Surgical Outcomes in Radiation-induced Cataracts After External-beam Radiotherapy in Retinoblastoma

Objectives: To investigate visual outcomes, surgical complications and tumor recurrence among children with retinoblastoma undergoing phacoemulsification and posterior chamber intraocular lens (PCIOL) implantation for radiation-induced cataract secondary to external beam radiotherapy. Materials and Methods: The medical records of all patients treated by phacoemulsification and PCIOL implantation for radiation-induced cataract after external beam radiotherapy for retinoblastoma at a single institution between 1980 and 2014 were reviewed retrospectively. The study included 6 eyes of 6 children (4 girls, 2 boys). Results: Four patients had bilateral and two patients had unilateral retinoblastoma. The median age at diagnosis of retinoblastoma was 28.3 months (range, 12-96 months). All patients received chemoreduction (OPEC protocol) and external beam radiotherapy with or without local ophthalmic therapies and developed radiation-induced cataracts. The median interval from retinoblastoma diagnosis to cataract surgery was 96.3 months (range, 73-122 months). Time interval between surgery and last retinoblastoma treatment was 67.2 months. Postoperative complications included iridocyclitis in 2 eyes and posterior capsule opacification in all eyes. The mean follow-up after surgery was 105.8 months (range, 59-120 months). Final visual acuity was better in all eyes than preoperative visual acuities. Conclusion: Phacoemulsification and PCIOL implantation is an effective method of managing radiation-induced cataracts in eyes with previously treated retinoblastoma. However, visual acuity was limited by the presence of primary macular tumor

Global Retinoblastoma Presentation and Analysis by National Income Level.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs

The global retinoblastoma outcome study : a prospective, cluster-based analysis of 4064 patients from 149 countries

DATA SHARING : The study data will become available online once all analyses are complete.BACKGROUND : Retinoblastoma is the most common intraocular cancer worldwide. There is some evidence to suggest that major differences exist in treatment outcomes for children with retinoblastoma from different regions, but these differences have not been assessed on a global scale. We aimed to report 3-year outcomes for children with retinoblastoma globally and to investigate factors associated with survival. METHODS : We did a prospective cluster-based analysis of treatment-naive patients with retinoblastoma who were diagnosed between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Patients were recruited from 260 specialised treatment centres worldwide. Data were obtained from participating centres on primary and additional treatments, duration of follow-up, metastasis, eye globe salvage, and survival outcome. We analysed time to death and time to enucleation with Cox regression models. FINDINGS : The cohort included 4064 children from 149 countries. The median age at diagnosis was 23·2 months (IQR 11·0–36·5). Extraocular tumour spread (cT4 of the cTNMH classification) at diagnosis was reported in five (0·8%) of 636 children from high-income countries, 55 (5·4%) of 1027 children from upper-middle-income countries, 342 (19·7%) of 1738 children from lower-middle-income countries, and 196 (42·9%) of 457 children from low-income countries. Enucleation surgery was available for all children and intravenous chemotherapy was available for 4014 (98·8%) of 4064 children. The 3-year survival rate was 99·5% (95% CI 98·8–100·0) for children from high-income countries, 91·2% (89·5–93·0) for children from upper-middle-income countries, 80·3% (78·3–82·3) for children from lower-middle-income countries, and 57·3% (52·1-63·0) for children from low-income countries. On analysis, independent factors for worse survival were residence in low-income countries compared to high-income countries (hazard ratio 16·67; 95% CI 4·76–50·00), cT4 advanced tumour compared to cT1 (8·98; 4·44–18·18), and older age at diagnosis in children up to 3 years (1·38 per year; 1·23–1·56). For children aged 3–7 years, the mortality risk decreased slightly (p=0·0104 for the change in slope). INTERPRETATION : This study, estimated to include approximately half of all new retinoblastoma cases worldwide in 2017, shows profound inequity in survival of children depending on the national income level of their country of residence. In high-income countries, death from retinoblastoma is rare, whereas in low-income countries estimated 3-year survival is just over 50%. Although essential treatments are available in nearly all countries, early diagnosis and treatment in low-income countries are key to improving survival outcomes.The Queen Elizabeth Diamond Jubilee Trust and the Wellcome Trust.https://www.thelancet.com/journals/langlo/homeam2023Paediatrics and Child Healt

