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: Sahar Memar Montazerin, M.D.[2] Simrat Sarai, M.D. [3]
Overview
Staging
Retinoblastoma is classified in two subgroups:
Extraocular disease
- This category first subclassified via International Retinoblastoma Staging System different staging system to 0–IV.[1]
- This sub-classification is established by Chantada and colleagues in 2006.
- This staging system is used in the clinical setting greater than the AJCC staging system (Please see below).
- This sub-classification is established by Chantada and colleagues in 2006.
- 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 | ||||
- Intraocular disease which first classified by Reese-Ellsworth Classification method in the 1960s and used to predict the survival chance of salvaging eye following external beam radiotherapy.
- This classification replaced by International Intraocular Retinoblastoma Classification (IIRC) scheme following the introduction of chemotherapy for the treatment of retinoblastoma in 1990.[2]
- IIRC grouping system sub-classifies the tumor according to from A to E according to tumor characteristics such as:
- Size
- Location
- Presence of retinoblastoma ‘seeds’ (small colonies of cancerous cells in the vitreous)
- Retinal detachment
In 2006, Intraocular Classification of Retinoblastoma (ICRB), has been developed by Dr. Shields and colleagues.[3]
- This classification is modified version of IIRC and the main difference is in advanced categories of the tumor (D and E).
- This classification method has been observed to be successful at predicting the outcome of chemotherapy.
- IIRC and ICRB are now the most commonly used classification system for retinoblastoma.[4]
- American Joint Committee on Cancer (AJCC) staging system is another suggested grouping system for the classification of the tumor using TNM system.
- This system is not frequently used for pediatrics and is not grouped according to the prognostic values.
- American Joint Committee on Cancer (AJCC) staging system
| The table below adopted from Community Eye Health Journal [5] |
|---|
| Primary tumour (cT)* | ||
|---|---|---|
| Stage | Sub-stage | Finding |
| cTX | Unknown evidence of intraocular tumor | |
| 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 |
| 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 |
| cT3 | a | Advanced intraocular tumor(s)
Phthisis or pre-phthisis bulbi |
| cT3 | b | Advanced intraocular tumor(s)
Tumor 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) |
| 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 node 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 | |
References
- ↑ Chantada G, Doz F, Antoneli CB, Grundy R, Clare Stannard FF, Dunkel IJ, Grabowski E, Leal-Leal C, Rodríguez-Galindo C, Schvartzman E, Popovic MB, Kremens B, Meadows AT, Zucker JM (November 2006). "A proposal for an international retinoblastoma staging system". Pediatr Blood Cancer. 47 (6): 801–5. doi:10.1002/pbc.20606. PMID 16358310.
- ↑ Linn Murphree A (March 2005). "Intraocular retinoblastoma: the case for a new group classification". Ophthalmol Clin North Am. 18 (1): 41–53, viii. doi:10.1016/j.ohc.2004.11.003. PMID 15763190.
- ↑ Shields CL, Mashayekhi A, Au AK, Czyz C, Leahey A, Meadows AT, Shields JA (December 2006). "The International Classification of Retinoblastoma predicts chemoreduction success". Ophthalmology. 113 (12): 2276–80. doi:10.1016/j.ophtha.2006.06.018. PMID 16996605.
- ↑ Scelfo C, Francis JH, Khetan V, Jenkins T, Marr B, Abramson DH, Shields CL, Pe'er J, Munier F, Berry J, Harbour JW, Yarovoy A, Lucena E, Murray TG, Bhagia P, Paysse E, Tuncer S, Chantada GL, Moll AC, Ushakova T, Plager DA, Ziyovuddin I, Leal CA, Materin MA, Ji XD, Cursino JW, Polania R, Kiratli H, All-Ericsson C, Kebudi R, Honavar SG, Vishnevskia-Dai V, Epelman S, Daniels AB, Ling JD, Traore F, Ramirez-Ortiz MA (2017). "An international survey of classification and treatment choices for group D retinoblastoma". Int J Ophthalmol. 10 (6): 961–967. doi:10.18240/ijo.2017.06.20. PMC 5515152. PMID 28730089.
- ↑ "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.