Purpose The Children’s Hospital Lahore is a tertiary government centre with 45 paediatricOncology beds. It receives over 500 new cancer patients per year. The purpose of this study was to analyse treatment and outcome of children with biopsy proven retinoblastoma and discuss various factors causing poor survival.
Materials and methods Retrospective review of 48 patients enrolled between January 2011 – October 2013 was done. Data regarding age, stage, histopathology, treatment, outcome and impact of delayed presentation were analysed. Patients were treated according to UKCCSG Retinoblastoma protocol along with Enucleation.
Results Total 48 patients with age ranging from 1 to 10 (94% <5 yrs) were included. M: F Ratio was 1.3:1. 39/48(81%) presented with optic nerve involvement and 15/48(31%) with brain metastases. Total 21/48 (44%) have completed treatment, 10/48 (21%) are on treatment, 5/48 (10%) left against medical advice (LAMA) and 8/48 (17%) expired due to brain involvement and progressive disease, two of them during early phase of RX. Four patients (8%) were put on palliative treatment on presentation. Enucleation refused initially in 18/48(38%) and 21/48(44%) defaulted at some point of their treatment requiring multiple reminders to attend the clinic. Neoadjuvant Chemotherapy was given in 12/48(25%) to reduce the size of tumour prior to Enucleation. Follow- up is short so overall and EFS cannot be calculated.
Conclusion Mortality of 17% can be reduced by early diagnosis and strategies to combat complications. Majority of patients have advanced extra ocular disease at presentation and a high rate of abandoning treatment. The successful management of retinoblastoma depends on the ability to detect the disease while it is still intraocular. The extent of disease progression usually correlates with the extent of delay in diagnosis. In developing countries, late referrals are strongly associated with orbital and metastatic disease. The prognosis can significantly be improved by public awareness to seek early treatment and establishing safe and effective shared care oncology pathways, multidisciplinary team approach and providing strong social support and extensive psychosocial counselling.
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