A proposal for an international retinoblastoma staging system.
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.


METHODS
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.
Chantada
Guillermo
G
Department of Hematology-Oncology, Hospital JP Garrahan, Buenos Aires, Argentina. gchantada@garraha.gov.ar
Doz
François
F
Antoneli
Celia B G
CB
Grundy
Richard
R
Clare Stannard
F F
FF
Dunkel
Ira J
IJ
Grabowski
Eric
E
Leal-Leal
Carlos
C
Rodríguez-Galindo
Carlos
C
Schvartzman
Enrique
E
Popovic
Maja Beck
MB
Kremens
Bernhard
B
Meadows
Anna T
AT
Zucker
Jean-Michel
JM
D006801:Humans; D009367:Neoplasm Staging / Q000592:standards*; D019572:Retinal Neoplasms / Q000145:classification* / Q000175:diagnosis* / Q000473:pathology; D012175:Retinoblastoma / Q000145:classification* / Q000175:diagnosis* / Q000473:pathology