Retinoblastoma staging

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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

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
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