[Treatment of retinoblastoma: Senegalese experience]. / Thérapeutique du rétinoblastome: expérience sénégalaise.

ABSTRACT

INTRODUCTION: Retinoblastoma is a malignant tumor arising from retinal stem cells or retinoblasts. Its management has been well determined. The goal of this study is to report preliminary results obtained with combined chemotherapy and surgical treatment. PATIENTS AND METHODS: Our transverse prospective study includes retinoblastomas diagnosed and treated on our service from January 2006 to December 2010. Fundus examination under general anesthesia and radiological examination, orbital/brain CT or if unavailable, ultrasound, were systematically performed. MRI was able to be obtained in one case. The patients received 6 courses of chemotherapy (vincristin-carboplatin-etoposide or vincristin-cyclophosphamide), including 2 preoperative courses and 4 postoperative courses. A surgical procedure (enucleation or exenteration) supplemented the treatment, followed by histologic analysis of the specimen. External radiation therapy and conservative management were not available. RESULTS: Fifty-nine patients were treated. Median age at time of diagnosis was 9months (range 2months to 7years); the gender ratio was 1.18. Tumors were bilateral in 12 cases and unilateral in 47 cases, intraocular in 34 cases and extraocular in 25 cases. Optic nerve involvement was confirmed by histology in 12 cases. Twenty-one patients died from recurrence and/or metastatic disease, or during chemotherapy. Seven were lost to follow-up, and 31 have survived uneventfully. DISCUSSION: Improvement of mortality is still our major concern. Radiation therapy is essential in the presence of optic nerve involvement. CONCLUSION: Long-term survival will determine the efficacy of this form of management. The contribution of new therapeutic means will improve these results.