Retinoblastoma staging
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Retinoblastoma Microchapters |
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Diagnosis |
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Retinoblastoma staging On the Web |
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American Roentgen Ray Society Images of Retinoblastoma staging |
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Risk calculators and risk factors for Retinoblastoma staging |
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 | ||||