Pattern of presentation and outcome of ophthalmic rhabdomyosarcoma in Ibadan

Abstract

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma, and the commonest primary orbital malignancy in children. Studies on ophthalmic RMS are scanty in Nigeria, and other parts of Africa. This study thus describes the pattern of ophthalmic RMS in Ibadan and treatment outcome. Method: A retrospective, non-comparative, consecutive, interventional analysis of patients with ophthalmic RMS in an ocular oncology unit of a teaching hospital over 20 years was carried out. Results: Records of 22 patients, 15 males and 7 females, aged 2 months to 50 years (Median 7 years, Range 5-13 years) were analyzed. All patients presented with visual loss, orbital swelling and proptosis. Histological examination revealed embryonal RMS in 16 (72.7%) patients, alveolar in three (13.6%), pleomorphic in two (9.1%) and "not otherwise stated" in one (4.5%). Treatment outcome was poor as only two (9.1%) patients remained tumour-free 12 and 36 months after diagnosis. Three (13.6%) patients died on admission, while two (9.1%) patients are presently undergoing treatment. Fifteen (68.2%) patients were lost to follow-up, financial constraint and perceived poor response to treatment being the main reasons for incomplete and inconsistent treatment regimen and resultant poor outcome. Conclusion: Demography and morphology of ophthalmic RMS in Ibadan is comparable to other studies. However, treatment outcome remains poor, mainly due to late presentation, incomplete and inconsistent treatment. Strengthening the national health insurance scheme to cater for the indigent and increased public enlightenment on early presentation of cases may go a long way in improving the outcome in our patients.