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==Diagnostic Study of Choice==
 
==Diagnostic Study of Choice==
 
===Study of Choice===
 
===Study of Choice===
 
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[[Ultrasound imaging]] is the gold standard test for the diagnosis of retinoblastoma. However, MRI is recommended for the following goals:<ref name="SyedRamasubramanian2017">{{cite journal|last1=Syed|first1=Reema|last2=Ramasubramanian|first2=Aparna|title=Advances in Management of Retinoblastoma|journal=Advances in Ophthalmology and Optometry|volume=2|issue=1|year=2017|pages=87–100|issn=24521760|doi=10.1016/j.yaoo.2017.03.005}}</ref>
 +
*Confirmation
 +
*Evaluation of local tumor extent
 +
*Assessment of associated developmental malformation of the brain
 +
*Detection of trilateral retinoblastoma (pinealoblastoma)
 +
Ultrasound biomicroscopy (UBM) has been observed to have 81% sensitivity and 100% specificity in the evaluation of the anterior extension of tumor and assessment of posterior chamber involvement.<ref name="MoulinGaillard2012">{{cite journal|last1=Moulin|first1=Alexandre P|last2=Gaillard|first2=Marie-Claire|last3=Balmer|first3=Aubin|last4=Munier|first4=Francis L|title=Ultrasound biomicroscopy evaluation of anterior extension in retinoblastoma: a clinicopathological study|journal=British Journal of Ophthalmology|volume=96|issue=3|year=2012|pages=337–340|issn=0007-1161|doi=10.1136/bjophthalmol-2011-300051}}</ref>
 
===Staging===
 
===Staging===
 
A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. There are several [[classification]] system available for [[retinoblastoma]].. For intraocular [[diseases]] the available grouping systems include the International Intraocular [[Retinoblastoma]] [[Classification]] (IIRC), Intraocular [[Classification]] of Retinoblastoma (ICRB) and [[TNM|cTNMH systems]]. For extraocular [[diseases]], the International [[Retinoblastoma]] [[Cancer staging|Staging]] System (IRSS) and [[TNM Staging System|cTNMH]] schemes can be used.
 
A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. There are several [[classification]] system available for [[retinoblastoma]].. For intraocular [[diseases]] the available grouping systems include the International Intraocular [[Retinoblastoma]] [[Classification]] (IIRC), Intraocular [[Classification]] of Retinoblastoma (ICRB) and [[TNM|cTNMH systems]]. For extraocular [[diseases]], the International [[Retinoblastoma]] [[Cancer staging|Staging]] System (IRSS) and [[TNM Staging System|cTNMH]] schemes can be used.

Revision as of 20:22, 14 June 2019

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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

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

Ultrasound imaging is the gold standard test for the diagnosis of retinoblastoma. However, MRI is recommended for the following goals:[1]

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

Ultrasound biomicroscopy (UBM) has been observed to have 81% sensitivity and 100% specificity in the evaluation of the anterior extension of tumor and assessment of posterior chamber involvement.[2]

Staging

A common method of retinoblastoma classification is critical to plan treatment, evaluate prognosis and compare outcomes. There are several classification system available for retinoblastoma.. 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. Retinoblastoma is classified in two subgroups:
Extraocular disease

  • This category first subclassified via International Retinoblastoma Staging System different staging system to 0–IV.[3]
    • This sub-classification is established by Chantada and colleagues in 2006.
      • This staging system is used in the clinical setting greater than the AJCC staging system (Please see below).
International Retinoblastoma Staging System
Stage Description
Stage 0 Eye enucleated and no dissemination of the disease
Stage I Eye enucleated, completely resected histologically
Stage II Eye enucleated, microscopic residual tumor
Stage III Regional extension a.Overt orbital disease

b.Preauricular or cervical lymph node extension

Stage IV Metastatic disease a.Hematogenous metastasis (without CNS involvement)

