Retinoblastoma staging: Difference between revisions

Jump to navigation Jump to search
m (Bot: Removing from Primary care)
 
(60 intermediate revisions by 5 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Retinoblastoma}}
{{Retinoblastoma}}
{{CMG}},{{AS}}{{JC}}
{{CMG}}; {{AE}} {{Sahar}} {{Simrat}}
 
==Overview==
==Overview==
The International Retinoblastoma Staging System (IRSS) stages are based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread.
A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. The available grouping systems include the International Intraocular [[Retinoblastoma]] [[Classification]] (IIRC), Intraocular [[Classification]] of Retinoblastoma (ICRB) and [[TNM|cTNMH systems]] [[diseases]].
 
==Staging==
==Staging==
 
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.
The Reese-Ellsworh classification was developed in the 1960s by Dr.Algernon Reese and Dr.Robert Ellsworth, two prominent New York doctors specialising in retinoblastoma. The system was designed to predict outcome from treatment with [[external beam radiotherapy(EBRT)]], used internationally as the primary eye salvage treatment until intoduction of chemotherapy in the 1980s.
Retinoblastoma is classified in two subgroups:<br>
{| class="wikitable"
'''Extraocular disease'''
|+ '''Reese-Ellsworth classification(REC) for Retinoblastoma'''
*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>
! Groups !! prognosis!! Tumor size and number
**This sub-classification is established by Chantada and colleagues in 2006.
***This staging system is used in the [[clinical]] setting greater than the AJCC staging system (Please see below).
;International Retinoblastoma Staging System
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Stage
| colspan="6" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Description
|-
|-
|'''Group 1'''|| Very favourable for maintenance of sight||'''A:''' Solitary tumor, smaller than 4 disc diameters(DD) at or behind the equator <BR> '''B:''' Multiple tumors, none larger than 4 DD, all at or behind the equator
| 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]]
|-
|-
|'''Group 2'''|| Favourable for maintenance of sight||'''A:''' Solitary tumor, 4 to 10 DD at or behind the equator <BR> '''B:''' Multiple tumors, 4 to 10 DD behind the equator
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage I
| colspan="6" style="background: #F5F5F5; padding: 5px;" |Eye enucleated, completely resected [[Histology|histologically]]
|-
|-
|'''Group 3'''|| Possible for maintenance of sight||'''A:''' Any lesion anterior to the equator <BR> '''B:''' Solitary tumor, larger than 10 DD behind the equator
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Stage II
| colspan="6" style="background: #F5F5F5; padding: 5px;" |Eye enucleated, [[microscopic]] residual [[tumor]]
|-
|-
|'''Group 4'''|| Unfavourable for maintenance of sight||'''A:''' Multiple tumors, some larger than 10 DD <BR> '''B:''' Any lesion extending anteriorly to the [[ora serrata]]
| 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;" |a.Overt [[orbital disease]]
 
b.Preauricular or cervical [[lymph node]] extension
|-
|-
|'''Group 5'''|| very unfavourable for maintenance of sight||'''A:''' Massive tumors involving more than one half of the [[retina]] <BR> '''B:''' [[Vitreous]] seeding
| 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;" |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]]
|}
|}
The REC is rarely used today as chemotherapy has superseded radiotherapy as the favoured treatment for eye salvage.


===Essen classification:===
*'''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]].
[[image:Essen classification.jpg|center|650px]]
**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>
<br clear="left"/>
*IIRC grouping system sub-classifies the [[tumor]] according to from A to E according to [[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), 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.


===International retinoblastoma classification:===
;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>
|-
|}
{| class="wikitable"
{| class="wikitable"
|+
|+
! Staging !! Metastasis and treatment
 
