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Case Reports in Urology
Volume 2014, Article ID 275850, 3 pages
http://dx.doi.org/10.1155/2014/275850
Case Report

IgG4-Related Disease in a Urachal Tumor

1Department of Urology, University of Kansas Medical Center, 3901 Rainbow Boulevard, MS 3016, Kansas City, KS 66160, USA
2Department of Pathology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA

Received 12 June 2014; Revised 18 July 2014; Accepted 1 August 2014; Published 18 August 2014

Academic Editor: David Duchene

Copyright © 2014 Travis W. Dum et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

IgG4-related disease is a newly recognized fibroinflammatory disorder that has the ability to affect nearly every organ system. It is characterized by tumefactive lesions and fibrosis and closely mimics neoplasms. Only one case of IgG4-related bladder mass has been reported in the literature, but there are no reports of IgG4-related disease in a urachal mass. Herein, we report a 26-year-old male who initially presented with symptoms of recurrent UTI. Work-up revealed a 6 cm urachal tumor, a 1.4 cm pulmonary lesion, and mediastinal lymphadenopathy; all metabolically active on PET scan and suspicious for urachal adenocarcinoma. Lung lesion fine needle aspiration and TURBT pathology revealed inflammation but no evidence of malignancy. The patient underwent a partial cystectomy and umbilectomy with pathology demonstrating dense plasmacytic cells, a high rate of immunohistochemistry staining positive for IgG4 plasma cells, a storiform pattern of fibrosis, and an obliterative phlebitis. Furthermore, the patient had an elevated serum IgG4 level of 227 mg/dL (range 2.4–121 mg/dL). IgG4-related disease is a newly recognized fibroinflammatory disorder that can mimic neoplastic processes and a high index of suspicion and accurate tissue pathology is necessary for an accurate diagnosis.