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13-04-2024 | Retinoblastoma | Review Article

Management of Intraocular Retinoblastoma: ICMR Consensus Guidelines

Authors: Rachna Meel, Suyash Kulkarni, Lata Singh, Girish Chinnaswamy, Venkatraman Radhakrishnan, Renu Madan, Archana Sasi, Tanvir Kaur, R. S. Dhaliwal, Sameer Bakhshi

Published in: Indian Journal of Pediatrics

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Abstract

Retinoblastoma (RB) is the most common childhood intraocular malignancy. Delayed presentation due to a lack of awareness and advanced intraocular tumors are a common scenario in low-middle income countries (LMICs). Remarkable treatment advances have been made in the past few decades allowing globe salvage in advanced intraocular RB (IORB) including systemic chemotherapy with focal consolidation and targeted treatments like intraarterial chemotherapy and intravitreal chemotherapy. However, a lack of availability and affordability limits the use of such advances in LMICs. External beam radiotherapy, despite risk of second cancers in RB with germline mutations, still remains useful for recalcitrant RB not responding to any other treatment. When choosing conservative treatment for advanced IORB, the cost and long duration of treatment, morbidity from multiple evaluation under anesthesias (EUAs), side effects of treatment and risk of treatment failure need to be taken into account and discussed with the parents. In this article, the authors discuss the ICMR consensus guidelines on the management of IORB.
Literature
2.
go back to reference Meel R, Radhakrishnan V, Bakhshi S. Current therapy and recent advances in the management of retinoblastoma. Indian J Med Paediatr Oncol. 2012;33:80–8.PubMedPubMedCentralCrossRef Meel R, Radhakrishnan V, Bakhshi S. Current therapy and recent advances in the management of retinoblastoma. Indian J Med Paediatr Oncol. 2012;33:80–8.PubMedPubMedCentralCrossRef
4.
go back to reference Shields CL, Mashayekhi A, Au AK, et al. The international classification of retinoblastoma predicts chemoreduction success. Ophthalmology. 2006;113:2276–80.PubMedCrossRef Shields CL, Mashayekhi A, Au AK, et al. The international classification of retinoblastoma predicts chemoreduction success. Ophthalmology. 2006;113:2276–80.PubMedCrossRef
5.
go back to reference Novetsky DE, Abramson DH, Kim JW, Dunkel IJ. Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies—an analysis of impact. Ophthalmic Genet. 2009;30:40–4.PubMedCrossRef Novetsky DE, Abramson DH, Kim JW, Dunkel IJ. Published international classification of retinoblastoma (ICRB) definitions contain inconsistencies—an analysis of impact. Ophthalmic Genet. 2009;30:40–4.PubMedCrossRef
6.
go back to reference Kivelä T. Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. J Clin Oncol. 1999;17:1829–37.PubMedCrossRef Kivelä T. Trilateral retinoblastoma: a meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. J Clin Oncol. 1999;17:1829–37.PubMedCrossRef
7.
go back to reference Shields CL, Meadows AT, Shields JA, Carvalho C, Smith AF. Chemoreduction for retinoblastoma may prevent intracranial neuroblastic malignancy (trilateral retinoblastoma). Arch Ophthalmol. 2001;119:1269–72.PubMedCrossRef Shields CL, Meadows AT, Shields JA, Carvalho C, Smith AF. Chemoreduction for retinoblastoma may prevent intracranial neuroblastic malignancy (trilateral retinoblastoma). Arch Ophthalmol. 2001;119:1269–72.PubMedCrossRef
8.
go back to reference Roarty JD, McLean IW, Zimmerman LE. Incidence of second neoplasms in patients with bilateral retinoblastoma. Ophthalmology. 1988;95:1583–7.PubMedCrossRef Roarty JD, McLean IW, Zimmerman LE. Incidence of second neoplasms in patients with bilateral retinoblastoma. Ophthalmology. 1988;95:1583–7.PubMedCrossRef
9.
