Clinical presentation of retinoblastoma in Malaysia: a review of 64 patients

Abstract

Aim: To analyze the demography, presenting clinical features, spread of the disease of retinoblastoma in patients who were treated in two tertiary hospitals in Malaysia.

Methods: In this retrospective study, information of gender, age, race, presenting clinical features, findings of CT scan orbits and brain, lumbar puncture and bone marrow aspiration results were collected from the medical records of retinoblastoma patients diagnosed in Hospital of Universiti Sains Malaysia and General Hospital of Kuala Lumpur over a period of ten years. The data were collected in the same type of proforma from both hospitals.

Results: A total of 64 patients were treated in both hospitals together, of whom boys and girls were almost equally affected. The mean age of children at presentation was 24.2 (range 3-84) months, 53 (82.8%) children were under 36 months old. The disease was unilateral in 39 (60.9%) patients. The most common presenting signs were leukocoria in 46 (71.8%), followed by proptosis in 21 (32.8%) patients. Routine screening of the siblings of affected children revealed retinoblastoma in 2 patients. There was intracranial extension in 8 (12.5%) and metastasis in bone marrow in 6 (9.3%) patients.

Conclusion: Leukocoria is the most common presentation of retinoblastoma in Malaysia. However, the disease was intraocular in 40 (62.5%) and extraocular (orbital involvement, intracranial and distant metastasis) in 24 (37.5%) patients.

Keywords: leukocoria; proptosis; retinoblastoma; strabismus.