BACKGROUND: Access to intra-arterial chemotherapy for retinoblastoma in low- and middle-income countries (LMICs) is limited. There is a need to optimize the efficacy of systemic chemotherapy for advanced intraocular retinoblastoma, particularly in LMICs. The aim was to compare the efficacy of standard versus higher dose carboplatin-based intravenous chemotherapy for group D and E retinoblastoma.
METHODS: The single-center, single-blinded, randomized study was conducted during 2019-2021. Patients with newly diagnosed group D or E retinoblastoma were randomized to receive vincristine, etoposide, and standard versus higher dose (<36 months: 18.6 vs. 28 mg/kg; ≥36 months: 560 vs. 840 mg/m2 ) carboplatin. Examination under anesthesia and ultrasonography was performed at diagnosis and following three cycles of chemotherapy. Group E eyes with poor likelihood of globe/vision salvage at diagnosis were excluded.
RESULTS: Thirty-two eyes of 30 patients were analyzed: 17 group D and 15 group E eyes. The tumor response to chemotherapy with regards to regression pattern (p = .72), tumor shrinkage (diameter: p = .11, height: p = .96), subretinal seeds (p = .91), and vitreous seeds (p = .9) were comparable between the two treatment arms. The globe salvage (group D [82% vs. 67%; p = .58]; group E [12.5% vs. 29%; p = .57]) and salvage of meaningful vision (group D [100% vs. 75%; p = .13]; group E [100% vs. 50%; p = .48]) were comparable between standard and higher dose arms. No excess treatment-related toxicity was observed in the higher dose arm.
CONCLUSIONS: Higher dose carboplatin-based intravenous chemotherapy did not result in superior globe or vision salvage in group D or E retinoblastoma.

UNASSIGNED: This study aimed to evaluate the efficacy and complications of intra-arterial chemotherapy (IAC) as a primary treatment for advanced unilateral retinoblastoma in Chinese patients.
UNASSIGNED: This study was a retrospective review of patients with advanced unilateral retinoblastoma treated with IAC as the primary treatment. The IAC procedures were performed using a balloon-assisted technique. The clinical status and treatment complications were recorded at each visit. Kaplan-Meier analysis was performed to estimate recurrence-free survival and ocular survival.
UNASSIGNED: In total, 116 eyes of 116 patients with advanced unilateral retinoblastoma were enrolled, including 66 eyes (57%) in group D and 50 eyes (43%) in group E. All treated eyes received a mean of 3 cycles of IAC (range, 3-5), and 66% of the eyes were combined with local consolidation therapy. The median follow-up time was 39 months (range, 22-57 months). The 3-year recurrence-free survival and ocular survival rates were 68.8% (95% CI, 59.2-76.6%) and 88.5% (95% CI, 80.9-93.2%), respectively. Moreover, the 3-year ocular survival rate in group D was significantly higher than that in group E (96.9%, 76.3%; P < 0.01). The common ocular complication was vitreous hemorrhage (19.8%). No deaths or severe systemic complications occurred.
UNASSIGNED: Primary intra-arterial chemotherapy is effective for the treatment of advanced unilateral retinoblastoma, especially in group D, with acceptable toxicity.

OBJECTIVE: To figure out the impact of national health insurance program and treatment modalities on the clinical outcomes of patients with retinoblastoma.
METHODS: Retrospective cohort study.
METHODS: Enrolled patients were classified into three groups according to the time period in which their initial diagnosis was performed: patients diagnosed before 1995 (group 1), patients diagnosed in the time period 1995-2010 (group 2), patients diagnosed after 2010 (group 3). Comparison was made of clinical features including presenting signs, tumor spreading, and treatment modes. Survival and globe salvage rates were analyzed within each group.
RESULTS: There were 157 patients (202 eyes) enrolled from 1978 to 2015. The overall mortality rate was 24.2%. The overall 5-year survival rate was 63.4%, 73.8%, 89.1% in the three time periods respectively (P = 0.007). The 5-year survival improved continuously in both unilateral and bilateral disease (P = 0.057; P = 0.015). Patients without extraocular spreading or diagnosed before 2 years of age had a better 5- year survival of 97.4% and 81.1% respectively. The globe salvage rate improved from 8.2%, 14.5%, to 35.1% (P < 0.001). The globe salvage rate was 46.9% in patients who received ophthalmic artery chemosurgery (OAC) and 6.4% in patients not received OAC.
CONCLUSIONS: Improvement of survival and globe salvage rate was observed over time periods. National health insurance program has made medical care easily accessible to the public, leading to earlier diagnosis and treatment. Different treatment modes could achieve a better clinical outcome; among the new treatment modalities, OAC played an important role.

