A 37-year-old, previously healthy woman presented during her first trimester of pregnancy with a two-week history of rapidly progressive proptosis in the left eye. Clinical examination revealed limited left supraduction and diplopia in upward gaze. Orbital magnetic resonance imaging showed a medial orbital mass adjacent to the globe with secondary proptosis. Pathologic examination of a biopsied specimen of the orbital mass and subsequent immunophenotyping by flow cytometry revealed an extranodal marginal zone B-cell lymphoma. Clinical and histological features as well as a review of the literature are described.
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