IgG4-related disease of the paratestis in a patient with Wells syndrome: a case report

Abstract

BackgroundWe report a case of a 33-year-old man who presented with immunoglobulin (Ig)G4-related disease (IgG4-RD) forming a pseudotumor in the left paratesticular region during oral administration of corticosteroid for Wells syndrome, which involves cellulitis with eosinophilia.Case presentationThe patient was introduced to our institution from a private hospital with a 3-month history of asymptomatic left scrotal mass. A 5-cm diameter nodule was palpable in the left scrotum. Tumor lesion in the left paratestis involving the epididymis and spermatic cord was observed on computed tomography and magnetic resonance imaging. Blood testing showed no abnormalities other than a minimal increase in C-reactive protein levels. Urine examination likewise revealed no significant findings. Left radical orchidectomy was performed under a diagnosis of left paratesticular neoplasm suspected as malignant tumor. The tumor was pathologically identified as IgG4-RD of the left paratestis involving the epididymis and spermatic cord.ConclusionsWe present a first description of IgG4-RD in a patient with Wells syndrome and the ninth case of IgG4-RD in a scrotal organ, and discuss this very rare entity with reference to the literature.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_225

Case of hydrocele testis caused by vaginalitis, a rare manifestation of immunoglobulin G4‐related disease

“…So far, just 10 cases of paratesticular (fibrous) pseudotumor have been reported as intrascrotal manifestations of IgG4-RD, half of which presented with multi-organ disease. [2][3][4][5][6][7][8][9] All but one case were submitted to surgical excision because of the inability to exclude malignancy as a potential etiology. The most recent case avoided orchiectomy, and was successfully treated with steroid therapy owing to the accumulating knowledge of this rare entity.…”

IgG4 Related Disease of Epididymis, Mimicking Testicular Malignancy – A Rare Entity

“…The genitourinary system comprising of the kidneys, ureters, urinary bladder, urethra, prostate gland, testes and penis is one of the multiple organ systems to be affected by IgG4-RD [4]. Involvement of epididymis is uncommon compared to kidney, ureter, bladder and prostate [5]. We report one such entity, where an isolated epididymal IgG4 disease was treated with total orchiectomy.…”

Editorial Comment to Immunoglobulin G4‐related disease in the urinary bladder

“…Therefore, urologists should pay particular attention to tumor‐like lesions of IgG4‐related disease. The histology of IgG4‐related disease seems to show consistent appearance, irrespective of affected organs, namely such as the formation of lymphoid follicles, the prominent infiltration of plasma cells in the interfollicular area, frequent infiltration of eosinophils, storiform fibrosis and obstructive phlebitis . However, the frequency of these characteristic findings seems to slightly differ from organ to organ.…”

“…Immunoglobulin G4 (IgG4)‐related disease often involves various anatomical sites including pancreas, biliary tract, retroperitoneum, salivary gland, lacrimal gland, lung, thyroid gland, breast, lymph node, aorta and hypophysis . Additionally, this disease could affect the genitourinary system, such as kidneys, renal pelvis, prostate, paratesticular area and urinary bladder . Previously, three cases involving urinary bladder have been reported .…”

“…In corticosteroid‐resistant cases, immunosuppressive agents can be generally applied. In 18 F‐fluorodeoxyglucose‐positron emission tomography, the accumulation can be observed in IgG4‐related disease as well as malignancy . Therefore, urologists should pay particular attention to tumor‐like lesions of IgG4‐related disease.…”