BACKGROUND: Although intra-retinal tumor has long been staged presurgically according to the Reese-Ellsworth (R-E) system, retinoblastoma differs from other pediatric neoplasms in never having had a widely accepted classification system that encompasses the entire spectrum of the disease. Comparisons among studies that consider disease extension, risk factors for extra-ocular relapse, and response to therapy require a universally accepted staging system for extra-ocular disease. PROCEDURE: A committee of retinoblastoma experts from large centers worldwide has developed a consensus classification that can encompass all retinoblastoma cases and is presented herein. Patients are classified according to extent of disease and the presence of overt extra-ocular extension. In addition, a proposal for substaging considering histopathological features of enucleated specimens is presented to further discriminate between Stage I and II patients. RESULTS: The following is a summary of the classification system developed-Stage 0: Patients treated conservatively (subject to presurgical ophthalmologic classifications); Stage I: Eye enucleated, completely resected histologically; Stage II: Eye enucleated, microscopic residual tumor; Stage III: Regional extension [(a) overt orbital disease, (b) preauricular or cervical lymph node extension]; Stage IV: Metastatic disease [(a) hematogenous metastasis: (1) single lesion, (2) multiple lesions; (b) CNS extension: (1) prechiasmatic lesion, (2) CNS mass, (3) leptomeningeal disease]. A proposal is also presented for substaging of enucleated Stages I and II eyes. CONCLUSIONS: The proposed staging system is the product of an international effort to adopt a uniform staging system for patients with retinoblastoma to cover the whole spectrum of the disease. (c) 2005 Wiley-Liss, Inc.
BACKGROUND: Although intra-retinal tumor has long been staged presurgically according to the Reese-Ellsworth (R-E) system, retinoblastoma differs from other pediatric neoplasms in never having had a widely accepted classification system that encompasses the entire spectrum of the disease. Comparisons among studies that consider disease extension, risk factors for extra-ocular relapse, and response to therapy require a universally accepted staging system for extra-ocular disease. PROCEDURE: A committee of retinoblastoma experts from large centers worldwide has developed a consensus classification that can encompass all retinoblastoma cases and is presented herein. Patients are classified according to extent of disease and the presence of overt extra-ocular extension. In addition, a proposal for substaging considering histopathological features of enucleated specimens is presented to further discriminate between Stage I and II patients. RESULTS: The following is a summary of the classification system developed-Stage 0: Patients treated conservatively (subject to presurgical ophthalmologic classifications); Stage I: Eye enucleated, completely resected histologically; Stage II: Eye enucleated, microscopic residual tumor; Stage III: Regional extension [(a) overt orbital disease, (b) preauricular or cervical lymph node extension]; Stage IV: Metastatic disease [(a) hematogenous metastasis: (1) single lesion, (2) multiple lesions; (b) CNS extension: (1) prechiasmatic lesion, (2) CNS mass, (3) leptomeningeal disease]. A proposal is also presented for substaging of enucleated Stages I and II eyes. CONCLUSIONS: The proposed staging system is the product of an international effort to adopt a uniform staging system for patients with retinoblastoma to cover the whole spectrum of the disease. (c) 2005 Wiley-Liss, Inc.
Authors: Verónica Pérez; Claudia Sampor; Guadalupe Rey; Andreu Parareda-Salles; Katherine Kopp; Agustín P Dabezies; Gustavo Dufort; Marta Zelter; Juan P López; Marcelo Urbieta; Elisa Alcalde-Ruiz; Jaume Catala-Mora; Mariona Suñol; Diego Ossandon; Adriana C Fandiño; J Oscar Croxatto; María T G de Dávila; Gregory Reaman; Yaddanapudi Ravindranath; Guillermo L Chantada Journal: JAMA Ophthalmol Date: 2018-07-01 Impact factor: 7.389
Authors: Patricia Chévez-Barrios; Ralph C Eagle; Mark Krailo; Jin Piao; Daniel M Albert; Yun Gao; Geeta Vemuganti; Mohammad Javed Ali; Vikas Khetan; Santosh G Honavar; Joan O'Brien; Ann-Marie Leahey; Katherine Matthay; Anna Meadows; Murali Chintagumpala Journal: J Clin Oncol Date: 2019-09-20 Impact factor: 44.544
Authors: Marco A Ramírez-Ortiz; M Veronica Ponce-Castañeda; M Lourdes Cabrera-Muñoz; Aurora Medina-Sansón; Xinhua Liu; Manuela A Orjuela Journal: Cancer Epidemiol Biomarkers Prev Date: 2014-02-12 Impact factor: 4.254