A single-center retrospective clinical study on comprehensive treatment for 157 patients with retinoblastoma_Chinese Journal of Ocular Fundus Diseases

Authors：

Zou Yihua ¹ ,  Ji Xunda ¹ , Li Jiakai ¹ , Liang Tingyi ¹ , Hua Xuming ² , Zhao Peiquan ¹

1. Department of Ophthalmology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China;
2. Department of Neurosurgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China;

Corresponding author：

Ji Xunda, Email: jixunda@xinhuamed.com.cn

Keywords：

Retinoblastoma; Comprehensive treatment; Intravenous chemotherapy; Intra-arterial chemotherapy; Intravitreous chemotherapy

DOI：

10.3760/cma.j.cn511434-20230801-00326

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Objective To evaluate the efficacy and safety of comprehensive treatment for retinoblastoma (RB). Methods A retrospective clinical study. From January to December in 2019, 157 cases (203 eyes) of RB who were diagnosed by the Department of Ophthalmology of Xinhua Hospital and received comprehensive treatment were included in this study. Of cases, 76 were male, and 81 were female; 111 were unilateral, and 46 were bilateral. The medium of age at diagnosis was 20.1 months. All patients received treatment for the first time. Patients with intraocular tumors were divided into A-E stages, extraocular stage and distant metastasis according to international intraocular RB classification standard. The median follow-up time was 37.4 months. Clinical features, treatment, prognosis and ocular complications of all cases were recorded. Results Among 157 cases (203 eyes), 137 cases (180 eyes) were in intraocular stage; 6, 14, 10, 98, and 52 of eyes were in A-E stages, respectively. Twelve cases (12 eyes) were in extraocular stage; 8 cases (11 eyes) were in distant metastasis stag; 8 cases died due to distant metastasis; 149 cases (94.9%, 149/157) survived; 48 eyes were enucleated, 34 of which underwent initial enucleation, and 14 eyes underwent enucleation after eye-preserving treatment. The overall global salvage rate was 155 eyes (76.4%,155/203), and that after eye-preserving treatment was 91.7% (155/169). Severer eye for bilateral cases was taken into account for statistic; 120 cases (120 eyes) received initial eye-preserving treatment. Among them, 36 and 84 eyes underwent initial intravenous chemotherapy (IVC) and initial intra-arterial chemotherapy (IAC), respectively. The enucleation of the two groups was 7(19.4%, 7/36), 7(8.3%, 7/84); 33 (91.7%, 33/36) and 33 (39.3%, 33/84) eyes received the second treatment, respectively. There was no significant difference in the rate of enucleation between the two treatments (χ²=2.037, P=0.154). There was significant difference in the percentage of secondary treatment (χ²=27.937, P＜0.001). Fifty-four eyes (45.0%, 54/120) stabilized after initial treatment, and 66 eyes (55.0%, 66/120) underwent secondary treatments due to poor response or tumor recurrence. For 66 eyes receiving secondary treatments, enucleation, IAC, intravitreous chemotherapy (IVitC), IAC combined with IVitC, and laser and/or cryotherapy was performed in 6, 18, 12, 13, and 17 eyes, respectively. The number of eyes of enucleation among the IAC, IVitC, and IAC combined with IVitC group was 5 (27.8%, 5/18), 1 (9.3%, 1/12), and 2 (15.4%, 2/13) eyes, which was no significantly different (χ²=2.001, P=0.368). Until the last follow-up, visual acuity outcomes were acquired in 148 eyes (72.9%, 148/203). Among them, 41, 53, 16 and 38 eyes had no light perception, light perception to finger counting, 20/400, and ≥20/200, respectively. In total, among 203 eyes, 121 eyes received IAC, of which 2, 4, and 1 eyes had optic disc atrophy, vitreous hemorrhage, and severe retinal-choroidal atrophy, respectively; 60 eyes received IVitC, of which one and one eye had vitreous hemorrhage and macular hemorrhagic necrosis, respectively. Conclusions In this study, the overall survival rate was 94.9% after comprehensive treatment and the rate of global salvage after eye-preserving treatment was 91.7%. The comprehensive treatment of retinoblastoma had a relatively high efficacy and safety.

