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  Table of Contents  
Year : 2021  |  Volume : 58  |  Issue : 2  |  Page : 259-261

Masquerader in oncology: IgG4-related kidney disease presenting as right upper ureteric mass

1 Department of Urology, Amrita Institute of Medical Sciences and Research Center, AIMS Kochi, Kerala, India
2 Department of Pathology, Amrita Institute of Medical Sciences and Research Center, AIMS Kochi, Kerala, India

Date of Submission06-May-2019
Date of Decision19-Sep-2019
Date of Acceptance20-Sep-2019
Date of Web Publication27-Jan-2021

Correspondence Address:
Abhishek Laddha
Department of Urology, Amrita Institute of Medical Sciences and Research Center, AIMS Kochi, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijc.IJC_402_19

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 » Abstract 

Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition that can mimic several diseases and can present as a malignant tumor. We present a case of a 53-year-old woman who presented with a right upper ureteric mass. On pathologic evaluation, a diagnosis of IgG4-RD was made. In the absence of preoperative biopsy and other clinical manifestations, preoperative clinical diagnosis remains challenging and high index of suspicion and accurate pathological evaluation may help in avoiding misdiagnosis.

Keywords: IgG4-related disease, right upper ureteric mass, transitional cell carcinoma

How to cite this article:
Laddha A, Pooleri GK, Thomas A, Bindhu M R, Thomas A. Masquerader in oncology: IgG4-related kidney disease presenting as right upper ureteric mass. Indian J Cancer 2021;58:259-61

How to cite this URL:
Laddha A, Pooleri GK, Thomas A, Bindhu M R, Thomas A. Masquerader in oncology: IgG4-related kidney disease presenting as right upper ureteric mass. Indian J Cancer [serial online] 2021 [cited 2022 Dec 4];58:259-61. Available from:

 » Introduction Top

Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition that can mimic several diseases and can present as a malignant tumor, autoimmune disease, or rheumatologic disease. This tendency to form tumefactive lesions when localized to an organ may lead to misdiagnosis as a malignant lesion.[1],[2] Commonly affected anatomic sites are pancreas, salivary gland, liver, lung, periorbital tissue, thyroid gland, breast, and kidney. We report a case of IgG4-related kidney disease presenting as right upper ureteric mass.

 » Case Report Top

A 53-year-old woman with past history of hypertension and hypothyroidism presented with complaints of right flank pain off and on for 3 years; she underwent ultrasound (USG) followed by computed tomography (CT) abdomen, which was suggestive of a soft tissue attenuating mass measuring 6.5 × 3.5 × 6 cm in the right upper ureter just distal to pelvic-ureteric junction, causing complete obliteration of ureter with severe right-sided hydroureteronephrosis and thinning of the renal parenchyma and enlarged retrocaval lymph node [Figure 1]. All her blood parameters were normal and urine cytology was negative for malignant cells. Whole-body positron emission tomography-computed tomography (PET-CT) was not suggestive of metastatic disease. Preoperative tissue diagnosis (failed ureteroscopy and inconclusive CT guided Tru-cut biopsy from lymph node) failed and she underwent right open radical nephroureterectomy with paracaval lymph node dissection. On gross examination, a gray-white ill circumscribed firm-to-hard lesion was seen occupying the upper ureter. Microscopic examination showed storiform fibrosis with lymphoplasmacytic infiltrate suggestive of IgG4-related kidney disease with no evidence of malignancy [Figure 2]; On immunohistochemistry (IHC), more than 100 IgG4 cells were seen per high power field in many areas further confirming our diagnosis [Figure 3]. Serum IgG4 was not done preoperatively as IgG4 –RD was not suspected. Prednisolone 60 mg per day (for 2 weeks) was started on follow-up which was gradually tapered and stopped at 3 months. She is doing well on 6-month follow-up with normal renal function and serum IgG4 levels 62 mg/dL and reference range from less than 10 mg/dL to 140 mg/dL.
Figure 1: (a) right upper ureteric mass, (b) FDG avid right upper ureteric mass

