Presentation
Fatigue and migratory arthralgia. Mediastinal mass. Elevated rheumatoid factor, ANA, ENA, anti-SS-A/Ro60 and Ro52.
Patient Data
Mildly heterogeneous and mildly lobulated, cone-shaped prevascular mediastinal mass. No evidence of local invasion, lymphadenopathy, pleural disease or other metastases.
Multiple lower zone predominant perivascular cysts, varying in size from subcentimeter to several centimeters in diameter. Several cysts demonstrate internal strands and vessels.
Case Discussion
CT-guided core biopsy of the thymic mass was performed.
Histology report summary: "Consistent with extra-nodal marginal zone lymphoma (MALT lymphoma), CD-20 positive, BCL-2 positive, PAX-5 weak positive and CD-79A positive."
She had a complete FDG-PET CT response to three cycles of chemotherapy.
Her Sjogren's syndrome was treated with hydoxychloroquine and prednisolone.
Other solid thymic masses that have been linked to Sjogren's syndrome include hyperplasia and rarely carcinoma.
Cystic lung disease in Sjogren’s syndrome is most likely the result of lung destruction by macrophage metalloproteinases. Macrophages are attracted to sites of protein deposition and the cysts with internal strands have similar morphology to those seen in lymphoplasmacytic proliferative disorders 5.
The presence of typical lung cysts in combination with the thymic mass were important in differentiating between rheumatoid disease and Sjogren's syndrome. Both LIP and MALT lymphoma are lymphoproliferative manifestations of Sjogren's syndrome 4.
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