Citation： Zou Yihua, Ji Xunda, Li Jiakai, Liang Tingyi, Hua Xuming, Zhao Peiquan. A single-center retrospective clinical study on comprehensive treatment for 157 patients with retinoblastoma. Chinese Journal of Ocular Fundus Diseases, 2023, 39(10): 810-816. doi: 10.3760/cma.j.cn511434-20230801-00326 Copy

1.	Chen M, Jiang H, Zhang J, et al. Outcome of intra-arterial chemotherapy for retinoblastoma and its influencing factors: a retrospective study[J]. Acta Ophthalmol, 2017, 95(6): 613-618. DOI: 10.1111/aos.13333.
2.	Linn Murphree A. Intraocular retinoblastoma: the case for a new group classification[J]. Ophthalmol Clin North Am, 2005, 18(1): 41-53. DOI: 10.1016/j.ohc.2004.11.003.
3.	Shields CL, Mashayekhi A, Au AK, et al. The International Classification of Retinoblastoma predicts chemoreduction success[J]. Ophthalmology, 2006, 113(12): 2276-2280. DOI: 10.1016/j.ophtha.2006.06.018.
4.	季迅达, 李家恺, 赵军阳, 等. 眼内晚期视网膜母细胞瘤静脉化学治疗失败后行眼动脉介入化学治疗的疗效观察[J]. 中华眼底病杂志, 2015, 31(6): 556-559. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.011.Ji XD, Li JK, Zhao JY, et al. Efficacy of intra-arterial chemotherapy for advanced retinoblastoma after failure of intravenous chemotherapy[J]. Chin J Ocul Fundus Dis, 2015, 31(6): 556-559. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.011.
5.	Shields CL, Palamar M, Sharma P, et al. Retinoblastoma regression patterns following chemoreduction and adjuvant therapy in 557 tumors[J]. Arch Ophthalmol, 2009, 127(3): 282-290. DOI: 10.1001/archophthalmol.2008.626.
6.	Gündüz AK, Mirzayev I, Dinçaslan H, et al. Recurrence and new tumor development after frontline intravenous chemotherapy for retinoblastoma risk factors and treatment results[J]. Eur J Ophthalmol, 2022, 32(3): 1795-1803. DOI: 10.1177/11206721211023311.
7.	魏文斌, 周楠. 重视视网膜母细胞瘤的规范化治疗, 提升视网膜母细胞瘤的治疗水平[J]. 中华眼底病杂志, 2020, 36(6): 413-418. DOI: 10.3760/cma.j.cn511434-20200520-00209.Wei WB, Zhou N. Attach importance to standardized treatment of retinoblastoma to improve its treatment outcome[J]. Chin J Ocul Fundus Dis, 2020, 36(6): 413-418. DOI: 10.3760/cma.j.cn511434-20200520-00209.
8.	Kaliki S, Srinivasan V, Gupta A, et al. Clinical features predictive of high-risk retinoblastoma in 403 Asian Indian patients: a case-control study[J]. Ophthalmology, 2015, 122(6): 1165-1172. DOI: 10.1016/j.ophtha.2015.01.018.
9.	Fang X, Chen J, Wang Y, et al. RB1 germline mutation spectrum and clinical features in patients with unilateral retinoblastomas[J]. Ophthalmic Genet, 2021, 42(5): 593-599. DOI: 10.1080/13816810.2021.1946703.
10.	Zou Y, Li J, Hua P, et al. Spectrum of germline mutations in RB1 in Chinese patients with retinoblastoma: application of targeted next-generation sequencing[J]. Mol Vis, 2021, 27: 1-16.
11.	程湧, 贾忠旭, 赵明威, 等. 视网膜母细胞瘤综合治疗的回顾性临床研究[J]. 中华眼底病杂志, 2020, 36(6): 419-424. DOI: 10.3760/cma.j.cn511434-20200205-00040.Chen Y, Jia ZX, Zhao MW, et al. Retrospective clinical study on the comprehensive treatment of retinoblastoma[J]. Chin J Ocul Fundus Dis, 2020, 36(6): 419-424. DOI: 10.3760/cma.j.cn511434-20200205-00040.
