Risk definition and management strategies in retinoblastoma: current perspectives

Abstract

This manuscript focuses on high-risk factors of metastatic disease in retinoblastoma and evaluation of the current treatments of retinoblastoma. Presence of histopathologic high-risk factors is associated with a higher risk of local recurrence and systemic metastasis. Currently, globe-sparing therapies, including systemic chemotherapy, intra-arterial chemoreduction, intravitreal chemotherapy, focal consolidation, and combination therapies, are being used and investigated actively. Major advances are being made in the diagnosis and management of retinoblastoma that will lead to improved morbidity and mortality rates in patients with retinoblastoma. By saving the globes, fronting with some high-risk factors for metastasis would be inevitable. International multi-institutional prospective studies could resolve current uncertainties regarding the main tumor treatment regimens for each patient and indications for chemoprophylaxis for high-risk-factor-bearing retinoblastoma cases.

Clinical behavior of intraocular teratoid medulloepithelioma in two‐related Quarter Horses

“…Retinoblastomas that are classified as ‘high‐risk’, result in metastasis in approximately 24% of patients not treated with adjunctive systemic chemotherapy although there are no prospective, randomized studies confirming this . The main histologic risk factors of metastasis include extension into the anterior chamber, iris and ciliary body infiltration, tumor invasion beyond the lamina cribosa, disease at the optic nerve transection site, choroidal infiltration, and extrascleral extension . These characteristics are similar to prognostic factors noted for medulloepitheliomas and as discussed were not overtly present in Case 1.…”

“…Although these tumors are also of primitive neuroepithelial origin, the presence of other cell types such as cartilage, neuronal, glial, and mesenchymal elements warrants designating the current cases as teratoid medulloepitheliomas. Retinoblastomas that are classified as ‘high‐risk’, result in metastasis in approximately 24% of patients not treated with adjunctive systemic chemotherapy although there are no prospective, randomized studies confirming this . The main histologic risk factors of metastasis include extension into the anterior chamber, iris and ciliary body infiltration, tumor invasion beyond the lamina cribosa, disease at the optic nerve transection site, choroidal infiltration, and extrascleral extension .…”

Clinical and genetic associations for carboplatin‐related ototoxicity in children treated for retinoblastoma: A retrospective noncomparative single‐institute experience

“…This is in accordance with previously published reports of risk factors of carboplatin‐induced hearing loss in pediatric patients . In younger children with retinoblastoma, the potential benefit of carboplatin‐based chemotherapy can be carefully weighed against risk of ototoxicity . Multiple treatment modalities are now available that might either avoid or postpone carboplatin use at lower dosage, topotecan‐based chemotherapy, periocular chemotherapy, or intra‐arterial chemotherapy …”

Adenocarcinoma of the Retinal Pigment Epithelium Arising in Conjunction with Late Recurrence and Systemic Metastasis of Retinoblastoma

“…Eyes that meet criteria for high-risk retinoblastoma have a 24% chance of developing metastatic disease, which is subsequently reduced to 4% if treated appropriately with systemic chemotherapy [12]. Therefore, the ability to thoroughly, and accurately, identify patients with high-risk retinoblastoma is thought by some care providers to be of critical importance.…”