Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed VI university hospital of Marrakech

Abstract

Retinoblastoma is the most frequent childhood intraocular tumor. The aim of our study is to evaluate the clinical features and management of extra-ocular retinoblastoma in the Mohamed VI university hospital of Marrakech. Retrospective case series, the patient's records were reviewed for patient and tumor features, ocular management, histopathological findings, and patient survival. Over a period of three years, 35 eyes were diagnosed with retinoblastoma; 12 children (16 eyes) (46%) had extra-ocular retinoblastoma. Mean age was 27 months, 60% were males. Six cases had unilateral tumor, five bilateral and one case of trilateral retinoblastoma. There was no positive family history, proptosis was the mean mode of presentation (41,6%) followed by staphyloma (25%) orbital cellulitis (25%) and hyphema(8,3%). The median lag period was 18 months. On imaging and histopathological analysis, there was extrascleral involvement in 41.6%, involvement of orbital part of optic nerve (75%), of orbital muscles (50%) and eyelids in 16.6%. the surgical treatment included according to the degree of extension enucleation (75%) or exenteration (25%) associated to chemotherapy in all cases and one case of external beam radiation. There were 2 cases of orbital recurrence, one death and no metastases at 30 months follow-up.Orbital retinoblastoma still stands as a tall challenge requiring multi-modal and multi-disciplinary approach. Although the survival has increased over the last few years, lack of access to medical facilities, lack of education about the need for early medical attention and cultural resistance to enucleation continue to contribute to an epidemic of extra ocular disease at diagnosis in the developing world.

Primary orbital rhabdoid tumour masquerading as atypical persistent foetal vasculature

“…In cases of late diagnosis, extraocular retinoblastoma can present with proptosis, hyphaemia and features of orbital cellulitis. Patients with extraocular progression are generally older (around 2–3 years of age) and have a lack of access to healthcare resources causing a late stage diagnosis 2 3. This differs from our case in clinical history with no family history, no bilateral progression as well as signs with a lack of leucokoria in the early stages and rapid progression to proptosis at an early age.…”

Lag time to diagnosis as a predictor of mortality in children with extraocular retinoblastoma: Experience from a developing country

“…Refusal of enucleation by parents is common because it means loss of vital organs which greatly affects aesthetics and is crucial for patients 25 . Research in Morocco also shows that traditional medicine before going to the hospital is one of the causes of delay in diagnosis 26 …”

“…25 Research in Morocco also shows that traditional medicine before going to the hospital is one of the causes of delay in diagnosis. 26 Parental education in this study is categorized into two, junior high school or lower and senior high school or higher. This finding shows a significant relationship with 24 months survival with p = .047 and HR 1.96 (95% CI: 1.01-3.8) in bivariate analysis, but not in multivariate analysis, with p = .264 and HR 1.56 (95% CI: .72-3.39).…”

Imagerie des tumeurs orbitaires chez l'enfant

“…Rétinoblastome : Le rétinoblastome est la tumeur intraoculaire la plus commune de l'enfance, 90% des cas surviennent avant l'âge de 5 ans [ 1 ] rejoignant ainsi nos résultats. La leucocorie est le signe le plus fréquent du rétinoblastome, suivi par le strabisme [ 2 ]; dans notre étude, le signe le plus fréquent était l'exophtalmie. L'imagerie radiologique peut être utilisée pour confirmer le diagnostic et déterminer la stadification.…”