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A proposal for an international retinoblastoma staging system.

Abstract：

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.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Chantada G,Doz F,Antoneli CB,Grundy R,Clare Stannard FF,Dunkel IJ,Grabowski E,Leal-Leal C,Rodríguez-Galindo C,Schvartzman E,Popovic MB,Kremens B,Meadows AT,Zucker JM

doi

10.1002/pbc.20606

keywords:

subject

Has Abstract

pub_date

2006-11-01 00:00:00

pages

801-5

issue

eissn

1545-5009

issn

1545-5017

journal_volume

pub_type

杂志文章,多中心研究

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A proposal for an international retinoblastoma staging system.