—Single lesion

—Multiple lesions

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

—Prechiasmatic lesion

CNS mass

—Leptomeningeal and CSF disease

  • Intraocular disease which first classified by Reese-Ellsworth Classification method in the 1960s and used to predict the survival chance of salvaging eye following external beam radiotherapy.
    • This classification replaced by International Intraocular Retinoblastoma Classification (IIRC) scheme following the introduction of chemotherapy for the treatment of retinoblastoma in 1990.[4]
  • IIRC grouping system sub-classifies the tumor according to from A to E according to tumor characteristics such as:

In 2006, Intraocular Classification of Retinoblastoma (ICRB), has been developed by Dr. Shields and colleagues.[5]

  • This classification is modified version of IIRC and the main difference is in advanced categories of the tumor (D and E).
  • This classification method has been observed to be successful at predicting the outcome of chemotherapy.
  • IIRC and ICRB are now the most commonly used classification system for retinoblastoma.[6]
  • American Joint Committee on Cancer (AJCC) staging system is another suggested grouping system for the classification of the tumor using TNM system.
    • This system is not frequently used for pediatrics and is not grouped according to the prognostic values.
American Joint Committee on Cancer (AJCC) staging system
The table below adopted from Community Eye Health Journal [7]
Primary tumour (cT)*
Stage Sub-stage Finding
cTX Unknown evidence of intraocular tumor
cT0 No evidence of intraocular tumor
cT1 a Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor

Tumors ≤ 3mm and further than 1.5 mm from the disc and fovea

cT1 b Intraocular tumor(s) with sub-retinal fluid ≤ 5mm from the base of any tumor

Tumors > 3 mm or closer than 1.5 mm to the disc and fovea

cT2 a Intraocular tumor(s) with retinal detachment, vitreous seeding or sub-retinal seeding

Sub-retinal fluid > 5 mm from the base of any tumor

cT2 b Intraocular tumor(s) with retinal detachment, vitreous seeding or sub-retinal seeding

Tumors with vitreous seeding and/or sub-retinal seeding

cT3 a Advanced intraocular tumor(s)

Phthisis or pre-phthisis bulbi

cT3 b Advanced intraocular tumor(s)

Tumor invasion of the pars plana, ciliary body, lens, zonules, iris or anterior chamber

cT3 c Advanced intraocular tumor(s)

Raised intraocular pressure with neovascularization and/or buphthalmos

cT3 d Advanced intraocular tumor(s)

Hyphema and/or massive vitreous hemorrhage

cT3 e Advanced intraocular tumor(s)

Aseptic orbital cellulitis

cT4 a Extraocular tumor(s) involving the orbit, including the optic nerve

Radiological evidence of retrobulbar optic nerve involvement or thickening of the optic nerve or involvement of the orbital tissues

cT4 b Extraocular tumor(s) involving the orbit, including the optic nerve

Extraocular tumor clinically evident with proptosis and orbital mass

Regional lymph nodes (cN)
Stage Sub-stage Finding
cNX Regional lymph nodes cannot be assessed
cN0 No regional lymph node involvement
cN1 Evidence of preauricular, submandibular, and cervical lymph node involvement
Distant metastasis
Stage Sub-stage Finding
cM0 No signs or symptoms of intracranial or distant metastasis
cM1 a Distant metastasis without microscopic confirmation

Tumor(s) involving any distant site (e.g. bone marrow, liver) on clinical or radiological tests

cM1 b Distant metastasis without microscopic confirmation

Tumour involving the central nervous system on radiological imaging (not including trilateral retinoblastoma)

pM1 a Distant metastasis with microscopic confirmation

Histopathological confirmation of tumor at any distant site (e.g. bone marrow, liver, or other)

pM1 b Distant metastasis with microscopic confirmation

Histopathological confirmation of tumor in the cerebrospinal fluid or CNS parenchyma

Heritable trait (H)
Stage Sub-stage Finding
HX Unknown or insufficient evidence of a constitutional RB1 gene mutation
H0 Normal RB1 alleles in blood tested with demonstrated high sensitivity assays
H1 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

References

  1. 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.
  2. 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.
  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.


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