go back to reference Singh U, Katoch D, Kaur S, Dogra MR, Bansal D, Kapoor R. Retinoblastoma: a sixteen-year review of the presentation, treatment, and outcome from a tertiary care institute in northern India. Ocul Oncol Pathol. 2017;4:23–32.PubMedPubMedCentralCrossRef Singh U, Katoch D, Kaur S, Dogra MR, Bansal D, Kapoor R. Retinoblastoma: a sixteen-year review of the presentation, treatment, and outcome from a tertiary care institute in northern India. Ocul Oncol Pathol. 2017;4:23–32.PubMedPubMedCentralCrossRef
10.
go back to reference Kaliki S, Patel A, Iram S, Ramappa G, Mohamed A, Palkonda VAR. Retinoblastoma in India: clinical presentation and outcome in 1,457 patients (2,074 eyes). Retina. 2019;39:379–91.PubMedCrossRef Kaliki S, Patel A, Iram S, Ramappa G, Mohamed A, Palkonda VAR. Retinoblastoma in India: clinical presentation and outcome in 1,457 patients (2,074 eyes). Retina. 2019;39:379–91.PubMedCrossRef
11.
go back to reference Shields CL, Manjandavida FP, Lally SE, et al. Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma. Ophthalmology. 2014;121:1453–60.PubMedCrossRef Shields CL, Manjandavida FP, Lally SE, et al. Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma. Ophthalmology. 2014;121:1453–60.PubMedCrossRef
12.
go back to reference Meel R, Bakhshi S, Pushker N, Vishnubhatla S. Randomized, controlled trial in groups C and D retinoblastoma. Ophthalmology. 2015;122:433–5.PubMedCrossRef Meel R, Bakhshi S, Pushker N, Vishnubhatla S. Randomized, controlled trial in groups C and D retinoblastoma. Ophthalmology. 2015;122:433–5.PubMedCrossRef
14.
go back to reference Fabian ID, Stacey AW, Johnson KC, et al. Primary enucleation for group D retinoblastoma in the era of systemic and targeted chemotherapy: the price of retaining an eye. Br J Ophthalmol. 2018;102:265–71.PubMedCrossRef Fabian ID, Stacey AW, Johnson KC, et al. Primary enucleation for group D retinoblastoma in the era of systemic and targeted chemotherapy: the price of retaining an eye. Br J Ophthalmol. 2018;102:265–71.PubMedCrossRef
15.
go back to reference Scelfo C, Francis JH, Khetan V, et al. An international survey of classification and treatment choices for group D retinoblastoma. Int J Ophthalmol. 2017;10:961–7.PubMedPubMedCentral Scelfo C, Francis JH, Khetan V, et al. An international survey of classification and treatment choices for group D retinoblastoma. Int J Ophthalmol. 2017;10:961–7.PubMedPubMedCentral
16.
go back to reference Kiratli H, Koç İ, Inam O, Varan A, Akyüz C. Retrospective analysis of primarily treated group D retinoblastoma. Graefes Arch Clin Exp Ophthalmol. 2018;256:2225–31.PubMedCrossRef Kiratli H, Koç İ, Inam O, Varan A, Akyüz C. Retrospective analysis of primarily treated group D retinoblastoma. Graefes Arch Clin Exp Ophthalmol. 2018;256:2225–31.PubMedCrossRef
17.
go back to reference Abramson DH, Dunkel IJ, Brodie SE, Kim JW, Gobin YP. A phase I/II study of direct intraarterial (ophthalmic artery) chemotherapy with melphalan for intraocular retinoblastoma initial results. Ophthalmology. 2008;115:1398–404.PubMedCrossRef Abramson DH, Dunkel IJ, Brodie SE, Kim JW, Gobin YP. A phase I/II study of direct intraarterial (ophthalmic artery) chemotherapy with melphalan for intraocular retinoblastoma initial results. Ophthalmology. 2008;115:1398–404.PubMedCrossRef
19.
go back to reference Shields CL, Ramasubramanian A, Thangappan A, et al. Chemoreduction for group E retinoblastoma: comparison of chemoreduction alone versus chemoreduction plus low-dose external radiotherapyin 76 eyes. Ophthalmology. 2009;116:544–51.PubMedCrossRef Shields CL, Ramasubramanian A, Thangappan A, et al. Chemoreduction for group E retinoblastoma: comparison of chemoreduction alone versus chemoreduction plus low-dose external radiotherapyin 76 eyes. Ophthalmology. 2009;116:544–51.PubMedCrossRef
20.
