A 68-year-old female with a two-month history of right sided ocular pruritus, progressive local edema, and wasting was referred for evaluation and biopsy of a soft-tissue mass, identi fied on a previous computed tomography (CT) scan, centered in the right orbit and extending to the maxillary, ethmoid, and sphenoid sinuses. At one month after the initial CT scan, she presented to our facility with proptosis, severe ocular pain, and ocular secretion. A new CT scan showed that the mass had ex panded, invading the inferior and medial orbital walls, as well as the ethmoid bone, occupying the entire orbit and extending to the skin. On magnetic resonance imaging (MRI), the mass showed hypointensity on a T1-weighted image (WI) and isoin tensity on a T2WI, together with restricted diffusion, intense contrast enhancement, and skull base invasion (Figure 1). Im munohistochemical analysis demonstrated large poorly differen tiated cells, numerous mitotic figures ( 10/high-power field), and foci of necrosis, as well as positivity for chromogranin A, CD56, and membrane epithelial antigen (Figure 2), rendering a.
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