Orbital MALT lymphoma

Case contributed by Francis Deng
Diagnosis certain

Presentation

Acute left lower extremity weakness after a peritoneal nodule biopsy. History of seminoma, on active surveillance.

Patient Data

Age: 60 years
Gender: Male
ct

No acute intracranial abnormality.

Soft tissue lesions within the bilateral orbits. The differential includes metastatic disease, lymphoma, sarcoidosis, and orbital pseudotumor.

mri

Bilateral enhancing soft tissue masses with restricted diffusivity centered primarily in the extraconal orbits which abut and may potentially infiltrate the extraocular muscles. Minimal right orbital proptosis. The dominant right-sided mass appears to abut both the inferior and lateral rectus muscle bodies and approaches the orbital apex. The left-sided mass abuts the medial rectus muscle body. No evidence of intracranial extension.

Differential diagnoses include metastatic disease given history of testicular seminoma, however, additional considerations include orbital lymphoma and other lymphoproliferative disorder. Inflammatory etiologies including granulomatous disease or inflammatory pseudotumor could have this appearance but are considered less likely in the differential.

Case Discussion

The initial presentation of lower extremity weakness was felt to be functional weakness after excluding infarct on MRI and vascular stenosis on CTA of the head and neck (not shown). Strength improved without intervention.

Right anterior orbitotomy was performed for biopsy.

Pathology: Low grade B-cell lymphoma, consistent with extranodal marginal zone lymphoma.

Notes: CD20+, PAX5+, CD5-, CD10-, BCL2+. Ki67 is approximately 10%.

The patient was treated with radiation to both orbits with resolution of the masses.

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