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Year : 2021  |  Volume : 11  |  Issue : 1  |  Page : 50-52

A renal unit saving approach to IgG4 related periureteral mass mimicking malignant ureteral tumor

Department of Urology, Sir Ganga Ram Hospital, New Delhi, India

Date of Submission11-Jan-2021
Date of Decision14-Jan-2021
Date of Acceptance20-Jan-2021
Date of Web Publication19-Feb-2021

Correspondence Address:
Dr. Ankit Tyagi
Department of Urology, Sir Gangaram Hospital, Rajinder Nagar, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/cmrp.cmrp_5_21

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Immunoglobulin G4-related disease (IgG4-RD) is a rare disease with few cases mimicking ureteral tumour identified after the ablative surgery. We report a case of IgG4-RD that presented as a ureteral mass which was diagnosed prior to surgery and managed conservatively. A 63-year-old man presented with prior diagnosis of the right ureteric tumour but a radiological review suspected it could be something else. The patient underwent laparoscopic biopsy from the periureteric mass. Biopsy reported to be IgG-4 disease and managed conservatively. This case highlights the rare possibility of IgG4-disease mimicking ureter tumour and if diagnosed prior to extirpative surgery can result in salvage of the kidney.

Keywords: Ankit, gangaram, IgG4 disease, periureteric mass, tyagi, ureteric tumour

How to cite this article:
Tyagi A, Tyagi V, Jain N. A renal unit saving approach to IgG4 related periureteral mass mimicking malignant ureteral tumor. Curr Med Res Pract 2021;11:50-2

How to cite this URL:
Tyagi A, Tyagi V, Jain N. A renal unit saving approach to IgG4 related periureteral mass mimicking malignant ureteral tumor. Curr Med Res Pract [serial online] 2021 [cited 2023 Feb 2];11:50-2. Available from: http://www.cmrpjournal.org/text.asp?2021/11/1/50/309918

  Introduction Top

Immunoglobulin G4-related disease (IgG4-RD) is a rare multisystem disease characterized by IgG4 positive plasma cell infiltration in various organs associated with elevated serum IgG4 concentrations.[1] Only few cases of IgG4-RD had ureteral involvement with mass resembling ‘pseudotumoral’ lesions were reported. Biopsy with review of histopathology and immunohistochemical (IHC) staining for IgG4 are essential for diagnosis.[2],[3] Most of the cases are diagnosed after either nephroureterectomy or segmental ureterectomy but no case was reported in which a diagnosis of IgG4-RD is made before ablative surgery. The commonest findings on biopsy or nephroureterectomy are tubulointerstitial nephritis with anIgG4+, plasma cell-rich inflammatory infiltrate, fibrosis and tubular atrophy.[4] Most patients have multiorgan involvement and shows good response to steroid therapy. These pseudotumor needs to be not only differentiated from ureteral tumour but also should be differentiated from other similar lesions such as inflammatory myofibroblastic tumour and fibrohistiocytic-type inflammatory pseudotumor.[5],[6] Ours is a unique case as the diagnosis was made prior to ablative surgery.

  Case Report Top

A 63-year-old man had complaints of the right flank pain since the past 1 month. Patient underwent non-contrast computed tomography kidneys, ureters and bladder (KUB) [Figure 1] which suggested irregular lesion involving right mid ureter with a significant periureteric component, approximately 3.5 cm length of ureter is involved with obstructive uropathy. Patient also underwent magnetic resonance (MR) urography [Figure 1] which suggested altered signal intensity lesion appearing isointense on T1W in the right mid ureter level at L4 vetebra level with endoluminal as well as extraluminal component with fat stranding and causing proximal hydroureteronephrosis as shown in [Figure 1]. Initially, the patient was diagnosed to have ureteral tumour and advised for by nephroureterectomy. Then, the patient reported to our department. Patient, s MR imaging (MRI) film was reviewed closely and as most of the component of the disease was looking to be extraluminal so a decision was made to do ureteroscopy first followed by diagnostic laparoscopy if no mass is seen within the ureter.
Figure 1: Comparison of pre-treatment (upper 2 images) computed tomography kidneys, ureters and bladder and magnetic resonance kidneys, ureters and bladder showing right periureteric lesion with hydronephrosis and post-treatment (lower 2 images) magnetic resonance kidneys, ureters and bladder showing decrease in size of periureteric lesion with the resolution of hydronephrosis and patent ureteric lumen

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Patient underwent ureteroscopy which suggested intraluminal narrowing with mild mucosal abnormality but no evidence of any intraluminal ureteric mass. Hence, we did a diagnostic laparoscopy in the same setting with biopsy from the periureteric mass by tru-cut biopsy needle through port after putting double J (DJ) stent. Biopsy from the mass suggested inflammatory lesion in the periureteric region composed of lymphocytes, plasma cells and eosinophils with focal prominence of IgG4 plasma cells (20–30/HPF) as shown in [Figure 2], also there was no evidence of malignancy which was confirmed by IHC staining. Subsequently, IgG4 levels were done which were significantly raised (210 ng/ml). Urinary cytology was also sent which was negative. Patient was put on steroids (omnacortil: 40 mg/day) and azathioprine (150 mg/day) after rheumatology consultation and responded well to the treatment. On follow-up MRI was done which suggested decrease in size of periureteric lesion and no evidence of hydroureteronephrosis.
Figure 2: Focal prominence of IgG4 plama cells

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Patient's DJ stent was then removed and subsequent investigation showed a non-obstructive kidney.

