Retinoblastoma staging: Difference between revisions

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Revision as of 18:18, 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


Definition of 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
T3c	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)
AnyT	IV	Growth involving one or both ovaries with distant metastases. If pleural effusion is present, there must be positive cytology to allow a case to stage IV. Parenchymal liver metastasis equals stage IV.
Regional lymph node involvement
Node involvement	FIGO stage	Finding
N0		No lymph node involvement
N0(i+)		Tumor cells are present in regional lymph node(s), no greater than 0.2 mm
N1	IIIA1	Histologically confirmed retroperitoneal lymph nodes
N1a	IIIA1i	Metastasis <and= 10 mm in greatest dimension
N1b	IIIA1ii	Metastasis more than 10 mm in greatest dimension
Distant metastasis
Presence of metastasis	FIGO stage	Finding
M0		No distant metastasis
M1	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
M1a	IVa	Pleural effusion with positive cytology
M1b	IVb	Liver or splenic parenchymal metastases; metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside the abdominal cavity); transmural involvement of intestine

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 23: / Line 23: @@
 | ||Unknown evidence of intraocular tumour
 |-
-|  T1a
+|  cT0
-| Ia||Growth limited to one [[ovary]]; no [[ascites]] present containing [[malignant]] cells. No [[tumor]] on the external [[Surface anatomy|surface]]; [[capsule]] intact
+| ||No evidence of intraocular tumor
 |-
-| T1b
+| cT1
-| Ib||Growth limited to one or both [[ovaries]]; no [[ascites]] present containing [[malignant]] cells. No [[tumor]] on the external [[Surface anatomy|surfaces]]; [[capsules]] intact
+| 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
 |-
-| T1c
+|cT1
-| Ic||[[Tumor]] either stage Ia or Ib, but with the [[tumor]] on [[Surface anatomy|surface]] of one or both [[ovaries]], or with [[capsule]] [[Rupture|ruptured]], or with [[ascites]] present containing [[malignant]] [[cells]], or with positive [[peritoneal]] washings
+| 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
 |-
-| T2
+| cT2
-| II||Growth involving one or both [[ovaries]] with [[pelvic]] [[extension]]
+| a||Intraocular tumor(s) with retinal detachment, vitreous seeding or sub-retinal seeding
+Sub-retinal fluid > 5 mm from the base of any tumor
 |-
-| T2a
+| cT2
-| IIa||[[Extension]] and/or [[metastases]] to the [[uterus]] and/or tubes
+| b||Intraocular tumor(s) with retinal detachment, vitreous seeding or sub-retinal seeding
+Tumors with vitreous seeding and/or sub-retinal seeding
 |-
-| T2b
+| cT3
-| IIb||[[Extension]] to other [[pelvic]] [[tissues]]
+| a||Advanced intraocular tumor(s)
+Phthisis or pre-phthisis bulbi
 |-
-| T2c
+| cT3
-| IIc||[[Tumor]] either stage IIa or IIb, but with the [[tumor]] on [[Surface anatomy|surface]] of one or both [[ovaries]], or with [[Capsule|capsule(s)]] [[Rupture|ruptured]], or with [[ascites]] present containing [[malignant]] cells, or with positive [[peritoneal]] washings
+| b||Advanced intraocular tumor(s)
+Tumour invasion of the pars plana, ciliary body, lens, zonules, iris or anterior chamber
 |-
-| T3
+| cT3
-| III||[[Tumor]] involving one or both [[ovaries]] with [[histologically]] confirmed [[peritoneal]] [[implants]] outside the [[pelvis]] and/or positive regional [[lymph nodes]]. [[Superficial]] [[liver]] [[metastases]] equal stage III. The [[tumor]] is limited to the [[true pelvis]], but with [[histologically]] proven [[malignant]] extension to [[small bowel]] or [[omentum]]
+| c||Advanced intraocular tumor(s)
+Raised intraocular pressure with neovascularization and/or buphthalmos
 |-
-| T3a
+| cT3
-| IIIa2||[[Tumor]] grossly limited to the [[true pelvis]] with [[histologically]] confirmed [[microscopic]] seeding of [[abdominal]] [[peritoneal]] [[Surface area|surfaces]], or [[histologic]] proven [[extension]] to [[small bowel]] or [[mesentery]] with or without positive [[retroperitoneal]] [[lymph nodes]]
+| d||Advanced intraocular tumor(s)
+Hyphema and/or massive vitreous hemorrhage
 |-
-| T3b
+| cT3
-| IIIb||[[Tumor]] of one or both [[ovaries]] with [[histologically]] confirmed [[implants]], [[peritoneal]] [[metastasis]] of [[abdominal]] [[peritoneal]] [[Surface area|surfaces]], none exceeding 2 cm in diameter with or without [[extension]] [[metastasis]] to the [[retroperitoneal]] [[Lymph node|lymph nodes]]
+| e||Advanced intraocular tumor(s)
+Aseptic orbital cellulitis
 |-
 | T3c

Retinoblastoma staging: Difference between revisions

Revision as of 18:18, 25 April 2019

Overview

Staging

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