A proposal for an international retinoblastoma staging system.

CHANTADA G, DOZ F, ANTONELI CB, GRUNDY R, CLARE STANNARD FF, DUNKEL IJ, GRABOWSKI

MEDICAL AND PEDIATRIC ONCOLOGY

Año: 2006 p. 801 - 805

0098-1532

BACKGROUND: Although intra-retinal tumor has long been staged presurgicallyaccording to the Reese-Ellsworth (R-E) system, retinoblastoma differs from other pediatric neoplasms in never having had a widely accepted classification systemthat encompasses the entire spectrum of the disease. Comparisons among studiesthat consider disease extension, risk factors for extra-ocular relapse, andresponse to therapy require a universally accepted staging system forextra-ocular disease. PROCEDURE: A committee of retinoblastoma experts from largecenters worldwide has developed a consensus classification that can encompass allretinoblastoma cases and is presented herein. Patients are classified accordingto extent of disease and the presence of overt extra-ocular extension. Inaddition, a proposal for substaging considering histopathological features ofenucleated specimens is presented to further discriminate between Stage I and II patients. RESULTS: The following is a summary of the classification systemdeveloped-Stage 0: Patients treated conservatively (subject to presurgicalophthalmologic classifications); Stage I: Eye enucleated, completely resectedhistologically; Stage II: Eye enucleated, microscopic residual tumor; Stage III: Regional extension [(a) overt orbital disease, (b) preauricular or cervical lymphnode 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 forsubstaging of enucleated Stages I and II eyes. CONCLUSIONS: The proposed staging system is the product of an international effort to adopt a uniform stagingsystem for patients with retinoblastoma to cover the whole spectrum of thedisease.