PMC3090307_01
A) Axial computed tomography (CT) scan showing extensive soft tissue swelling and mucosal thickening of right ethmoidal sinus (*). B) Axial CT scan (6 days after the first CT scan) showing increased enlargement of the extraconal soft tissue mass in the right orbital roof with inferior displacement of the globe. Note the marked proptosis and "banjo-string" deformation of the optic nerve (arrowhead) and increased mucosal thickening in the right maxillary and frontal sinuses (asterisks in scans). C) Coronal reconstruction view (6 days after the first CT scan) supported extension of the soft tissue mass in the superior lateral right orbit (arrowhead) as well as ethmoid sinusitis (*). D) Axial CT scan shows normal ocular presentation with no evidence of tumor in the orbit or sinus at 21 months post-initiation of treatment.
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Computed tomography (CT) scans of the orbit revealed extensive soft tissue swelling in the right paraseptal orbit and mucosal thickening in the right frontal and maxillary sinuses, and right anterior and medial ethmoidal cells (Figure 1A) consistent with a presumptive diagnosis of preseptal cellulitis. CT scans revealed increased swelling of soft tissue of the right lid, mucosal thickening of right frontal and maxillary sinuses, and proptosis (Figure 1B). Comparison of CT scans confirmed an aggressively expanding extraconal mass undifferentiated from the lacrimal glands, causing a stretched optic nerve yielding a "banjo string" sign, and downward displacement of the globe (Figure 1C). After 10 months of radiation and chemotherapy treatment, CT scans showed complete resolution of the ocular disease (Figure 1D).
A) Histopathology of the lid biopsy tissue showing inflammation and invasion of the lid tissue by large lymphocytes. B) High power magnification of the biopsy revealing the typical appearance of BL characterized by the tightly packed proliferative neoplastic lymphocytes (*) with high nuclear-to-cytoplasmic ratios interspersed with histiocytic cells (large tingible body macrophages) containing dead cell debris (arrows) yielding a "starry sky" appearance (ie, stars in a sky of B cells). (H&E; 400x).
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The histopathological evaluation revealed a homogenous mass infiltrating the soft orbital tissue (Figure 2A) composed of medium sized noncleaved cells with nuclei containing multiple nucleoli and a thin rim of basophilic cytoplasm. Also, the mass contained scattered histiocytic cells (macrophages containing phagocytized cellular debris) yielding a "starry sky" pattern consistent with a lymphoproliferative disorder, possibly high-grade lymphoma (Figure 2B).