Background
Retinoblastoma accounts for 2-4% of childhood cancers and is the most common malignant
ocular tumour in childhood. Current treatment modalities allow, in developed countries, a 95%
survival rate at 5 years. This is achieved by a combination of local treatments, systemic
chemotherapy with focal treatments (CT), external beam radiotherapy (EBR) and/or
enucleation. While enucleation causes mainly aesthetic sequelae, EBR increases the risk of
secondary radiation induced tumours. This explains why efforts have been made to develop
new, more conservative treatment techniques. Since a few years, intraarterial administration
of chemotherapy (IAC), and intra-vitreous administration of chemotherapy (IVC), are used as
new conservative treatments with the aim of avoiding enucleation and/or EBR.
Limited data is available regarding the impact of these treatments on the health status of
survivors and especially on their health-related quality of life (HRQoL).
Objective
To assess HRQoL in children and adolescents who survived retinoblastoma and compare the
results between four different treatment modalities: enucleation, EBR, CT and IAC/IVC.
Patients and methods
This is a population-based cross-sectional study. Questionnaires were sent out to all
retinoblastoma survivors who were entirely treated at our centre and who had a minimal followup
of 3 years since end of treatment. The HRQoL was assessed using the KIDSCREEN-52
self-reported and parent proxy version and the total score and the scores of each dimension
were compared between the four different treatment modalities.
Results
Our results showed that the perceived global HRQoL of retinoblastoma survivors was very
good with a total score and each dimension scores over 4 on a scale of 1 to 5. With regards to
the total quality of life score, retinoblastoma survivors who received primary CT (ref group)
scored significantly higher than the other groups. Those who were enucleated scored lower (β
(p-value) = -2,21 (0,04), followed by those who received IAC/IVC (-2,61 (0,02)) and the group
that was treated with EBR (-2,96 (0,03). The CT group reported also better HRQoL on the
school environment dimension. They scored significantly higher compared to those who were
enucleated in the dimension of psychological well-being, and scored higher than the EBR
group in the dimension of self- perception and autonomy. The groups did not differ in physical,
moods and emotions, parent relations, social support and social acceptance domains. Factors
associated with a lower score were: the older age of the patient at study time, older age at first
examination at HOP and HJG, self-reported questionnaires and unilaterality.
Conclusion and perspectives
Our results show that the perceived HRQoL by retinoblastoma survivors was globally good
and that the HRQoL differed according to the treatment received. Patients treated with EBR
have, as expected, the worst HRQoL. To our surprise, systemic chemotherapy, although
related to more general side effects, scored best in almost all domains, even when compared
to the newer local chemotherapies (IAC/IVC).
In order to confirm these data, patients who were not yet available will be included and sociodemographic
factors such as parents’ education, migration background and country of
residence, added since they could also affect the HRQoL.