Adjuvant therapy of histopathological risk factors of retinoblastoma in
Europe: a survey by the European Retinoblastoma group (EURbG)
Abstract
Introduction: Advanced intraocular retinoblastoma can be cured by
enucleation, but spread of retinoblastoma cells beyond natural limits of
the eye may lead to metastatic disease which is related to a high
mortality. Adjuvant therapy after enucleation has been shown to prevent
metastasis in children with risk factors for extraocular retinoblastoma.
However, histological criteria and adjuvant treatment regimens vary and
there is no unifying consensus on the optimal choice of treatment.
Method: Data on guidelines for adjuvant treatment in European
retinoblastoma referral centres were collected in an online survey among
all members of the European Retinoblastoma group (EURbG) network.
Extended information were gathered via personal Email communication.
Results: Data were collected from 26 centres in 17 countries. Guidelines
for adjuvant treatment were in place at 92.3% of retinoblastoma
centres. There was a consensus on indication for and intensity of
adjuvant treatment among more than 80% of all centres. The majority of
centres use no adjuvant treatment for isolated focal choroidal invasion
or prelaminar optic nerve invasion. Patients with massive choroidal
invasion or postlaminar optic nerve invasion receive adjuvant
chemotherapy, while microscopic invasion of the resection margin of the
optic nerve or extension through the sclera are treated with combined
chemo- and radiotherapy. Conclusion: Indications and adjuvant treatment
regimens in European retinoblastoma referral centres are similar but not
uniform. Further biomarkers in addition to histopathological risk
factors could improve treatment stratification. The high consensus in
European centres is an excellent foundation for a common European study
with prospective validation of new biomarkers.