go back to reference Rao R, Honavar SG, Reddy VP. Preservation of retinoblastoma group E eyes with neovascular glaucoma using intravenous chemotherapy: risk factors and outcomes. Br J Ophthalmol. 2019;103:1856–61.PubMed Rao R, Honavar SG, Reddy VP. Preservation of retinoblastoma group E eyes with neovascular glaucoma using intravenous chemotherapy: risk factors and outcomes. Br J Ophthalmol. 2019;103:1856–61.PubMed
21.
go back to reference Weng PY, Chen SH, Kao LY, et al. Clinical spectrum and treatment outcome of retinoblastoma with Group D and E diseases: a single institution retrospective review. Medicine. 2020;99:e22201.PubMedPubMedCentralCrossRef Weng PY, Chen SH, Kao LY, et al. Clinical spectrum and treatment outcome of retinoblastoma with Group D and E diseases: a single institution retrospective review. Medicine. 2020;99:e22201.PubMedPubMedCentralCrossRef
22.
go back to reference Temming P, Viehmann A, Biewald E, Lohmann DR. Sporadic unilateral retinoblastoma or first sign of bilateral disease? Br J Ophthalmol. 2013;97:475–80.PubMedCrossRef Temming P, Viehmann A, Biewald E, Lohmann DR. Sporadic unilateral retinoblastoma or first sign of bilateral disease? Br J Ophthalmol. 2013;97:475–80.PubMedCrossRef
23.
go back to reference Abramson DH, Servodidio CA. Retinoblastoma in the first year of life. Ophthalmic Paediatr Genet. 1992;13:191–203.PubMedCrossRef Abramson DH, Servodidio CA. Retinoblastoma in the first year of life. Ophthalmic Paediatr Genet. 1992;13:191–203.PubMedCrossRef
24.
go back to reference Hussain Z. Impact of laterality on cumulative survival in patients diagnosed with retinoblastoma: a retrospective cohort analysis of 1925 cases in the surveillance, epidemiology, and end results (SEER) program. Clin Ophthalmol. 2021;15:991–1001.PubMedPubMedCentralCrossRef Hussain Z. Impact of laterality on cumulative survival in patients diagnosed with retinoblastoma: a retrospective cohort analysis of 1925 cases in the surveillance, epidemiology, and end results (SEER) program. Clin Ophthalmol. 2021;15:991–1001.PubMedPubMedCentralCrossRef
25.
26.
go back to reference Kashyap S, Meel R, Pushker N, et al. Phthisis bulbi in retinoblastoma. Clin Exp Ophthalmol. 2011;39:105–10.PubMedCrossRef Kashyap S, Meel R, Pushker N, et al. Phthisis bulbi in retinoblastoma. Clin Exp Ophthalmol. 2011;39:105–10.PubMedCrossRef
27.
go back to reference Mallipatna AC, Sutherland JE, Gallie BL, Chan H, Héon E. Management and outcome of unilateral retinoblastoma. J AAPOS. 2009;13:546–50.PubMedCrossRef Mallipatna AC, Sutherland JE, Gallie BL, Chan H, Héon E. Management and outcome of unilateral retinoblastoma. J AAPOS. 2009;13:546–50.PubMedCrossRef
28.
go back to reference El Zomor H, Nour R, Saad A, et al. Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients. Eye (Lond). 2021;35:2564–72.PubMedCrossRef El Zomor H, Nour R, Saad A, et al. Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients. Eye (Lond). 2021;35:2564–72.PubMedCrossRef
30.
go back to reference Fabian ID, Shah V, Kapelushnik N, et al. Examinations under anaesthesia as a measure of disease burden in unilateral retinoblastoma: the London experience. Br J Ophthalmol. 2020;104:17–22.PubMedCrossRef Fabian ID, Shah V, Kapelushnik N, et al. Examinations under anaesthesia as a measure of disease burden in unilateral retinoblastoma: the London experience. Br J Ophthalmol. 2020;104:17–22.PubMedCrossRef
31.
