Retinoblastoma - Clinical Spectrum and Treatment Outcome in Children

Abstract

Background: To analyze outcome of
retinoblastoma (RB) in resource limited settings
Methods: In this descriptive study , patients of
retinoblastoma were treated according to UKCCSG
RB 2005 11 protocol with main chemotherapy
including Vincristine, Etoposide, Carboplatin and
intrathecal Cytarabine called JOE protocol. These
protocols were given 3 weeks apart in the
chemotherapy bay on outpatient basis. Total 6
courses were given to these patients. The patients
presenting with huge tumour with proptosis were
offered two courses of chemotherapy (Neoadjuvant)
before Enucleation (Removal of orbit and optic
nerve) done as upfront Enucleation not possible
without mutilating surgery-Exenteration. In our
study Group A included patients who refused
Enucleation and opted out of recommended
treatment plan and Group B patients had
Enucleations done and followed treatment plans
Results: Total 68 patients with age ranging from< 1
to 7 years (95% <5 yrs) were included. Thirty seven
percent presented with bilateral retinoblastoma, fifty
six percent with optic nerve involvement and 18%
with brain metastasis. 30/68(44%) defaulted
treatment, and 33/68 (49%) refused enucleation at
diagnosis. Only 3/68(5%) had bilateral enucleation
and 50/68(73%) unilateral enucleation with laser
therapy in 7/68(10%).Total 52% completed treatment,
26% left against medical advice and 9% expired due
to metastatic and progressive disease and sepsis. 9/68
(13%) patients relapsed. Neoadjuvant chemotherapy
was given in 59% with 61% of total abandonment
and 50% of deaths in this group. The number of
bilateral RB was increased to 37% as compared to
18%.Abandonment increased from 13% to 26%.
Conclusion: Mortality of 9% can be reduced by
early diagnosis and early treatment. Management of
RB needs efficient multidisciplinary team and long
term sustainable programs to improve survival. The
Neoadjuvant group needs extensive follow up and
counselling to decrease abandonment.