IgG4-related disease diagnosed in a paratesticular pseudotumor simulating malignancy, in a patient with retroperitoneal fibrosis

Abstract IgG4 related disease is a term used to describe a fibroinflammatory condition characterized by storiform fibrosis, inflammation with a dense lymphoplasmocytic infiltrate rich in plasma cells expressing IgG4, and often, if not always, raised serum levels of IgG4. We report a case of a patient with a past history of retroperitoneal fibrosis presenting with a swollen left testicle, who underwent an orchidectomy due to suspicion of malignancy. The surgical specimen revealed a paratesticular pseudotumor with histopathological and immunohistochemical characteristics of IgG4 related disease. To the best of our knowledge, just nine such cases have previously been reported, of which only three were manifestations of systemic disease, as in the present case. Whilst it is important to recognize the clinical and radiological features of this entity, histopathology is often essential in order to confirm the diagnosis.