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It is well known that immunoglobulin G4 (IgG4)-related sclerosing disease usually occurs in the pancreas, bile duct and gall bladder, but not in the mediastinum, trachea or superior vena cava (SVC). In this case, a patient underwent mediastinal mass excision and trachea resection and repair for a mediastinal and intratracheal mass 15 years ago. This mass was diagnosed postoperatively as an inflammatory pseudotumour (plasma cell granuloma). Subsequently, a mass was found to have recurred in the SVC. We performed a mass excision and innominate vein to the right atrium auricle bypass operation. The mass was diagnosed as IgG4-related sclerosing disease. This patient is now disease and recurrence free.

Immunoglobulin G4-related sclerosing disease invading the trachea and superior vena cava in mediastinum