go back to reference Chantada G, Luna-Fineman S, Sitorus RS, et al; SIOP-PODC Graduated-Intensity Retinoblastoma Guidelines Writing Committee. SIOP-PODC recommendations for graduated-intensity treatment of retinoblastoma in developing countries. Pediatr Blood Cancer. 2013;60:719–27.PubMedCrossRef Chantada G, Luna-Fineman S, Sitorus RS, et al; SIOP-PODC Graduated-Intensity Retinoblastoma Guidelines Writing Committee. SIOP-PODC recommendations for graduated-intensity treatment of retinoblastoma in developing countries. Pediatr Blood Cancer. 2013;60:719–27.PubMedCrossRef
32.
go back to reference Kaliki S, Shields CL, Eagle RC Jr, Iram S, Shields JA. High-risk intraocular retinoblastoma: comparison between Asian Indians and Americans from two major referral centers. Retina. 2018;38:2023–9.PubMedCrossRef Kaliki S, Shields CL, Eagle RC Jr, Iram S, Shields JA. High-risk intraocular retinoblastoma: comparison between Asian Indians and Americans from two major referral centers. Retina. 2018;38:2023–9.PubMedCrossRef
33.
go back to reference Kashyap S, Meel R, Pushker N, et al. Clinical predictors of high risk histopathology in retinoblastoma. Pediatr Blood Cancer. 2012;58:356–61.PubMedCrossRef Kashyap S, Meel R, Pushker N, et al. Clinical predictors of high risk histopathology in retinoblastoma. Pediatr Blood Cancer. 2012;58:356–61.PubMedCrossRef
34.
go back to reference Kashyap S, Sethi S, Meel R, et al. A histopathologic analysis of eyes primarily enucleated for advanced intraocular retinoblastoma from a developing country. Arch Pathol Lab Med. 2012;136:190–3.PubMedCrossRef Kashyap S, Sethi S, Meel R, et al. A histopathologic analysis of eyes primarily enucleated for advanced intraocular retinoblastoma from a developing country. Arch Pathol Lab Med. 2012;136:190–3.PubMedCrossRef
35.
go back to reference Sastre X, Chantada GL, Doz F, et al; International Retinoblastoma Staging Working Group. Proceedings of the consensus meetings from the International Retinoblastoma Staging Working Group on the pathology guidelines for the examination of enucleated eyes and evaluation of prognostic risk factors in retinoblastoma. Arch Pathol Lab Med. 2009;133:1199–202.PubMedCrossRef Sastre X, Chantada GL, Doz F, et al; International Retinoblastoma Staging Working Group. Proceedings of the consensus meetings from the International Retinoblastoma Staging Working Group on the pathology guidelines for the examination of enucleated eyes and evaluation of prognostic risk factors in retinoblastoma. Arch Pathol Lab Med. 2009;133:1199–202.PubMedCrossRef
36.
go back to reference Kashyap S, Singh L, Kumar N, et al. Combined association of massive choroidal and optic nerve invasion as a prognostic relevance in primary retinoblastoma: a 10-year study. Asia Pac J Clin Oncol. 2021;17:e100–8.PubMedCrossRef Kashyap S, Singh L, Kumar N, et al. Combined association of massive choroidal and optic nerve invasion as a prognostic relevance in primary retinoblastoma: a 10-year study. Asia Pac J Clin Oncol. 2021;17:e100–8.PubMedCrossRef
37.
go back to reference Messmer EP, Heinrich T, Höpping W, de Sutter E, Havers W, Sauerwein W. Risk factors for metastasis in patients with retinoblastoma. Ophthalmology. 1991;98:136–41.PubMedCrossRef Messmer EP, Heinrich T, Höpping W, de Sutter E, Havers W, Sauerwein W. Risk factors for metastasis in patients with retinoblastoma. Ophthalmology. 1991;98:136–41.PubMedCrossRef
38.
go back to reference Honavar SG, Singh AD, Shields CL, et al. Postenucleation adjuvant therapy in high-risk retinoblastoma. Arch Ophthalmol. 2002;120:923–31.PubMedCrossRef Honavar SG, Singh AD, Shields CL, et al. Postenucleation adjuvant therapy in high-risk retinoblastoma. Arch Ophthalmol. 2002;120:923–31.PubMedCrossRef
39.
