Orbital mucosa-associated lymphoid tissue (MALT) lymphoma is an uncommon disease, while the incidence is recently increasing. We describe the F-18 fluorodeoxyglucose positron emission tomography computerized tomography (FDG PET/CT) findings in a case of bilateral orbital MALT lymphomas with a coexisting gastric lesion. Although only the lesion in the left orbit was initially identified on MR imaging, FDG PET/CT scan unexpectedly and additionally could identify the tiny lesion of the contralateral orbit and the gastric lesion. This patient received radiotherapy to all these lesions, with a combination of rituximab monoclonal antibody therapy. The follow-up PET/CT studies at 3, 6, and 9 months and 1.5 years after treatment showed regression or disappearance of all these FDG-avid lesions. Accurate localization and staging are crucial to select an adequate treatment in MALT lymphoma at any location. This case indicates the feasibility of FDG PET/CT scan for accurate localization and staging and also for monitoring treatment in patients with orbital MALT lymphoma.

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