Retinoblastoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Retinoblastoma may be classified into several subgroups based on the International Retinoblastoma Staging System (IRSS).[1] [2][3]

Classification

Retinoblastoma has been classified according to different classification system for variable purpuses.[4]
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)
  • 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).[5]

  • 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 stageing systm click here.

Reese-Ellsworth Classification for Intraocular Tumors is another staging system for retinoblastoma.[3]

  • 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.


The International Classification of Retinoblastoma staging system [6][7][8]
Groups Features
Group A
  • Small intraretinal tumors away from foveola and optic nerve
    • 3mm or smaller in the greatest dimension, confined to retina
    • Located further than 3 mm from the foveola and 1.5 mm from the optic disc.
Group B
  • Tumors confined to the retina.
    • Not in the group A
    • Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
Group C
  • Local disease with minimal subretinal or vitreous seeding with following caracteristics:
    • 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
Group D
  • Diffuse tumor with significant vitreous or subretinal seeding
    • May be 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
Group E
  • 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


References

  1. Pratt CB, Fontanesi J, Lu X, Parham DM, Elfervig J, Meyer D (1997). "Proposal for a New Staging Scheme for Intraocular and Extraocular Retinoblastoma Based on an Analysis of 103 Globes". Oncologist. 2 (1): 1–5. PMID 10388023.
  2. Shields CL, Mashayekhi A, Demirci H, Meadows AT, Shields JA (2004). "Practical approach to management of retinoblastoma". Arch Ophthalmol. 122 (5): 729–35. doi:10.1001/archopht.122.5.729. PMID 15136321.
  3. 3.0 3.1 Linn Murphree A (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.
  4. 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.
  5. "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.
  6. 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.
  7. 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.
  8. 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.

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