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Primary tumour (cT)*
|-
| align="center" style="background:#f0f0f0;" |'''Stage'''
| align="center" style="background:#f0f0f0;" |'''Sub-stage'''
| align="center" style="background:#f0f0f0;" |'''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 [[Optic disc|disc]] and [[fovea]]
|-
|cT1
| 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]]
|-
| 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
[[Tumors]] with [[vitreous]] seeding and/or sub-retinal seeding
|-
| cT3
| a||Advanced intraocular [[tumor]](s)
[[Phthisis bulbi|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 of eyeball|anterior chamber]]
|-
| cT3
| c||Advanced intraocular [[tumor]](s)
Raised intraocular pressure with [[neovascularization]] and/or [[buphthalmos]]
|-
|-
| '''Stage 0'''|| Patients treated conservatively (subject to presurgical ophthalmologic classifications)
| cT3
| d||Advanced intraocular [[tumor]](s)
[[Hyphema]] and/or massive [[vitreous hemorrhage]]
|-
|-
| '''Stage I'''|| Eye enucleated, completely resected histologically
| cT3
| e||Advanced intraocular [[tumor]](s)
[[Aseptic]] [[orbital cellulitis]]
|-
|-
| '''Stage II'''|| Eye enucleated, microscopic residual tumor
| 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 [[Orbit (anatomy)|orbital]] tissues
|-
|-
| '''Stage III'''|| Regional extension <BR> a) Overt orbital disease  <BR>  b) Preauricular or cervical lymph node extension
| cT4
| b||Extraocular [[tumor]](s) involving the [[orbit]], including the [[optic nerve]]
Extraocular [[tumor]] clinically evident with [[Exophthalmos|proptosis]] and [[Orbital mass causes|orbital mass]]
|-
|-
| '''Stage IV'''|| Metastatic disease <BR>  a) Hematogenous metastasis <BR>  1. single lesion <BR> 2. multiple lesions <BR> b) CNS extension <BR> 1. Prechiasmatic lesion <BR> 2. CNS mass <BR> 3. Leptomeningeal disease
|+
|}


===The International Intraocular Retinoblastoma Classification(IIRC)or The ABC classification===
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |'''Regional lymph nodes (cN)'''
{| class="wikitable"
|+ The International Intraocular Retinoblastoma Classification(IIRC)or The ABC classification
!Groups !! Location !! Size
|-
|-
| '''Group A'''|| small tumors away from foveola and disc || Tumors <3 mm in greatest dimension confined to the retina and  <BR> Located at least 3 mm from the foveola and 1.5 mm from the optic disc
| align="center" style="background:#f0f0f0;" |'''Stage'''
| align="center" style="background:#f0f0f0;" |'''Sub-stage'''
| align="center" style="background:#f0f0f0;" |'''Finding'''
|-
|-
| '''Group B'''|| all remaining tumors confined to the retina || All other tumors confined to the retina and not in group A <BR> Subretinal fluid (without subretinal seeding) < 3 mm from the base of the tumor
| cNX
| ||Regional [[lymph nodes]] cannot be assessed
|-
|-
| '''Group C'''|| local subretinal fluid or vitreous seeding || Subretinal fluid alone >3 mm and < 6 mm from the tumor <BR> Vitreous or subretinal seeding < 3 mm from the tumor
| cN0
| ||No regional [[Lymph node metastases|lymph node involvement]]
|-
|-
| '''Group D'''|| diffuse subretinal fluid or seeding || Subretinal fluid > 6 mm from the tumor <BR> Vitreous or subretinal seeding > 3 mm from the tumor
| cN1
| ||Evidence of preauricular, [[submandibular]]<nowiki/>, and [[cervical]] [[Lymph node metastases|lymph node involvement]]
|-
|-
| '''Group E'''|| presence of any one or more of these poor prognosis features|| More than 2/3 of the globe filled with tumor <BR> Tumor in the anterior segment or anterior to the vitreous <BR> Tumor in or on the ciliary body <BR> Iris neovascularisation <BR> Neovascular glaucoma <BR> Opaque media from hemorrhage <BR> Tumor necrosis with aseptic orbital celullitis  <BR> Phthisis bulbi
|+
|}


