Clinical stage |
When primary tumor (cT) status is... | And lymph node (cN) status is... | And distant metastasis (M) status is... | And heritable trait (H) status is... | Then the clinical stage is... |
cT1, cT2, cT3 | cN0 | cM0 | Any | Stage I |
cT4a | cN0 | cM0 | Any | Stage II |
cTb | cN0 | cM0 | Any | Stage III |
Any | cN1 | cM0 | Any | Stage III |
Any | Any | cM1 or pM1 | Any | Stage IV |
Definition of primary tumor (cT) – Based on the most advanced eye |
cTX | Unknown evidence of intraocular tumor |
cT0 | No evidence of intraocular tumor |
cT1 | Intraocular tumor(s) with subretinal fluid ≤5 mm from the base of any tumor: - cT1a – Tumors ≤3 mm and further than 1.5 mm from the disc and fovea
- cT1b – Tumors >3 mm or closer than 1.5 mm to the disc and fovea
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cT2 | Intraocular tumor(s) with retinal detachment, vitreous seeding or subretinal seeding: - cT2a – Subretinal fluid >5 mm from the base of any tumor
- cT2b – Tumors with vitreous seeding and/or subretinal seeding
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cT3 | Advanced intraocular tumor(s): - cT3a – Phthisis or pre-phthisis bulbi
- cT3b – Tumor invasion of the pars plana, ciliary body, lens, zonules, iris, or anterior chamber
- cT3c – Raised intraocular pressure with neovascularization and/or buphthalmos
- cT3d – Hyphema and/or massive vitreous hemorrhage
- cT3e – Aseptic orbital cellulitis
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cT4 | Extraocular tumor(s) involving the orbit, including the optic nerve: - cT4a – Radiologic evidence of retrobulbar optic nerve involvement or thickening of the optic nerve or involvement of the orbital tissues
- cT4b – Extraocular tumor clinically evident with proptosis and orbital mass
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Definition of regional lymph nodes (cN) |
cNX | Regional lymph nodes cannot be assessed |
cN0 | No regional lymph nodes involvement |
cN1 | Evidence of preauricular, submandibular, and cervical lymph node involvement |
Definition of distant metastasis (M) |
cM0 | No signs or symptoms of intracranial or distant metastasis |
cM1 | Distant metastasis without microscopic confirmation: - cM1a – Tumor(s) involving any distant site (eg, bone marrow, liver) on clinical or radiologic tests
- cM1b – Tumor involving the CNS on radiologic imaging, not including pineoblastoma (ie, "trilateral" retinoblastoma)
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pM1 | Distant metastasis with microscopic confirmation: - pM1a – Histopathologic confirmation of tumor at any distant site (eg, bone marrow, liver, or other)
- pM1b – Histopathologic confirmation of tumor in the cerebrospinal fluid or CNS parenchyma
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Definition of heritable trait (H) |
HX | Unknown or insufficient evidence of a germline RB1 gene mutation |
H0 | Germline RB1 gene mutation has been excluded (ie, based on genetic testing with a high-sensitivity assay) |
H1 | Known or presumed germline RB1 gene mutation, based on any of the following: - Bilateral retinoblastoma
- Retinoblastoma associated with an intracranial CNS midline embryonic tumor (ie, "trilateral" retinoblastoma)
- Patient has a family history of retinoblastoma
- Molecular diagnosis of germline RB1 gene mutation
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