12.	Zhou C, Wen X, Ding Y, et al. Eye-preserving therapies for advanced retinoblastoma: a multicenter cohort of 1678 patients in China[J]. Ophthalmology, 2022, 129(2): 209-219. DOI: 10.1016/j.ophtha.2021.09.002.
13.	Fabian ID, Stacey AW, Johnson KP, et al. Primary intravenous chemotherapy for group D retinoblastoma: a 13-year retrospective analysis[J]. Br J Ophthalmol, 2017, 101(1): 82-88. DOI: 10.1136/bjophthalmol-2016-309710.
14.	Abramson DH, Daniels AB, Marr BP, et al. Intra-arterial chemotherapy (ophthalmic artery chemosurgery) for group D retinoblastoma[J/OL]. PLoS One, 2016, 11(1): e0146582[2016-01-12]. https://pubmed.ncbi.nlm.nih.gov/26756643/. DOI: 10.1371/journal.pone.0146582.
15.	Gobin YP, Dunkel IJ, Marr BP, et al. Intra-arterial chemotherapy for the management of retinoblastoma: four-year experience[J]. Arch Ophthalmol, 2011, 129(6): 732-737. DOI: 10.1001/archophthalmol.2011.5.
16.	Shields CL, Manjandavida FP, Lally SE, et al. Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma[J]. Ophthalmology, 2014, 121(7): 1453-1460. DOI: 10.1016/j.ophtha.2014.01.026.
17.	Munier FL, Mosimann P, Puccinelli F, et al. First-line intra-arterial versus intravenous chemotherapy in unilateral sporadic group D retinoblastoma: evidence of better visual outcomes, ocular survival and shorter time to success with intra-arterial delivery from retrospective review of 20 years of treatment[J]. Br J Ophthalmol, 2017, 101(8): 1086-1093. DOI: 10.1136/bjophthalmol-2016-309298.
18.	Munier FL, Mosimann P, Puccinelli F, et al. Efficacy and complications of super-selective intra-ophthalmic artery melphalan for the treatment of refractory retinoblastoma[J]. Br J Ophthalmol, 2017, 101(8): 1086-1093. DOI: 10.1016/j.ophtha.2011.08.045.
19.	Shields CL, Bianciotto CG, Jabbour P, et al. Intra-arterial chemotherapy for retinoblastoma: report No. 2, treatment complications[J]. Arch Ophthalmol, 2011, 129(11): 1407-1415. DOI: 10.1001/archophthalmol.2011.151.
20.	Shields CL, Bianciotto CG, Jabbour P, et al. Intra-arterial chemotherapy for retinoblastoma: report No. 1, control of retinal tumors, subretinal seeds, and vitreous seeds[J]. Arch Ophthalmol, 2011, 129(11): 1399-1406. DOI: 10.1001/archophthalmol.2011.150.
21.	Abramson DH, Marr BP, Dunkel IJ, et al. Intra-arterial chemotherapy for retinoblastoma in eyes with vitreous and/or subretinal seeding: 2-year results[J]. Br J Ophthalmol, 2012, 96(4): 499-502. DOI: 10.1136/bjophthalmol-2011-300498.
22.	Munier FL, Gaillard MC, Balmer A, et al. Intravitreal chemotherapy for vitreous disease in retinoblastoma revisited: from prohibition to conditional indications[J]. Br J Ophthalmol, 2012, 96(8): 1078-1083. DOI: 10.1136/bjophthalmol-2011-301450.
23.	Shields CL, Douglass AM, Beggache M, et al. Intravitreous chemotherapy for active vitreous seeding from retinoblastoma: outcomes after 192 consecutive injections. The 2015 Howard Naquin Lecture[J]. Retina, 2016, 36(6): 1184-1190. DOI: 10.1097/iae.0000000000000903.