暂无摘要。

OBJECTIVE: To determine which IIRC scheme was used by retinoblastoma centers worldwide and the percentage of D eyes treated primarily with enucleation versus globe salvaging therapies as well as to correlate trends in treatment choice to IIRC version used and geographic region.
METHODS: An anonymized electronic survey was offered to 115 physicians at 39 retinoblastoma centers worldwide asking about IIRC classification schemes and treatment patterns used between 2008 and 2012. Participants were asked to record which version of the IIRC was used for classification, how many group D eyes were diagnosed, and how many eyes were treated with enucleation versus globe salvaging therapies. Averages of eyes per treatment modality were calculated and stratified by both IIRC version and geographic region. Statistical significance was determined by Chi-square, ANOVA and Kruskal-Wallis tests using Prism.
RESULTS: The survey was completed by 29% of physicians invited to participate. Totally 1807 D eyes were diagnosed. Regarding IIRC system, 27% of centers used the Children\'s Hospital of Los Angeles (CHLA) version, 33% used the Children\'s Oncology Group (COG) version, 23% used the Philadelphia version, and 17% were unsure. The rate for primary enucleation varied between 0 and 100% and the mean was 29%. By IIRC version, primary enucleation rates were: Philadelphia, 8%; COG, 34%; and CHLA, 37%. By geographic region, primary enucleation rates were: Latin America, 57%; Asia, 40%; Europe, 36%; Africa, 10%, US, 8%; and Middle East, 8%. However, systemic chemoreduction was used more often than enucleation in all regions except Latin America with a mean of 57% per center (P<0.0001).
CONCLUSIONS: Worldwide there is no consensus on which IIRC version is used, systemic chemoreduction was the most frequently used initial treatment during the study period followed by enucleation and primary treatment modality, especially enucleation, varied greatly with regards to IIRC version used and geographic region.

Retinoblastoma (RB) is the most common primary cancer to affect the eyes in children with approximately 350 cases/year in the United States and 8000 worldwide. Today, sadly, 50% of children with RB worldwide die from their disease. In our experience, utilization of ophthalmic artery chemosurgery (OAC) has transformed the treatment plan for patients; with over 1500 procedures performed, our survival rate exceeds 98%. It is now our standard first-line therapy for RB. OAC is a surgical outpatient procedure which delivers concentrated doses of chemotherapy directly to the tumor without the toxicities of systemic chemotherapy. Our team approach and nursing management of these patients are the focus of this article. Nursing navigation and collaboration after OAC is vital and requires a combined effort by the nurses along with physicians, interventional radiologists, and the patient\'s families to ensure appropriate follow-up is established. Proper patient education throughout the process is crucial as is open and available communication for parents of patients with the nursing staff. The success in our treatment of this disease can be much accredited to the multidisciplinary team approach, with nursing playing an integral part in the support and management of these patients.

Surgical removal of one or both eyes has been the most common way to treat children with retinoblastoma worldwide for more than 100 years. Ophthalmic artery chemosurgery (OAC) was introduced 10 years ago and it has been used as an alternative to enucleation for eyes with advanced retinoblastoma. The purpose of this report is to analyze our 9-year experience treating advanced retinoblastoma eyes with OAC.
Single-arm retrospective study from a single center of 226 eyes with eyes of retinoblastoma patients with advanced intraocular disease defined as both Reese-Ellsworth (RE) \"Va\" or \"Vb\" and International Classification Retinoblastoma (ICRb) group \"D\" or \"E\" (COG Classification). Ocular survival, patient survival, second cancers, and electroretinography (ERG) were assessed.
Ocular survival at five years for these advanced eyes was 70.2% (95% confidence interval, 57.3%-79.8%). When eyes were divided into groups either by RE classification or ICRb, no significant differences in ocular survival were seen. Ocular survival was significantly better in naïve compared to non-naïve eyes (80.2% vs 58.4%, p = 0.041). The ERG distribution was very similar before and after OAC treatment for the patient population that did not receive intravitreal chemotherapy. Three patients (1.5%) have developed metastatic retinoblastoma (previously reported) and were successfully treated (no deaths).
Using OAC for advanced eyes (the majority of such eyes have been enucleated in the past) enables 70% 5-year ocular survival. Treated eyes have a similar ERG distribution before and after treatment. No patient has died of metastatic retinoblastoma.