Introduction: Burkitt lymphoma (BL) is a highly aggressive B-cell non-Hodgkin lymphoma rarely affecting the paranasal sinuses. A case of a 14-year-old boy presenting with an unilateral abducens paresis as the only clinical symptom is reported. He was initially treated in a children's hospital and diagnostic testing of cerebrospinal fluid showed normal results.

Material and methods: A systematic review of the literature using Medline and Embase electronic databases was conducted. Radiological findings were analysed and detailed intraoperative photo- and video-documentation was performed. Histological features of the resected material from the sphenoid sinus were evaluated.

Results: Using the keywords "Burkitt lymphoma" and "sphenoid sinus" only 9 publications could be identified throughout the current literature. Non-contrast CT-scan of the skull showed a highly attenuating mass within the left sphenoid sinus but no bone destruction. MR imaging showed a solid marginally enhancing mass in contrast-enhanced T1-W-imaging of the left sphenoid sinus with no dural enhancement. Intraoperatively, the tumor presented as a soft tissue mass, severly pulsating, strongly bleeding and completely obliterating the left sphenoid sinus. Histopathological examination showed massive lymphomatous cell proliferation and the tumor cells showed a strong expression of CD20, Pax 5, BCL 2, CD 10 and Ki 67 (> 90%).

Conclusion: BL rarely develops within paranasal sinuses, especially in children. However, BL can mimic other infectious and tumorous conditions which can mislead the diagnosis and delay treatment. As prognosis is highly related to a soon start of chemotherapy treatment, early endoscopic biopsy of suspicious lesions of the paranasal sinuses should be performed.

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