Characteristics of patients with recurrent retinoblastoma: a survival analysis

Abstract Background Management guidelines and corresponding survival data for patients with recurrent retinoblastoma (RB) are lacking. This study aimed to summarize the clinical characteristics of patients with recurrent RB and analyze their survival outcomes. Methods We retrospectively analyzed 68...

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Main Authors: Nan Li, Yi-Zhuo Wang, Yi Zhang, Wei-Ling Zhang, Dong-Sheng Huang
Format: Article
Language:English
Published: BMC 2024-03-01
Series:BMC Cancer
Subjects:
Online Access:https://doi.org/10.1186/s12885-024-12058-3
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author Nan Li
Yi-Zhuo Wang
Yi Zhang
Wei-Ling Zhang
Dong-Sheng Huang
author_facet Nan Li
Yi-Zhuo Wang
Yi Zhang
Wei-Ling Zhang
Dong-Sheng Huang
author_sort Nan Li
collection DOAJ
description Abstract Background Management guidelines and corresponding survival data for patients with recurrent retinoblastoma (RB) are lacking. This study aimed to summarize the clinical characteristics of patients with recurrent RB and analyze their survival outcomes. Methods We retrospectively analyzed 68 patients with recurrent RB who underwent treatment in our institution from January 2016 to December 2020. Patients were grouped according to location of recurrence: intraocular, orbital, and distant metastasis. Results The male:female ratio was 1.3:1 and the median age at recurrence was 37.5 months (range, 30.3–62.8). The number of patients in the intraocular recurrence, orbital recurrence, and metastasis groups was 13 (19.1%), 23 (33.8%), and 32 (47.1%), respectively. Thirty patients died, 36 were alive at last follow-up, and two were lost to follow-up. Eye enucleation was performed in 94.1% of patients. Five-year overall survival in patients with intraocular recurrence, orbital recurrence, and metastasis was 84.6%, 69.6%, and 31.3%, respectively (P = 0.001). Most deaths occurred within 2 years of recurrence. Presence of high-risk pathological factors, central nervous system invasion, and absence of combination therapy were independent predictors of worse 5-year overall survival. Conclusion The rate of eye preservation in survivors of recurrent RB was very low. Although 5-year overall survival in patients who underwent treatment for intraocular and orbital recurrence was high, it was low in those with metastasis. RB patients may need lifelong follow-up for recurrence and secondary malignancy.
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spelling doaj.art-e7548f10f49248a3af2850bd42b5077b2024-03-05T19:24:20ZengBMCBMC Cancer1471-24072024-03-012411710.1186/s12885-024-12058-3Characteristics of patients with recurrent retinoblastoma: a survival analysisNan Li0Yi-Zhuo Wang1Yi Zhang2Wei-Ling Zhang3Dong-Sheng Huang4Department of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Yizhuang Economic and Technological Development ZoneDepartment of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Yizhuang Economic and Technological Development ZoneDepartment of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Yizhuang Economic and Technological Development ZoneDepartment of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Yizhuang Economic and Technological Development ZoneDepartment of Pediatrics, Beijing Tongren Hospital, Capital Medical University, Yizhuang Economic and Technological Development ZoneAbstract Background Management guidelines and corresponding survival data for patients with recurrent retinoblastoma (RB) are lacking. This study aimed to summarize the clinical characteristics of patients with recurrent RB and analyze their survival outcomes. Methods We retrospectively analyzed 68 patients with recurrent RB who underwent treatment in our institution from January 2016 to December 2020. Patients were grouped according to location of recurrence: intraocular, orbital, and distant metastasis. Results The male:female ratio was 1.3:1 and the median age at recurrence was 37.5 months (range, 30.3–62.8). The number of patients in the intraocular recurrence, orbital recurrence, and metastasis groups was 13 (19.1%), 23 (33.8%), and 32 (47.1%), respectively. Thirty patients died, 36 were alive at last follow-up, and two were lost to follow-up. Eye enucleation was performed in 94.1% of patients. Five-year overall survival in patients with intraocular recurrence, orbital recurrence, and metastasis was 84.6%, 69.6%, and 31.3%, respectively (P = 0.001). Most deaths occurred within 2 years of recurrence. Presence of high-risk pathological factors, central nervous system invasion, and absence of combination therapy were independent predictors of worse 5-year overall survival. Conclusion The rate of eye preservation in survivors of recurrent RB was very low. Although 5-year overall survival in patients who underwent treatment for intraocular and orbital recurrence was high, it was low in those with metastasis. RB patients may need lifelong follow-up for recurrence and secondary malignancy.https://doi.org/10.1186/s12885-024-12058-3RetinoblastomaRecurrenceSurvival analysis
spellingShingle Nan Li
Yi-Zhuo Wang
Yi Zhang
Wei-Ling Zhang
Dong-Sheng Huang
Characteristics of patients with recurrent retinoblastoma: a survival analysis
BMC Cancer
Retinoblastoma
Recurrence
Survival analysis
title Characteristics of patients with recurrent retinoblastoma: a survival analysis
title_full Characteristics of patients with recurrent retinoblastoma: a survival analysis
title_fullStr Characteristics of patients with recurrent retinoblastoma: a survival analysis
title_full_unstemmed Characteristics of patients with recurrent retinoblastoma: a survival analysis
title_short Characteristics of patients with recurrent retinoblastoma: a survival analysis
title_sort characteristics of patients with recurrent retinoblastoma a survival analysis
topic Retinoblastoma
Recurrence
Survival analysis
url https://doi.org/10.1186/s12885-024-12058-3
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