Not Surprised Surprise (video)

Orbital MRI showed hyperintense T1 and isointense to hypertense T2 signals in the bilateral sphenoid sinuses with enhancement mainly of the right sphenoid sinus. There was a 25 x 27 mm markedly expanded left sphenoid sinus lesion and/or Onodi air cell extending into an opacified left anterior clinoid process with bony erosion and involvement of the left optic canal and orbital apex with resultant compression and enhancement of the left intracanalicular and prechiasmatic optic nerve. Mild perioptic nerve sheath enhancement was present. Paranasal sinus CT showed near to complete opacification of the sphenoid sinuses with an ill-defined erosive lesion. The patient underwent left maxillary antrostomy, left total ethmoidectomy, left sphenoidoctomy and biopsy/debulking of left sphenoid mass. Frozen section analysis was interpreted as a malignant neoplasm and possible esthesioneuroblastoma; however there were abundant apoptosis. Final pathology showed small blue cell tumor positive for CD20, BCL 2 (week, subset), CD30 (subset, 20%) and negative for CD3, CD5, CD10, BCL 6, CD4, CD8. By immunohistochemistry, the tumor cells were negative for keratin, synaptophysin and negative for S100. EBER was negative by in situ hybridization. K167 proliferation index in viable foci was 90%. The final diagnosis was diffuse large B-cell lymphoma (DLBCL). DLBCL is a common non-Hogkins lymphoma, but paranasal sinus DLBCL is very rare with more cases affecting the maxillary and ethmoid sinuses than sphenoid and frontal sinuses. Sphenoid DLBCL usually has very subtle presentation due to its deep location. Headache is the most common presentation, but ophthalmic signs and symptoms such as visual disturbance and diplopia may occur due to the close proximity of orbit to paranasal sinuses and can be very aggressive. The main treatment is chemotherapy. Our patient remained NLP after the decompression with no further response to steroid and was treated with chemotherapy.