The value of "en toto" globe submission in the assessment of high-risk retinoblastoma cases and staging.

PURPOSE

To evaluate the importance of "en toto" globe submission in the assessment of high-risk pathological findings in retinoblastoma cases, aiming at identifying the additional value from full globe examination of enucleated eyes (including the calottes).

METHODS

Retrospective histopathological case series of eighty-one enucleated globes with the clinical diagnosis of retinoblastoma, histopathologically examined at King Khaled Eye Specialist Hospital (KKESH) between January 2010 and December 2013. We included retinoblastoma eyes with any type of invasion (more than pT1) in which the globe was submitted "en toto." All cases were histopathologically classified according to the American Joint Cancer Classification (AJCC TNM classification, 2009 6th edition).

RESULTS

81 enucleated globes were examined, out of which 30 globes were classified as high-risk cases (more than pT1). 8 cases had massive choroidal invasion in both the PO sections and calottes. 5 cases had focal choroidal invasion in both. One case has massive choroidal invasion in calottes with very superficial ON head invasion. Two cases were found to have focal choroidal invasion in the calottes with prelaminar ON invasion. In these three cases (10%), the presence of focal or massive choroidal invasion in the calottes has affected the classification.

CONCLUSIONS

This study consolidates the guidelines recommended in the consensus meeting: from the International Retinoblastoma Staging Working Group on the pathology guidelines for the examination and evaluation of prognostic risk factors in retinoblastoma eyes. Full globe submission and examination should be the routine accepted practice universally even in underdeveloped countries.