Retinoblastoma classification

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

There are several classification systems available for retinoblastoma. As the treatment of the tumor has evolved, a new classification system has been introduced. For intraocular disease, the available grouping systems include International Intraocular Retinoblastoma Classification (IIRC), Intraocular Classification of Retinoblastoma (ICRB), and cTNMH systems. For extraocular disease, the International Retinoblastoma Staging System (IRSS) and cTNMH schemes can be used.

Classification

Retinoblastoma has been classified according to different classification systems for variable purposes.^[1]
Each classification system has been developed depending on the change in the management of the tumor.
For treatment purposes, retinoblastoma is classified into:

Intraocular Retinoblastoma Classification System

- This classification system incudes the following:
  - International Intraocular Retinoblastoma Classification (IIRC)^[2]^[3]^[4]
  - Intraocular Classification of Retinoblastoma (ICRB)
  - The cTNMH system of American Joint Committee on Cancer (AJCC)

Intraocular Classifications of Retinoblastoma and their Features

	International Intraocular Retinoblastoma Classification (IIRC)	Intraocular Classification of Retinoblastoma (ICRB)
Group A (very low risk)	Small intraretinal tumors away from foveola and optic nerve 3mm or smaller in the greatest dimension, confined to the retina Located > 3 mm from the foveola and 1.5 mm from the optic disc	Tumors ≤ 3 mm (in basal dimension or thickness)
Group B (low risk)	Tumors confined to the retina Not in group A Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding	Tumors > 3 mm (in basal dimension or thickness) or Macular location (≤ 3 mm to foveola) Juxtapapillary location (≤ 1.5 mm to disc) Additional subretinal fluid (≤3 mm from margin)
Group C (moderate risk)	Local disease with minimal subretinal or vitreous seeding with the following characteristics: Discrete Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina Local fine vitreous seeding may be present close to the discrete tumor Local subretinal seeding less than 3 mm (2 DD) from the tumor	Tumor with: Subretinal seeds ≤ 3 mm from tumor Vitreous seeds ≤ 3 mm from tumor Both subretinal and vitreous seeds ≤ 3 mm from the tumor
Group D (high risk)	Diffuse tumor with significant vitreous or subretinal seeding Maybe massive or diffuse Subretinal fluid present or past without seeding, involving up to total retinal detachment The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses Diffuse subretinal seeding may include subretinal plaques or tumor nodules	Tumor with: Subretinal seeds > 3 mm from tumor Vitreous seeds > 3 mm from tumor Both subretinal and vitreous seeds > 3 mm from retinoblastoma
Group E (very high risk)	Presence of any one or more of the following poor prognostic features: Tumor touching the lens Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment Diffuse infiltrating retinoblastoma Neovascular glaucoma Opaque media from hemorrhage Tumor necrosis with aseptic orbital cellulitis Phthisis bulbi	Extensive tumor filling >50% globe or with neovascular glaucoma Opaque media from hemorrhage in the anterior chamber, vitreous, or subretinal space Invasion of the post-laminar optic nerve choroid (>2 mm), sclera, orbit, and anterior chamber

Reese-Ellsworth Classification for Intraocular Tumors^[5]

This classification system was first introduced in 1960 and used to predict the chance of salvaging the eye after external beam radiotherapy.
However, following the introduction of chemotherapy for retinoblastoma treatment, it lost its significance.

Reese-Ellsworth Classification^[6]

Stage	Sub-stage	Features	Prognosis
Group I	a	Solitory tumor < 6 mm at or behind the equator	Very favorable
Group I	b	Multiple tumors, none > 6 mm, all are at or behind the equator	Very favorable
Group II	a	Solitary tumor, 6 to 15 mm, all at or behind the equator	Favorable
Group II	b	Multiple tumors, 6 to 15 mm, behind the equator	Favorable
Group III	a	Lesion anterior to the equator	Undetermind
Group III	b	Solitary tumor larger than 15 mm, behind the equator	Undetermined
Group IV	a	Multiple tumors, some larger than 15 mm	Unfavorable
Group IV	b	Any lesions extending to the ora serrata	Unfavorable
Group V	a	Massive tumor occupying over half the retina	Very unfavorable
Group V	b	Vitreous seeding	Very unfavorable

Extra-ocular Retinoblastoma Classification System

- This classification system includes the following:
  - International retinoblastoma staging system
  - cTNMH system of American Joint Committee on Cancer (AJCC)^[7]

To see the full staging system click here.

References

↑ Chantada GL, Sampor C, Bosaleh A, Solernou V, Fandiño A, de Dávila MT (September 2013). “Comparison of staging systems for extraocular retinoblastoma: analysis of 533 patients”. JAMA Ophthalmol. 131 (9): 1127–34. doi:10.1001/jamaophthalmol.2013.260. PMID 23787805.
↑ 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.
↑ Zage PE, Reitman AJ, Seshadri R, Weinstein JL, Mets MB, Zeid JL, Greenwald MJ, Strauss LC, Goldman S (March 2008). “Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma”. Pediatr Blood Cancer. 50 (3): 567–72. doi:10.1002/pbc.21301. PMID 17729249.
↑ Novetsky DE, Abramson DH, Kim JW, Dunkel IJ (March 2009). “Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies–an analysis of impact”. Ophthalmic Genet. 30 (1): 40–4. doi:10.1080/13816810802452168. PMID 19172510.
↑ 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.
↑ Murphree, A. Linn; Chantada, Guillermo L. (2015). “Retinoblastoma: Staging and Grouping”: 29–37. doi:10.1007/978-3-662-43451-2_3.
↑ “TNM8: The updated TNM classification for retinoblastoma”. Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.

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[pmid23787805-1] Chantada GL, Sampor C, Bosaleh A, Solernou V, Fandiño A, de Dávila MT (September 2013). “Comparison of staging systems for extraocular retinoblastoma: analysis of 533 patients”. JAMA Ophthalmol. 131 (9): 1127–34. doi:10.1001/jamaophthalmol.2013.260. PMID 23787805.

[pmid16996605-2] 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.

[pmid17729249-3] Zage PE, Reitman AJ, Seshadri R, Weinstein JL, Mets MB, Zeid JL, Greenwald MJ, Strauss LC, Goldman S (March 2008). “Outcomes of a two-drug chemotherapy regimen for intraocular retinoblastoma”. Pediatr Blood Cancer. 50 (3): 567–72. doi:10.1002/pbc.21301. PMID 17729249.

[pmid19172510-4] Novetsky DE, Abramson DH, Kim JW, Dunkel IJ (March 2009). “Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies–an analysis of impact”. Ophthalmic Genet. 30 (1): 40–4. doi:10.1080/13816810802452168. PMID 19172510.

[pmid15763190-5] 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.

[MurphreeChantada2015-6] Murphree, A. Linn; Chantada, Guillermo L. (2015). “Retinoblastoma: Staging and Grouping”: 29–37. doi:10.1007/978-3-662-43451-2_3.

[pmid29915471-7] “TNM8: The updated TNM classification for retinoblastoma”. Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.

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