RETINOBLASTOMA By Dr Suhail Mushtaq Boobak MBBS DOMS

RETINOBLASTOMA By Dr. Suhail Mushtaq Boobak MBBS, DOMS, MCPS, ICO(UK), FCFS, FRCS(UK) Associate Professor Ophthalmology Sargodha Medical College, University of Sargodha

• Most common primary intraocular malignancy of childhood • 3% of all childhood cancers • 1: 17, 000 live births

Histology • Composed of small basophilic cells(Retinoblast)With large hyperchromic nuclei and scanty cytoplasm • Rosettes formation

1 - Flexner-Wintersteiner rosette - Central lumen surrounded by tall columnar cells, the nuclei lie away from the lumen 2 - Homer-Wright rosettes – No lumen 3 - Fleurettes-S of cells with long cytoplasmic processes. resembles a bouquet of flowers

Patterns of tumour spread 1 -Growth pattern endophytic(into the vitreous) exophytic(into the subretinal space) 2 - Optic nerve invasion 3 - diffuse infiltration of the retina 4 -Metastatic spread to regional lymph nodes, lungs and brain

Genetics • Heritable 40% • Non-heritable 60% Presentation • Leukocoria 60% • Strabismus 20% • Secondary glaucoma

• Uveitis • Orbital inflammation---- Proptosis Signs Intraretinal tumour--- Homogenous dome shaped white lesion with irregular borders and calcification

Endophytic tumour --- White mass in the vitreous Exophytic--- Multilobulated mass in subretinal space, retinal detachment

• Investigation 1 - USG 2 - CT Scan 3 - MR scan 4 - Systemic– Bone scan, bone marrow aspiration, lumber puncture

• Genetic studies • Treatment Small tumours 1 - Photocoagulation 2 -Cryotherapy 3 - Chemotherapy Medium-sized tumours 1 - Brachtherapy 2 - Chemotherapy 3 - Radiotherapy

Large tumours 1 - Chemotherapy 2 - Enucleation Follow-up • Tumour regress to ‘cotton-cheese’ calcified mass • New tumour may develop • Exam. after every 2 -8 weeks until 3 years age

Differential diagnosis 1 - Congenital cataract 2 -Persistant anterior fetal vasculature 3 - Coats disease 4 - Retinopathy of prematurity 5 -Toxocariosis 6 -Uveitis 7 -Retinal dysplasia 8 -Retinoma 9 -Retina astrocytoma

Thanks