Retinoblastoma classification

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

Classification

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

• Intra-ocular retinoblastoma classification system includes:
  • International Intraocular Retinoblastoma Classification (IIRC)
  • 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 further than 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 retinoblastoma
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 prognosis 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, anterior chamber

Reese-Ellsworth Classification for Intraocular tumors is another staging system for retinoblastoma.

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

Stage	Sub-stage	Features	Prognosis
Group I	a	Solitory tumor < 6 mm at or behind the equator	Very favorable
	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
	b	Multiple tumors, 6 to 15 mm, behind the equator	Favorable
Group III	a	Lesion anterior to the equator	Undetermind
	b	Solitary tumor larger than 15 mm, behind the equator	Undetermined
Group IV	a
	b
Group V	a
	b

• Extra-ocular retinoblastoma classification system are such as:
  • International retinoblastoma staging system
  • cTNMH system of American Joint Committee on Cancer (AJCC)

the cTNMH system has been developed by the American Joint Committee on Cancer (AJCC).

• The tumor classified according to the size of the primary tumor (T), lymph node involvement (N) and presence of systemic metastasis (M).
• The recently edited guideline added H for retinoblastoma as the hereditary component for Rb gene.
• This category also includes a pathological subgroup (pTNM) which is frequently used by the ophthalmic pathologist.
• To see the full staging system click here.