A proposal for an international retinoblastoma staging system

Autor: Eric F. Grabowski, Bernhard Kremens, F.F. Clare Stannard, Célia Beatriz Gianotti Antoneli, Richard Grundy, Carlos Leal-Leal, Maja Beck Popovic, Anna T. Meadows, Ira J. Dunkel, Carlos Rodriguez-Galindo, François Doz, Enrique Schvartzman, Jean-Michel Zucker, Guillermo L. Chantada
Rok vydání: 2006
Předmět:
Zdroj: Pediatric Blood & Cancer. 47:801-805
ISSN: 1545-5017
1545-5009
Popis: 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.
Databáze: OpenAIRE
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