go back to reference Kaliki S, Shields CL, Shah SU, et al. Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma. Arch Ophthalmol. 2011;129:1422–7.PubMedCrossRef Kaliki S, Shields CL, Shah SU, et al. Postenucleation adjuvant chemotherapy with vincristine, etoposide, and carboplatin for the treatment of high-risk retinoblastoma. Arch Ophthalmol. 2011;129:1422–7.PubMedCrossRef
40.
go back to reference Ye H, Du Y, Chen R, et al. The potential benefit of three vs. six cycles of carboplatin, etoposide, and vincristine in post enucleation high-risk patients with IRSS stage I retinoblastoma. Curr Eye Res. 2016;41:1507–12.PubMedCrossRef Ye H, Du Y, Chen R, et al. The potential benefit of three vs. six cycles of carboplatin, etoposide, and vincristine in post enucleation high-risk patients with IRSS stage I retinoblastoma. Curr Eye Res. 2016;41:1507–12.PubMedCrossRef
41.
go back to reference Rishi P, Agarwal A, Chatterjee P, et al. Intra-arterial chemotherapy for retinoblastoma: four-year results from tertiary center in India. Ocul Oncol Pathol. 2020;6:66–73.PubMedCrossRef Rishi P, Agarwal A, Chatterjee P, et al. Intra-arterial chemotherapy for retinoblastoma: four-year results from tertiary center in India. Ocul Oncol Pathol. 2020;6:66–73.PubMedCrossRef
42.
go back to reference Francis JH, Abramson DH, Ji X, et al. Risk of extraocular extension in eyes with retinoblastoma receiving intravitreous chemotherapy. JAMA Ophthalmol. 2017;135:1426–9.PubMedPubMedCentralCrossRef Francis JH, Abramson DH, Ji X, et al. Risk of extraocular extension in eyes with retinoblastoma receiving intravitreous chemotherapy. JAMA Ophthalmol. 2017;135:1426–9.PubMedPubMedCentralCrossRef
43.
go back to reference Mohanan A, Meel R, Venkatesh P, Kashyap S. Retinal tumour outcome with IVC in retinoblastoma. Eye. 2021;35:2046–7.PubMedCrossRef Mohanan A, Meel R, Venkatesh P, Kashyap S. Retinal tumour outcome with IVC in retinoblastoma. Eye. 2021;35:2046–7.PubMedCrossRef
44.
go back to reference Kaliki S, Mittal P, Mohan S, et al. Bilateral advanced (group D or E) intraocular retinoblastoma: outcomes in 72 Asian Indian patients. Eye (Lond). 2019;33:1297–304.PubMedCrossRef Kaliki S, Mittal P, Mohan S, et al. Bilateral advanced (group D or E) intraocular retinoblastoma: outcomes in 72 Asian Indian patients. Eye (Lond). 2019;33:1297–304.PubMedCrossRef
45.
go back to reference Abramson DH, Marr BP, Francis JH, et al. Simultaneous bilateral ophthalmic artery chemosurgery for bilateral retinoblastoma (Tandem therapy). PLoS One. 2016;11:e0156806.PubMedPubMedCentralCrossRef Abramson DH, Marr BP, Francis JH, et al. Simultaneous bilateral ophthalmic artery chemosurgery for bilateral retinoblastoma (Tandem therapy). PLoS One. 2016;11:e0156806.PubMedPubMedCentralCrossRef
Metadata
Title
Management of Intraocular Retinoblastoma: ICMR Consensus Guidelines
Authors
Rachna Meel
Suyash Kulkarni
Lata Singh
Girish Chinnaswamy
Venkatraman Radhakrishnan
Renu Madan
Archana Sasi
Tanvir Kaur
R. S. Dhaliwal
Sameer Bakhshi
Publication date
13-04-2024
Publisher
Springer India
Published in
Indian Journal of Pediatrics
Print ISSN: 0019-5456
Electronic ISSN: 0973-7693
DOI
https://doi.org/10.1007/s12098-024-05095-0