| Summary: | Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ disorder predominantly occurring in middle-aged to elderly male patients characterized by multi-organ fibrosis, specific pathological findings of storiform fibrosis with IgG4-positive plasma cell infiltration, and elevated serum IgG4 level. We herein report a rare presentation of IgG4-RD forming an isolated mass in the middle mediastinum mimicking a mediastinal tumor and discuss the clinical significance of mediastinal IgG4-RD. Case presentation An 82-year-old male patient without any symptom was referred due to left middle mediastinal mass (3.8 × 2.4 cm). Because of suspected lymphoma, Castleman’s disease, and lymphangitis due to tuberculosis, we performed a thoracoscopic resection for diagnosis and treatment. The mass was yellowish white with well-encapsulated, and storiform fibrosis with plasma cell infiltration, and obliterative phlebitis were observed microscopically. Additional immunohistochemical stain revealed IgG4-RD. Other radiological findings and serological results did not show evidence of other organs being affected from IgG4-RD nor autoimmune diseases. He is now followed at outpatient clinic without additional treatment for over a year, and an enhanced computed tomography does not show any recurrence. Conclusion It was a rare presentation of IgG4-RD forming isolated middle mediastinal mass, which suggests that we might suspect IgG4-RD for undetermined mediastinal mass in case of middle to elderly male patient.
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