Sporadic Orbital Burkitt Lymphoma in Pediatric Population: A Case Series and Review of the Literature.
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PURPOSE: Burkitt lymphoma (BL) is a highly aggressive B-cell malignancy with 3 recognized subtypes, of which the sporadic form is the most prevalent outside mainland Africa. Sporadic BL typically presents in the abdomen, with fewer than 9% of cases involving the head and neck region; orbital presentation is even rarer. The authors report 4 pediatric cases of orbital BL and provide a comprehensive review of the literature, focusing on clinical presentation, imaging features, histopathology, genetic findings, and treatment outcomes. METHODS: A multicenter retrospective case series combined with a literature review of pediatric BL cases with orbital involvement. RESULTS: The analysis includes 28 published cases and 4 additional cases from the authors' centers. Orbital BL typically presents with acute symptoms due to mass effect, such as proptosis, ophthalmoplegia, and periorbital swelling. All patients in the series were treated according to the French-American-British Mature B-Cell Lymphoma 96 protocol and are currently in remission. Poor prognostic indicators include elevated lactate dehydrogenase levels, central nervous system involvement, bone marrow infiltration, and disease relapse. CONCLUSIONS: Orbital involvement in sporadic pediatric BL is rare but should be considered in the differential diagnosis of a child presenting rapidly progressive proptosis. Prompt recognition and early treatment are associated with favorable outcomes.