Retinoblastoma staging: Difference between revisions

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Revision as of 18:37, 25 April 2019

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

Overview

According to the International classification of retinoblastoma, there are five stages of retinoblastoma based on the tumor size, location, local spread, neovascular glaucoma, intravascular hemorrhage, and orbital cellulitis.

Staging

There is two known staging system for retinoblastoma:
- American Joint Committee on Cancer (AJCC) staging system
- International Retinoblastoma Staging System
  - This staging system is used in the clinical setting greater than the AJCC staging system.

American Joint Committee on Cancer (AJCC) staging system


Primary tumour (cT)
Stage	Sub-stage	Finding
cTX		Unknown evidence of intraocular tumour
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) Tumour 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 nodes 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

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

@@ Line 14: / Line 14: @@
 |+
-! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Definition of primary tumour (cT)
+! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Primary tumour (cT)
 |-
 | align="center" style="background:#f0f0f0;" |'''Stage'''
@@ Line 62: / Line 62: @@
 Aseptic orbital cellulitis
 |-
-| T3c
+| cT4
-| IIIc||[[Peritoneal]] [[metastasis]] beyond the [[pelvis]] >2 cm in diameter with or without [[metastasis]] to the [[retroperitoneal]] [[lymph nodes]] (includes the [[extension]] of [[tumor]] to the [[capsule]] of [[liver]] and [[spleen]] without [[parenchymal]] involvement of either [[Organ (anatomy)|organ]])
+| 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
 |-
-| AnyT
+| cT4
-| IV||Growth involving one or both [[ovaries]] with distant [[Metastasis|metastases]]. If [[pleural effusion]] is present, there must be positive [[cytology]] to allow a case to stage IV. [[Parenchymal]] [[liver]] [[metastasis]] equals stage IV.
+| b||Extraocular tumor(s) involving the orbit, including the optic nerve
+Extraocular tumor clinically evident with proptosis and orbital mass
 |-
 |+
-! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Regional lymph node involvement
+! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |'''Regional lymph nodes (cN)'''
 |-
-| align="center" style="background:#f0f0f0;" |'''Node involvement'''
+| align="center" style="background:#f0f0f0;" |'''Stage'''
-| align="center" style="background:#f0f0f0;" |'''FIGO stage'''
+| align="center" style="background:#f0f0f0;" |'''Sub-stage'''
 | align="center" style="background:#f0f0f0;" |'''Finding'''
 |-
-| N0
+| cNX
-| ||No [[lymph node]] involvement
+| ||Regional lymph nodes cannot be assessed
 |-
-| N0(i+)
+| cN0
-| ||[[Tumor cell|Tumor cells]] are present in regional [[Lymph nodes|lymph node(s)]], no greater than 0.2 mm
+| ||No regional lymph nodes involvement
 |-
-| N1
+| cN1
-| IIIA1||[[Histologically]] confirmed [[Retroperitoneal|retroperitonea]]<nowiki/>l [[lymph nodes]]
+| ||Evidence of preauricular, submandibular<nowiki/>, and cervical lymph node involvement
-|-
-| N1a
-| IIIA1i||[[Metastasis]] <and= 10 mm in greatest dimension
-|-
-| N1b
-| IIIA1ii||[[Metastasis]] more than 10 mm in greatest dimension
 |-
 |+
@@ Line 95: / Line 91: @@
 ! colspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Distant metastasis
 |-
-| align="center" style="background:#f0f0f0;" |'''Presence of metastasis'''
+| align="center" style="background:#f0f0f0;" |'''Stage'''
-| align="center" style="background:#f0f0f0;" |'''FIGO stage'''
+| align="center" style="background:#f0f0f0;" |'''Sub-stage'''
 | align="center" style="background:#f0f0f0;" |'''Finding'''
 |-
-| M0
+| cM0
-| ||No distant [[metastasis]]
+| ||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)
 |-
-| M1
+| pM1
-| IV||Distant [[metastasis]], including positive [[cytology]] of [[pleural effusion]]; [[liver]] or [[splenic]] [[parenchymal]] [[metastasis]]; [[metastasis]] to extra-[[abdominal]] [[organs]] (including [[inguinal lymph nodes]] and [[lymph nodes]] outside the [[abdominal cavity]]); and transmural involvement of [[intestine]]
+| a||Distant metastasis with microscopic confirmation
+Histopathological confirmation of tumor at any distant site (e.g. bone marrow, liver, or other)
 |-
-| M1a
+|pM1
-| IVa||[[Pleural effusion]] with positive [[cytology]]
+|b
+|Distant metastasis with microscopic confirmation
+Histopathological confirmation of tumor in the cerebrospinal fluid or CNS parenchyma
 |-
-| M1b
+|
-| IVb||[[Liver]] or [[splenic]] [[parenchymal]] [[metastases]]; [[metastases]] to extra-[[abdominal]] [[organs]] (including [[inguinal lymph nodes]] and [[lymph nodes]] outside the [[abdominal]] [[Abdominal cavity|cavity]]); transmural involvement of [[intestine]]
+|
+|
 |-
 |}

Retinoblastoma staging: Difference between revisions

Revision as of 18:37, 25 April 2019

Overview

Staging

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