Comparison of Staging Systems for Extraocular Retinoblastoma

Abstract

IMPORTANCE Different staging systems for extraocular retinoblastoma have been published, but to date they have not been validated in large cohorts.OBJECTIVE To review 533 patients (and pathology slides) with retinoblastoma included in 4 protocols (January 1, 1988, to December 31, 2009 who received uniform treatment.DESIGN AND SETTING Retrospective review in a hospital setting. A critical analysis for detecting inconsistencies and omissions was performed.PARTICIPANTS Patients were reclassified according to the modified St Jude Children's Research Hospital staging system, Grabowski-Abramson staging system, International Retinoblastoma Staging System (IRSS), and American Joint Committee on Cancer TNM staging system. MAIN OUTCOME AND MEASUREThe main outcome measure was disease-free survival (DFS), considering only extraocular relapse as an event. RESULTSIn the IRSS and the St Jude system, higher stages correlated with poorer DFS. For intraocular disease, only the TNM system and the IRSS included pathological definitions, and all systems except for the IRSS included substages without differences in DFS. Omissions of factors significantly associated with lower DFS included scleral invasion by the TNM system and massive choroidal invasion by the Grabowski-Abramson system. The St Jude system omits postlaminar optic nerve involvement, but this omission did not correlate significantly with lower DFS because these patients received intensive therapy. No differences in DFS were observed among substages for metastatic disease except for the presence of central nervous system involvement. All staging systems had inconsistencies in definitions of extent of disease. No system provides guidelines for imaging. CONCLUSIONS AND RELEVANCEOnly the IRSS and the St Jude system allowed for grouping of patients with increasing risk of extraocular relapse. For lower stages, only the IRSS considers all unequivocal pathological prognostic factors. For higher stages, all systems had redundant information, resulting in an excess of substages.

Recent Advances in Retinoblastoma Treatment

“…Invasion to the prelaminar optic nerve is not related signifi cantly with a higher risk for extraocular relapse (Chantada et al 2013b ;Kaliki et al 2013 ). Invasion to the prelaminar optic nerve is not related signifi cantly with a higher risk for extraocular relapse (Chantada et al 2013b ;Kaliki et al 2013 ).…”

Histopathological Characteristics and Classification for Prognostic Indicators

“…It is considered one of the extraocular classification systems besides the International Retinoblastoma Staging System (IRSS) [47]. Unlike other staging systems, TNM and IRSS were developed by multiple specialists and medical centers worldwide [48,49]. The TNM8 system is subclassified into clinical, pathological, and hereditary classifications which stand for cTNM, pTNM, and H, respectively [45].…”

“…Furthermore, pathological staging necessitates enucleation of the eye to classify primary tumor plus examining local extension or distant metastasis by biopsies or total resection if present. The pTNM categories are divided to pT, the histological staging of the primary tumor after biopsy or enucleation, pN stands for microscopic examination of lymph node biopsy, and clinical plus microscopic examination of distant metastatic lesions implies as M [45,49]. However, on initial evaluation application of clinical or cTNM, subclassification is wildly used by ophthalmologist which subclassify the tumor burden to intraretinal, intraocular, advanced intraocular, or extraocular [47].…”

The bone marrow metastasis niche in retinoblastoma

“…One of these, the IRSS system (International Retinoblastoma Staging System) classifies patients according to an increased risk of extra-ocular relapses. Based on this system, Rb is classified into five stages, with stage IV being associated with hematogenous metastases [6].…”