Brachytherapy in the Management of Uveal Melanomas

Uveal melanoma is the most common intraocular tumor in adults. Clinical studies have shown similar patient survival rates after treatment of medium-sized melanomas when comparing plaque brachytherapy with radioactive iodine-125 versus enucleation. This finding further emphasizes the importance of this globe-sparing treatment. Brachytherapy is a special local radiotherapy technique that aims to deliver high-dose radiation directly to the tumor by sparing the periocular structures. Brachytherapy is still the most widely used treatment for uveal melanoma. Iodine-125 and ruthenium-106 are the most common radioisotopes used in brachytherapy. After brachytherapy, sight-threatening complications occur unavoidably in many patients. Brachytherapy is mostly associated with long-term complications. Radiation retinopathy and cataract formation are the most common treatment-related complications. Brachytherapy provides local tumor control (ocular salvage) in about 90% of patients. Adjunctive transpupillary thermotherapy (sandwich therapy) improves the control rate of local tumors to 97%. About 10% of patients treated with brachytherapy subsequently require enucleation because of local tumor recurrence or neovascular glaucoma at 5 years of follow-up. Metastatic disease occurs in 10% of patients with medium-sized melanoma at 5-year follow-up. This rate increases to 55% at 10-year follow-up in patients with large melanomas (thickness >8 mm). Thus, it is very important to inform the patients under the light of these data prior to brachytherapy. (Turk J Ophthalmol 2014; 44: Supplement 43-8

Recent Trends in the Management of Retinoblastoma

Retinoblastoma is the most common intraocular malignancy of childhood. Significant progress has been made in the past few years in the management of retinoblastoma. Thus, the patients' survival and ocular salvage rates have shown marked improvement. The current treatment strategies for retinoblastoma include enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Enucleation is generally reserved for eyes with extensive retinoblastoma, in which there is no hope for useful vision. From mid-1990s, intravenous chemoreduction has been used efficiently in the management of group A, B, or C, and some D eyes according to the International Classification of Retinoblastoma. Intravenous chemoreduction combined with focal treatments (cryotherapy, transpupillary thermotherapy, and brachytherapy) have provided favorable tumor control for most of these eyes. In 2008, superselective intra-arterial chemotherapy has been popularized. This treatment involves injection of a chemotherapeutic agent (mostly melphalan) after selective catheterization of the ophthalmic artery under neuroradiologic intervention. This provides higher success in advanced group D and Group E eyes that have relatively lower rate of tumor control with conventional intravenous chemoreduction. In conclusion, significant advances that have emerged recently in the management of retinoblastoma led to an increase in ocular salvage rates. Recently, popularized modality of intra-arterial chemotherapy offers hope in the control of eyes with advanced retinoblastoma

Dramatic Regression of Presumed Acquired Retinal Astrocytoma with Photodynamic Therapy

Photodynamic therapy (PDT) has been used for treatment of various intraocular tumors including choroidal hemangioma, vasoproliferative tumor, amelanotic choroidal melanoma and choroidal neovascular membrane due to choroidal osteoma. This case report documents the effect of PDT for a presumed acquired retinal astrocytoma. A 42-year-old female with a juxtapapillary acquired astrocytoma was treated with a single session of PDT using standard parameters. The tumor showed dramatic regression over 6 months into a fibrotic scar. It remained regressed and stable with 20/20 vision after 51 months of follow-up. We believe that PDT can be used as a primary treatment for acquired retinal astrocytoma

Recent Trends in the Management of Retinoblastoma

Retinoblastoma is the most common intraocular malignancy of childhood. Significant progress has been made in the past few years in the management of retinoblastoma. Thus, the patients’ survival and ocular salvage rates have shown marked improvement. The current treatment strategies for retinoblastoma include enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Enucleation is generally reserved for eyes with extensive retinoblastoma, in which there is no hope for useful vision. From mid-1990s, intravenous chemoreduction has been used efficiently in the management of group A, B, or C, and some D eyes according to the International Classification of Retinoblastoma. Intravenous chemoreduction combined with focal treatments (cryotherapy, transpupillary thermotherapy, and brachytherapy) have provided favorable tumor control for most of these eyes. In 2008, superselective intra-arterial chemotherapy has been popularized. This treatment involves injection of a chemotherapeutic agent (mostly melphalan) after selective catheterization of the ophthalmic artery under neuroradiologic intervention. This provides higher success in advanced group D and Group E eyes that have relatively lower rate of tumor control with conventional intravenous chemoreduction. In conclusion, significant advances that have emerged recently in the management of retinoblastoma led to an increase in ocular salvage rates. Recently, popularized modality of intra-arterial chemotherapy offers hope in the control of eyes with advanced retinoblastoma. (Turk J Ophthalmol 2015; 45: 71-6