===The International Retinoblastoma Staging System (IRSS)===
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Distant metastasis
 
|-
{| class="wikitable"
| align="center" style="background:#f0f0f0;" |'''Stage'''
|+The International Retinoblastoma Staging System (IRSS) <ref>http://www.cancer.gov/types/retinoblastoma/patient/retinoblastoma-treatment-pdq#section/_27</ref>
| align="center" style="background:#f0f0f0;" |'''Sub-stage'''
!Staging  !! how much cancer remains after surgery and whether the cancer has spread
| align="center" style="background:#f0f0f0;" |'''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 [[Imaging studies|radiological tests]]
|-
|-
| Stage 0 || The tumor is in the eye only. The eye has not been removed and the tumor was treated without surgery.
| cM1
| b||Distant [[metastasis]] without [[microscopic]] confirmation
Tumour involving the central nervous system on radiological imaging (not including trilateral retinoblastoma)
|-
|-
| Stage I || The tumor is in the eye only. The eye has been removed and no cancer cells remain.
| pM1
| a||Distant [[metastasis]] with [[microscopic]] confirmation
[[Histopathological]] confirmation of [[tumor]] at any distant site (e.g. [[bone marrow]], [[liver]], or other)
|-
|-
| Stage II || The tumor is in the eye only. The eye has been removed and there are cancer cells left that can be seen only with a microscope.
|pM1
|b
|Distant [[metastasis]] with [[microscopic]] confirmation
 
[[Histopathological]] confirmation of [[tumor]] in the [[cerebrospinal fluid]] or [[CNS]] [[parenchyma]]
|-
|-
| Stage III
! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Heritable trait (H)
|-
|-
| Stage IIIa || cancer has spread from the eye to tissues around the eye socket.
| align="center" style="background:#f0f0f0;" |'''Stage'''
| align="center" style="background:#f0f0f0;" |'''Sub-stage'''
| align="center" style="background:#f0f0f0;" |'''Finding'''
|-
|-
| Stage IIIb || cancer has spread from the eye to lymph nodes near the ear or in the neck.
| HX
| ||Unknown or insufficient evidence of a constitutional [[RB1]] [[gene]] [[mutation]]
|-
|-
| Stage IV
|H0
|
|Normal [[RB1]] alleles in blood tested with demonstrated high [[sensitivity]] assays
|-
|-
| Stage IVa || cancer has spread to the blood but not to the brain or spinal cord. One or more tumors may have spread to other parts of the body such as the bone or liver.
|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]]
|-
|-
| Stage IVb || cancer has spread to the brain or spinal cord. It also may have spread to other parts of the body.
|-
|-
|}
|}


==References==
==References==
{{reflist|2}}
{{Reflist|2}}
 
{{WH}}
{{WS}}
 
[[Category:Disease]]
 
==See also==
*[[Eye cancer]]
*[[Eye examination]]
 


{{Nervous tissue tumors}}


[[Category:Ophthalmology]]
[[Category:Medicine]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:hereditary cancers]]
[[Category:Up-To-Date]]
[[Category:Oncology stub]]
[[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 staging On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Retinoblastoma staging

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Retinoblastoma staging

CDC on Retinoblastoma staging

Retinoblastoma staging in the news

Blogs on Retinoblastoma staging

Directions to Hospitals Treating Retinoblastoma

Risk calculators and risk factors for Retinoblastoma staging

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

A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. The available grouping systems include the International Intraocular Retinoblastoma Classification (IIRC), Intraocular Classification of Retinoblastoma (ICRB) and cTNMH systems diseases.

Staging

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 cTNMH systems. For extraocular diseases, the International Retinoblastoma Staging System (IRSS) and cTNMH schemes can be used. Retinoblastoma is classified in two subgroups:
Extraocular disease

  • This category first subclassified via International Retinoblastoma Staging System different staging system to 0–IV.[1]
    • This sub-classification is established by Chantada and colleagues in 2006.
      • This staging system is used in the clinical setting greater than the AJCC staging system (Please see below).
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)

—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 which first classified by Reese-Ellsworth Classification method in the 1960s and used to predict the survival chance of salvaging eye following external beam radiotherapy.
    • This classification replaced by International Intraocular Retinoblastoma Classification (IIRC) scheme following the introduction of chemotherapy for the treatment of retinoblastoma in 1990.[2]
  • IIRC grouping system sub-classifies the tumor according to from A to E according to tumor characteristics such as:

In 2006, Intraocular Classification of Retinoblastoma (ICRB), has been developed by Dr. Shields and colleagues.[3]

  • 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.[4]
  • 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
The table below adopted from Community Eye Health Journal [5]
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

Tumors > 3 mm or closer than 1.5 mm to the disc and fovea

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

Tumors with vitreous seeding and/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)

Aseptic orbital cellulitis

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

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.