Article Text

Download PDFPDF
Unique case of IgG4-related disease of the renal pelvis involving the inferior vena cava masquerading as locally advanced urothelial cancer
  1. Mohammed Al-Zubaidi1,
  2. Steve McCombie1,2,
  3. Nicole Swarbrick3 and
  4. Dickon Hayne1,2
  1. 1Department of Urology, Fiona Stanley Hospital, Murdoch, Western Australia, Australia
  2. 2Harry Perkins Institute of Medical Research, Murdoch, Western Australia, Australia
  3. 3Histopathlogy (Pathwest), Fiona Stanley Hospital, Murdoch, Western Australia, Australia
  1. Correspondence to Dr Mohammed Al-Zubaidi; mohammed_az1985{at}


Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease which can affect any organ or tissue in the body but most commonly affects the pancreas, biliary ducts, salivary glands, ocular system and lymph nodes; renal involvement is relatively uncommon and there are no previous reported cases of inferior vena cava involvement. Herein, a 48-year-old Asian man with an unremarkable medical history was found to have an obstructing right renal pelvis mass extending to and involving the inferior vena cava, highly suspicious for upper tract urothelial carcinoma that could not be ruled out based on ureteroscopy and urine cytology. Open radical nephroureterectomy with enbloc resection of a segment of the inferior vena cava and left renal vein ostium was performed, with reconstruction of the inferior vena cava and left renal vein with polytetrafluoroethylene grafts. Final histopathology confirmed the diagnosis of IgG4-related disease. This case demonstrates that IgG4-related disease can mimic upper tract urothelial cancer and should be considered as a diagnosis in atypical presentations of tumours of the upper urinary tract.

  • urological surgery
  • urological cancer
  • pathology
  • radiology
  • vascular surgery

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors MA-Z wrote the manuscript and obtained consent from the patient. SM reviewed the manuscript. NS reviewed the manuscript and provided pathology images. DH reviewed the manuscript and was a supervisor.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.