Retinoblastoma classification

Jump to navigation Jump to search

Retinoblastoma Microchapters


Patient Information


Historical perspective




Differentiating Retinoblastoma from other Diseases

Epidemiology & Demographics

Risk Factors


Natural history, Complications, and Prognosis


Diagnostic Study of Choice

History & Symptoms

Physical Examination

Laboratory Findings



Echocardiography and Ultrasound

CT scan


Other Imaging Findings

Other Diagnostic Studies


Medical Therapy


Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Retinoblastoma classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides


American Roentgen Ray Society Images of Retinoblastoma classification

All Images
Echo & Ultrasound
CT Images

Ongoing Trials at Clinical

US National Guidelines Clearinghouse

NICE Guidance

FDA on Retinoblastoma classification

CDC on Retinoblastoma classification

Retinoblastoma classification in the news

Blogs on Retinoblastoma classification

Directions to Hospitals Treating Retinoblastoma

Risk calculators and risk factors for 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]


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.


Intraocular Retinoblastoma Classification System

Intraocular Classifications of Retinoblastoma and their Features

International Intraocular Retinoblastoma Classification (IIRC) Intraocular Classification of Retinoblastoma (ICRB)
Group A

(very low risk)

  • Tumors ≤ 3 mm (in basal dimension or thickness)
Group B

(low risk)

  • 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)

Group D

(high risk)

Group E

(very high risk)

Reese-Ellsworth Classification for Intraocular Tumors[5]

Reese-Ellsworth Classification[6]

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
  • Undetermind
  • b
  • Solitary tumor larger than 15 mm, behind the equator
  • Undetermined
Group IV
  • a
  • Multiple tumors, some larger than 15 mm
  • Unfavorable
  • b
  • Unfavorable
Group V
  • a
  • Very unfavorable
  • b
  • 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]


  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.
  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.
  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.
  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.
  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.
  6. Murphree, A. Linn; Chantada, Guillermo L. (2015). "Retinoblastoma: Staging and Grouping": 29–37. doi:10.1007/978-3-662-43451-2_3.
  7. "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.

Template:WH Template:WS