A proposal for an international retinoblastoma staging system

• Published in: Pediatric Blood & Cancer Vol. 47; no. 6; pp. 801 - 805
• Main Authors: Chantada, Guillermo, Doz, François, Antoneli, Celia B.G., Grundy, Richard, Clare Stannard, F.F., Dunkel, Ira J., Grabowski, Eric, Leal-Leal, Carlos, Rodríguez-Galindo, Carlos, Schvartzman, Enrique, Popovic, Maja Beck, Kremens, Bernhard, Meadows, Anna T., Zucker, Jean-Michel
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.11.2006; Wiley
• Subjects: Biological and medical sciences; classification; extra-ocular; General aspects; Humans; Medical sciences; metastasis; Neoplasm Staging - standards; Retinal Neoplasms - classification; Retinal Neoplasms - diagnosis; Retinal Neoplasms - pathology; retinoblastoma; Retinoblastoma - classification; Retinoblastoma - diagnosis; Retinoblastoma - pathology; staging; Tumors; Human; Pediatrics; Nervous system diseases; Retinopathy; International system; Stage classification; Malignant tumor; Metastasis; classification; extra-ocular; Eye disease; Cancerology; Retinoblastoma; Child; staging
• ISSN: 1545-5009; 1545-5017; 1096-911X
• DOI: 10.1002/pbc.20606

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. Pediatric Blood Cancer 2006;47:801–805. © 2005 Wiley‐Liss, Inc.