Immunoglobulin G4 (IgG4)-related disease is a rare immune-mediated fibro-inflammatory disorder with many clinical manifestations. Most commonly affected sites include the pancreas, salivary glands, kidneys, lachrymal glands, biliary tree, aorta and retroperitoneum; testicular involvement is a rare entity. This report describes a man in his 20s who presented with a persistent painless mass in his left testis. After a thorough examination he underwent orchiectomy to rule out any malignancy of the testis. Histopathology and immunohistochemistry were suggestive of IgG4-related disease. Systemic evaluation showed normal IgG4 levels with no other site of involvement. This is a unique presentation of this rare diagnosis and remains a diagnostic challenge which is difficult to distinguish from the more common testicular malignancy. We conducted a review of the current literature regarding IgG4-related disease to complement the case discussion and provide a comprehensive review regarding this diagnostic dilemma.
Keywords: Pathology; Rheumatology; Urology.
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