AIM： 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 trea...AIM： 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）. CONCLUSION： 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.展开更多
Retinoblastoma is the most common primary childhood ocular tumor, affecting nearly 3.5 per million children worldwide. A mutation in the RB1 gene, which presents as either germline or sporadic, along with additional m...Retinoblastoma is the most common primary childhood ocular tumor, affecting nearly 3.5 per million children worldwide. A mutation in the RB1 gene, which presents as either germline or sporadic, along with additional mutational events, promote neoplastic growth in the retina. Fortunately, current treatment protocols result in success rates approaching 99% at specialized centers, with many children maintaining useful vision. Overall, treatment is guided by aggressiveness and size, and is classified by systems such as the ReeseEllsworth System and the International Classification of Retinoblastoma. Due to advances in chemotherapy protocols combined with use of focal laser consolidation, treatment paradigms have shifted from enucleation to external beam radiation therapy to chemotherapy as globe-salvaging therapies. Smaller, less complex tumors may be controlled by plaque radiotherapy or focal laser ablative therapy. However, larger and more complex tumors, such as those that have vitreous or subretinal seeding, require methods of chemoreduction combined with focal consolidation to yield better outcomes. Standard chemotherapy protocols utilize vincristine, etoposide, and carboplatin with or withoutcyclophosphamide. Finally, there has been a recent push in local treatments for retinoblastoma to minimize systemic toxicities. These modalities include intravitreal or subconjunctival injections and more recently, direct chemotherapy administration into the ophthalmic artery. As a result, enucleation is used less often, but remains an important treatment for the most aggressive, refractory cases. The advancement of retinoblastoma treatment looks promising; however, worldwide access to these treatments and the lack of long-term followup of new local treatment modalities constitute current and future challenges.展开更多
Background:Patients with Sturge-Weber syndrome can have ipsilateral diffuse or circumscribed choroidal hemangiomas.These hemangiomas have been seen to undergo spontaneous exudative or hemorrhagic retinal detachments.T...Background:Patients with Sturge-Weber syndrome can have ipsilateral diffuse or circumscribed choroidal hemangiomas.These hemangiomas have been seen to undergo spontaneous exudative or hemorrhagic retinal detachments.There is no definitive treatment for these types of retinal detachments,but radiotherapy,photodynamic therapy,oral propranolol,pegaptinib and bevacizumab have been used.Case presentation:A 26-year-old male with Sturge-Weber Syndrome developed an exudative retinal detachment that occurred immediately after taking a supplement containing arginine.The patient was treated with intravitreal bevacizumab 1.25 mg in 0.05 ml solution.Resolution of the retinal detachment was seen after 4 treatments over a six-month period.Conclusions:Arginine and other medications that cause a release of nitric oxide may lead to intravascular leakage and exudative retinal detachments in patients who have a choroidal hemangioma.展开更多
基金Supported in part by grants NIH/NCI Cancer Center Support Grant P30 CA008748
文摘AIM： 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）. CONCLUSION： 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.
文摘Retinoblastoma is the most common primary childhood ocular tumor, affecting nearly 3.5 per million children worldwide. A mutation in the RB1 gene, which presents as either germline or sporadic, along with additional mutational events, promote neoplastic growth in the retina. Fortunately, current treatment protocols result in success rates approaching 99% at specialized centers, with many children maintaining useful vision. Overall, treatment is guided by aggressiveness and size, and is classified by systems such as the ReeseEllsworth System and the International Classification of Retinoblastoma. Due to advances in chemotherapy protocols combined with use of focal laser consolidation, treatment paradigms have shifted from enucleation to external beam radiation therapy to chemotherapy as globe-salvaging therapies. Smaller, less complex tumors may be controlled by plaque radiotherapy or focal laser ablative therapy. However, larger and more complex tumors, such as those that have vitreous or subretinal seeding, require methods of chemoreduction combined with focal consolidation to yield better outcomes. Standard chemotherapy protocols utilize vincristine, etoposide, and carboplatin with or withoutcyclophosphamide. Finally, there has been a recent push in local treatments for retinoblastoma to minimize systemic toxicities. These modalities include intravitreal or subconjunctival injections and more recently, direct chemotherapy administration into the ophthalmic artery. As a result, enucleation is used less often, but remains an important treatment for the most aggressive, refractory cases. The advancement of retinoblastoma treatment looks promising; however, worldwide access to these treatments and the lack of long-term followup of new local treatment modalities constitute current and future challenges.
文摘Background:Patients with Sturge-Weber syndrome can have ipsilateral diffuse or circumscribed choroidal hemangiomas.These hemangiomas have been seen to undergo spontaneous exudative or hemorrhagic retinal detachments.There is no definitive treatment for these types of retinal detachments,but radiotherapy,photodynamic therapy,oral propranolol,pegaptinib and bevacizumab have been used.Case presentation:A 26-year-old male with Sturge-Weber Syndrome developed an exudative retinal detachment that occurred immediately after taking a supplement containing arginine.The patient was treated with intravitreal bevacizumab 1.25 mg in 0.05 ml solution.Resolution of the retinal detachment was seen after 4 treatments over a six-month period.Conclusions:Arginine and other medications that cause a release of nitric oxide may lead to intravascular leakage and exudative retinal detachments in patients who have a choroidal hemangioma.
