ! pM1 | ||
! 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 | |||
|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) | ||
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|- | |- | ||
! HX | ! HX | ||
! | | |||
| | |||
* Unknown or insufficient evidence of a constitutional [[RB1]] [[gene]] [[mutation]] | |||
|- | |- | ||
!H0 | !H0 | ||
! | |||
| | | | ||
* 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]] | |||
|- | |- | ||
|- | |- | ||
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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:Surgery]] | [[Category:Surgery]] |
Latest revision as of 23:59, 29 July 2020
Retinoblastoma Microchapters |
Diagnosis |
---|
Treatment |
Case Studies |
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 | ||||||
Stage I |
| |||||
Stage II |
| |||||
Stage III |
|
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]
- 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 |
| |
cT0 |
| |
cT1 | a | |
cT1 | b | |
cT2 | a |
|
cT2 | b |
|
cT3 | a |
|
cT3 | b |
|
cT3 | c |
|
cT3 | d |
|
cT3 | e |
|
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 |
|
pM1 | a |
|
pM1 | b |
|
Heritable trait (H) | ||
Stage | Sub-stage | Finding |
HX | ||
H0 |
| |
H1 |
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.