Retinoblastoma diagnostic study of choice

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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]


Ultrasound imaging is the gold standard test for the diagnosis of retinoblastoma. MRI can also be helpful in the diagnosis making. A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. Available grouping systems include the International Intraocular Retinoblastoma Classification (IIRC), Intraocular Classification of Retinoblastoma (ICRB) and cTNMH systems diseases.

Diagnostic Study of Choice

Study of Choice

    • Confirmation of the diagnosis
    • Evaluation of local tumor extent
    • Assessment of associated developmental malformation of the brain
    • Detection of trilateral retinoblastoma (pinealoblastoma)


Extraocular Disease

  • This category is sub-classified via International Retinoblastoma Staging System which is given below:[3]

International Retinoblastoma Staging System

Stage Description
Stage 0
  • Eye enucleated and no dissemination of the disease
Stage I
Stage II
Stage III
  • Regional extension
a. Overt orbital disease

b. Preauricular or cervical lymph node extension

Stage IV a. Hematogenous metastasis (without CNS involvement)

b. CNS extension (with or without any other site of regional or metastatic disease)

Intraocular Disease

  • The intraocular disease can be sub-classified by International Intraocular Retinoblastoma Classification (IIRC).[4]
  • In 2006, Intraocular Classification of Retinoblastoma (ICRB) was developed by Dr. Shields and colleagues.[5]
  • IIRC and ICRB are now the most commonly used classification systems for retinoblastoma.[6]
American Joint Committee on Cancer (AJCC) Staging System
The table below is adopted from Community Eye Health Journal [7]
Primary tumour (cT)*
Stage Sub-stage Finding
  • Unknown evidence of intraocular tumor
  • No evidence of intraocular tumor
cT1 a
  • Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor
cT1 b
  • Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor
cT2 a
  • Sub-retinal fluid > 5 mm from the base of any tumor
cT2 b
cT3 a
  • Advanced intraocular tumor(s)
cT3 b
  • Advanced intraocular tumor(s)
cT3 c
  • Advanced intraocular tumor(s)
cT3 d
  • Advanced intraocular tumor(s)
cT3 e
  • Advanced intraocular tumor(s)
cT4 a
cT4 b
Regional lymph nodes (cN)
Stage Sub-stage Finding
Distant metastasis
Stage Sub-stage Finding
cM1 a
cM1 b
  • Tumor involving the central nervous system on radiological imaging (not including trilateral retinoblastoma)
pM1 a
pM1 b
Heritable trait (H)
Stage Sub-stage Finding
  • 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


  1. Moulin, Alexandre P; Gaillard, Marie-Claire; Balmer, Aubin; Munier, Francis L (2012). "Ultrasound biomicroscopy evaluation of anterior extension in retinoblastoma: a clinicopathological study". British Journal of Ophthalmology. 96 (3): 337–340. doi:10.1136/bjophthalmol-2011-300051. ISSN 0007-1161.
  2. Syed, Reema; Ramasubramanian, Aparna (2017). "Advances in Management of Retinoblastoma". Advances in Ophthalmology and Optometry. 2 (1): 87–100. doi:10.1016/j.yaoo.2017.03.005. ISSN 2452-1760.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. "TNM8: The updated TNM classification for retinoblastoma". Community Eye Health. 31 (101): 34. 2018. PMC 5998398. PMID 29915471.