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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., (© 2024. The Author(s).)

Background: To analyze the clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma (RB) and intracranial segment of retrobulbar optic nerve invasion., Methods: A total of 14 children with unilateral RB and intracranial segment of retrobulbar optic nerve invasion were enrolled in this retrospective study from January 2009 to December 2018. Clinical characteristics, treatment and prognosis were collected and analyzed. Survival curves were calculated by Kaplan-Meier method., Results: Of 14 cases, there were 7 male and 7 female, ranging in age from 22.85 to 121.97 months (median, 41.03 months). Seventy-one percent of patients came from first-tier cities in China and effected in the left eye. Magnetic resonance imaging (MRI) results indicated that all patients presented with thickened and enhanced optic nerve and intracranial segment of optic nerve invasion. Nine patients received comprehensive therapeutic regimen (chemotherapy, eye enucleation, radiotherapy and intrathecal therapy). The patients were followed up to December 2019, with a median follow-up of 20.6 months. The median disease specific survival was 48.99 ± 8.62 months, and the overall survival (OS) rate was 64.3%. Radiotherapy and comprehensive therapeutic regimen had significant impact on survival time (all p < 0.05)., Conclusions: The overall prognosis of unilateral RB patients with intracranial segment of retrobulbar optic nerve invasion was poor. Chemotherapy and surgical treatment were necessary, but more attention should be paid to radiotherapy and intrathecal therapy for improving prognosis.

Objective: To retrospectively summarize the clinical features, diagnosis and treatment of retinoblastoma (RB) patients in the children aged 5 years or above., Methods: This was a case-series study. Total of 92 patients (107 eyes) with RB aged 5 years or above in Department of Pediatric,Beijing Tongren Hospital from September 2005 to May 2010 were included in the study. The diagnosis of RB was established using clinical examination and histopathology analysis.Statistical analysis in the characteristics of disease and initial symptoms was performed in 92 cases. Kaplan-Meier curve was employed to analyze the survival function., Results: (1) The medium age of the patients was 6.25-year. Male accounted for 66.30% (61/92) and female was 33.69% (31/92) . According to the results of statistical scatter diagram, the age of the disease onset was ranged from 5.00 to 8.33 years. Percentile for age 5.77, 5.83 and 7.85 years were the 25th, 50th, 75th respectively. (2) In 92 cases, 83.69% (77/92) of the patients were unilateral RB and 16.31% (15/92) were bilateral RB. (3) Leukocoria was the most common manifestation in the diagnosis early RB, accounting for 55.43% (51/92) .Secondary symptoms were blurred vision, eye redness and photophobia, accounting for 23.91% (22/92). Other symptoms included strabismus, proptosis, glaucoma and the abnormal in fundus examination, accounting for 20.64% (19/92) .No family history of RB was found in all of cases studied. (4) Among 107 eyes with RB, 76 eyes were enucleated up to February 2013. The rate of enucleation was 71.02%. (5) By February 2013, the average following-up time was 39 months, during the time 1 case was lost, 6 cases were dead, the overall survival rate was 93.46% (85/92) . The result of Kaplan-Meier curve showed that the estimates of 5-year expected average total survival time was (72.61 ± 1.89) months(95%confidence interval was 68.9-76.3 months)., Conclusions: Unilateral RB are the most common cases in the age of 5 years or above, and the most common symptom is Leukocoria.Overall survival rate of older RB patient is high with comprehensive treatment.