1. Chen M, Jiang H, Zhang J, et al. Outcome of intra-arterial chemotherapy for retinoblastoma and its influencing factors: a retrospective study[J]. Acta Ophthalmol, 2017, 95(6): 613-618. DOI: 10.1111/aos.13333.
2. Linn Murphree A. Intraocular retinoblastoma: the case for a new group classification[J]. Ophthalmol Clin North Am, 2005, 18(1): 41-53. DOI: 10.1016/j.ohc.2004.11.003.
3. Shields CL, Mashayekhi A, Au AK, et al. The International Classification of Retinoblastoma predicts chemoreduction success[J]. Ophthalmology, 2006, 113(12): 2276-2280. DOI: 10.1016/j.ophtha.2006.06.018.
4. 季迅达, 李家恺, 赵军阳, 等. 眼内晚期视网膜母细胞瘤静脉化学治疗失败后行眼动脉介入化学治疗的疗效观察[J]. 中华眼底病杂志, 2015, 31(6): 556-559. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.011.Ji XD, Li JK, Zhao JY, et al. Efficacy of intra-arterial chemotherapy for advanced retinoblastoma after failure of intravenous chemotherapy[J]. Chin J Ocul Fundus Dis, 2015, 31(6): 556-559. DOI: 10.3760/cma.j.issn.1005-1015.2015.06.011.
5. Shields CL, Palamar M, Sharma P, et al. Retinoblastoma regression patterns following chemoreduction and adjuvant therapy in 557 tumors[J]. Arch Ophthalmol, 2009, 127(3): 282-290. DOI: 10.1001/archophthalmol.2008.626.
6. Gündüz AK, Mirzayev I, Dinçaslan H, et al. Recurrence and new tumor development after frontline intravenous chemotherapy for retinoblastoma risk factors and treatment results[J]. Eur J Ophthalmol, 2022, 32(3): 1795-1803. DOI: 10.1177/11206721211023311.
7. 魏文斌, 周楠. 重视视网膜母细胞瘤的规范化治疗, 提升视网膜母细胞瘤的治疗水平[J]. 中华眼底病杂志, 2020, 36(6): 413-418. DOI: 10.3760/cma.j.cn511434-20200520-00209.Wei WB, Zhou N. Attach importance to standardized treatment of retinoblastoma to improve its treatment outcome[J]. Chin J Ocul Fundus Dis, 2020, 36(6): 413-418. DOI: 10.3760/cma.j.cn511434-20200520-00209.
8. Kaliki S, Srinivasan V, Gupta A, et al. Clinical features predictive of high-risk retinoblastoma in 403 Asian Indian patients: a case-control study[J]. Ophthalmology, 2015, 122(6): 1165-1172. DOI: 10.1016/j.ophtha.2015.01.018.
9. Fang X, Chen J, Wang Y, et al. RB1 germline mutation spectrum and clinical features in patients with unilateral retinoblastomas[J]. Ophthalmic Genet, 2021, 42(5): 593-599. DOI: 10.1080/13816810.2021.1946703.
10. Zou Y, Li J, Hua P, et al. Spectrum of germline mutations in RB1 in Chinese patients with retinoblastoma: application of targeted next-generation sequencing[J]. Mol Vis, 2021, 27: 1-16.
11. 程湧, 贾忠旭, 赵明威, 等. 视网膜母细胞瘤综合治疗的回顾性临床研究[J]. 中华眼底病杂志, 2020, 36(6): 419-424. DOI: 10.3760/cma.j.cn511434-20200205-00040.Chen Y, Jia ZX, Zhao MW, et al. Retrospective clinical study on the comprehensive treatment of retinoblastoma[J]. Chin J Ocul Fundus Dis, 2020, 36(6): 419-424. DOI: 10.3760/cma.j.cn511434-20200205-00040.
12. Zhou C, Wen X, Ding Y, et al. Eye-preserving therapies for advanced retinoblastoma: a multicenter cohort of 1678 patients in China[J]. Ophthalmology, 2022, 129(2): 209-219. DOI: 10.1016/j.ophtha.2021.09.002.
