Retinoblastoma staging
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Retinoblastoma Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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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
| 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 | ||||