  Discussion Top

Tumour of upper urinary tract is a rare disease which constitute approximately 10% of renal neoplasms and around 5% of urothelial carcinomas. Ten years’ analysis of upper tract cancer Chitale et al. found that 10% of the cases were found to have benign pathology after nephroureterectomy.[7]

According to a study done by Hong et al., out of the 244 patients who underwent Radical Nephroureterectomy for suspected upper tract urothelial carcinoma, 7 had benign lesions out of which 2 were IgG4 related.[8] Thus, in every case of ureteric, periureteric or pelvic mass a benign possibility should be ruled out before doing a nephroureterectomy if there is high index of suspicion for the lesion to be not malignant which is not possible all the time.

In our case, the possibility of something else was thought because of periureteric component of the lesion and thus laparoscopic guided biopsy was done to make a conclusive diagnosis before ablative surgery.

IgG4-RD is a systemic inflammatory disorder which is characterized by dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, mass-forming lesions, storiform fibrosis and with raised serum IgG4.[9]

The annual incidence of IgG4-realted kidney disease is 0.28–1.08/100,000 population. Most patients had multiorgan involvement out of which renal involvement is seen in 23% patients.[10]

IgG4-RD involving ureter is a very rare disease, with only 13 cases reported to date. In each of the 13 reported cases, histological diagnosis was done after surgical resection,[11] the difference in our study is that diagnosis was made before the ablative surgery thus saving the kidney and ureter.

Histopathology and IHC staining for IgG4 are the most valuable in diagnosis of IgG4-related systemic disease.[12]

The current approach to treatment of the disease includes ‘Watch-and-wait’ strategy, systemic glucocorticoids, azathioprine, rituximab and steroid-sparing immunomodulators.[13]

Although spontaneous remission is possible in IgG4-RD, most patients require systemic glucocorticoids to suppress renal inflammation.[14]

In most of the studies, diagnosis is made after ablative surgery like segmental ureterectomy or nephroureterectomy, but there is no published data in review of literature in which diagnosis is made by diagnostic laparoscopy and tru-cut biopsy thus resulting in saving the renal unit.

  Conclusions Top

All the masses in ureter are not malignant tumour. If there is any doubt about the nature of the ureteral mass and/or mass is more periureteric, getting a laparoscopic biopsy is a good idea. Although rare but periuretric mass may be a IgG4 disease and if diagnosed early can be managed conservatively without any ablative surgery. Large clinical trials are needed for studying clinical manifestations, approaches to diagnosis and treatment of patients with IgG4-RD.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539-51.  Back to cited text no. 1
Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: Introducing the Mayo Clinic's HISORt criteria. J Gastroenterol 2007;42 Suppl 18:39-41.  Back to cited text no. 2
Lin YJ, Chen PC, Chen HA, Li CF. IgG4-related retroperitoneal fibrosis: The first reported case in a Chinese population. Int J Rheum Dis 2010;13:e70-3.  Back to cited text no. 3
Dhall D, Suriawinata AA, Tang LH, Shia J, Klimstra DS. Use of immunehistochemistry for IgG4 in the distinction of autoimmune pancreatitis from peritumoral pancreatitis. Hum Pathol? 2010;41:643-52.  Back to cited text no. 4
Marando A, D'Ambrosio G, Catanzaro F, La Rosa S, Sessa F. IgG4-related disease of the ureter: Report of two cases and review of the literature. Virchows Arch 2013;462:673-8.  Back to cited text no. 5
Moriarty MA, Dahmoush L, Nepple KG. IgG4 related disease of the ureter (inflammatory pseudotumor). J Urol 2014;191:1126-7.  Back to cited text no. 6
Chitale S, Mbakada R, Irving S, Burgess N. Nephroureterectomy for transitional cell carcinoma-The value of pre-operative histology. Ann R Coll Surg Engl 2008;90:45-50.  Back to cited text no. 7
Hong S, Kwon T, You D, Jeong IG, Hong B, Hong JH, et al. Incidence of benign results after laparoscopic radical nephroureterectomy. JSLS 2014;18:1-3.  Back to cited text no. 8
Yu KH, Chan TM, Tsai PH, Chen CH, Chang PY. Diagnostic performance of serum IgG4 levels in patients with IgG4-related disease. Medicine (Baltimore) 2015;94:e1707.  Back to cited text no. 9
Lang D, Zwerina J, Pieringer H. IgG4-related disease: Current challenges and future prospects. Ther Clin Risk Manag 2016;12:189-99.  Back to cited text no. 10
Abe H, Morikawa T, Araki A, Shima T, Nakatsu H, Fukayama M, et al. IgG4-related periureteral fibrosis presenting as a unilateral ureteral mass. Pathol Res Pract 2011;207:712-4.  Back to cited text no. 11
Lei WH, Xin J, Shao CX, Mao MF, Zhu CY, Wu CF, et al. IgG4-related kidney disease mimicking malignant ureter tumor: Case report and literature review. Medicine (Baltimore) 2016;95:e2550.  Back to cited text no. 12
Vasaitis L. IgG4-related disease: A relatively new concept for clinicians. Eur J Intern Med 2016;27:1-9.  Back to cited text no. 13
Saeki T, Saito A, Hiura T, Yamazaki H, Emura I, Ueno M, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med 2006;45:163-7.  Back to cited text no. 14


  [Figure 1], [Figure 2]


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