13. Fabian ID, Stacey AW, Johnson KP, et al. Primary intravenous chemotherapy for group D retinoblastoma: a 13-year retrospective analysis[J]. Br J Ophthalmol, 2017, 101(1): 82-88. DOI: 10.1136/bjophthalmol-2016-309710.
14. Abramson DH, Daniels AB, Marr BP, et al. Intra-arterial chemotherapy (ophthalmic artery chemosurgery) for group D retinoblastoma[J/OL]. PLoS One, 2016, 11(1): e0146582[2016-01-12]. https://pubmed.ncbi.nlm.nih.gov/26756643/. DOI: 10.1371/journal.pone.0146582.
15. Gobin YP, Dunkel IJ, Marr BP, et al. Intra-arterial chemotherapy for the management of retinoblastoma: four-year experience[J]. Arch Ophthalmol, 2011, 129(6): 732-737. DOI: 10.1001/archophthalmol.2011.5.
16. Shields CL, Manjandavida FP, Lally SE, et al. Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma[J]. Ophthalmology, 2014, 121(7): 1453-1460. DOI: 10.1016/j.ophtha.2014.01.026.
17. Munier FL, Mosimann P, Puccinelli F, et al. First-line intra-arterial versus intravenous chemotherapy in unilateral sporadic group D retinoblastoma: evidence of better visual outcomes, ocular survival and shorter time to success with intra-arterial delivery from retrospective review of 20 years of treatment[J]. Br J Ophthalmol, 2017, 101(8): 1086-1093. DOI: 10.1136/bjophthalmol-2016-309298.
18. Munier FL, Mosimann P, Puccinelli F, et al. Efficacy and complications of super-selective intra-ophthalmic artery melphalan for the treatment of refractory retinoblastoma[J]. Br J Ophthalmol, 2017, 101(8): 1086-1093. DOI: 10.1016/j.ophtha.2011.08.045.
19. Shields CL, Bianciotto CG, Jabbour P, et al. Intra-arterial chemotherapy for retinoblastoma: report No. 2, treatment complications[J]. Arch Ophthalmol, 2011, 129(11): 1407-1415. DOI: 10.1001/archophthalmol.2011.151.
20. Shields CL, Bianciotto CG, Jabbour P, et al. Intra-arterial chemotherapy for retinoblastoma: report No. 1, control of retinal tumors, subretinal seeds, and vitreous seeds[J]. Arch Ophthalmol, 2011, 129(11): 1399-1406. DOI: 10.1001/archophthalmol.2011.150.
21. Abramson DH, Marr BP, Dunkel IJ, et al. Intra-arterial chemotherapy for retinoblastoma in eyes with vitreous and/or subretinal seeding: 2-year results[J]. Br J Ophthalmol, 2012, 96(4): 499-502. DOI: 10.1136/bjophthalmol-2011-300498.
22. Munier FL, Gaillard MC, Balmer A, et al. Intravitreal chemotherapy for vitreous disease in retinoblastoma revisited: from prohibition to conditional indications[J]. Br J Ophthalmol, 2012, 96(8): 1078-1083. DOI: 10.1136/bjophthalmol-2011-301450.
23. Shields CL, Douglass AM, Beggache M, et al. Intravitreous chemotherapy for active vitreous seeding from retinoblastoma: outcomes after 192 consecutive injections. The 2015 Howard Naquin Lecture[J]. Retina, 2016, 36(6): 1184-1190. DOI: 10.1097/iae.0000000000000903.

Chinese Journal of Ocular Fundus Diseases

A single-center retrospective clinical study on comprehensive treatment for 157 patients with retinoblastoma

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