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Figure 2: (a) Histopathology image (4×) showing storiform fibrosis. (b) Histopathology image (10×) showing storiform fibrosis and dense Lymphoplasmacytic infiltrate

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Figure 3: (a-c) Histopathology sides taken at 10´ (a), 20´ (b), and 40´ (c) showing intense positivity for IgG4 antibiotics (Kit details: IgG4 (MRQ-44), Cell Marque, California. USA)

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 » Discussion Top

IgG4-RDs was classified as a separate disease entity in the 1st international symposium on IgG4-related disease which was held in Boston, Massachusetts.[3] The average age of presentation is between 61 and 70 years and predominantly involves men except for disease involving head and neck.[4],[5] Involvement of the kidney may be direct in the form of involvement of parenchyma or indirect as obstructive uropathy in a case of retroperitoneal fibrosis. Parenchymal involvement is usually in the form of IgG4-related tubulointerstitial nephritis, other forms such as membranous glomerulopathy and Henoch-Schonlein purpura nephritis may also be seen.[6]

Serum IgG4 is a good clinical marker of IgG4-RD and correlates with clinical activity. However, serum levels alone should not be used for diagnosis as it may be elevated in 3 to 7% of a normal healthy population. False-positive elevation of serum IgG4 can be seen in case of connective tissue disorders (Sjogren's syndrome, systemic lupus erythematosus, and rheumatoid arthritis), systemic vasculitis (particularly granulomatosis with polyangiitis), infections (chronic sinusitis, chronic sialadenitis, osteomyelitis, and invasive aspergillosis), malignancies (acute lymphoblastic leukemia, chronic myelomonocytic leukemia, Castleman disease, and malignant lymphoma), pancreato-biliary diseases (such as alcoholic pancreatitis and hepatitis), and immunodeficiency. Normal serum IgG4 levels can be seen in 20 to 40% of patients with IgG4-RD.[7],[8]

In our case, the woman presented with features of locally advanced upper tract mass with high suspicion of upper tract urothelial cancer. She underwent radical nephroureterectomy with the possibility of positive margins, residual disease, and adjuvant treatment. The biopsy came as a pathological surprise, which is a common presentation in many patients with IgG4-RD. Only a few such cases have been reported in the literature so far.[9] Pre-operative biopsy, if technically possible, may help in the identification of such cases. Once diagnosis is confirmed, treatment is mainly medical with glucocorticoids and rituximab. Some patients may have spontaneous remission.[10],[11]

 » Conclusion Top

IgG4-RD tends to form solid masses and can involve multiple organs, mimicking solid organ tumors. Presentation as solid ureteric tumor is rare. It is vital to keep the possibility of IgG4-RD in differential diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al.; Research Program for Intractable Disease by Ministry of Health, Labor and Welfare (MHLW) Japan G4 team. A novel clinical entity, IgG4-related disease (IgG4RD): General concept and details. Mod Rheumatol 2012;22:1-14.  Back to cited text no. 1
Pieringer H, Parzer I, Wöhrer A, Reis P, Oppl B, Zwerina J. IgG4-related disease: An orphan disease with many faces. Orphanet J Rare Dis 2014;9:110.  Back to cited text no. 2
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-92.  Back to cited text no. 3
Brito-Zerón P, Ramos-Casals M, Bosch X, Stone JH. The clinical spectrum of IgG4-related disease. Autoimmun Rev 2014;13:1203-10.  Back to cited text no. 4
Yamamoto M, Takahashi H, Shinomura Y. Mechanisms and assessment of IgG4-related disease: Lessons for the rheumatologist. Nat Rev Rheumatol 2014;10:148-59.  Back to cited text no. 5
Tamai R, Hasegawa Y, Hisano S, Miyake K, Nakashima H, Saito T. A case of IgG4-related tubulointerstitial nephritis concurrent with Henoch-Schönlein purpura nephritis. Allergy Asthma Clin Immunol 2011;7:5.  Back to cited text no. 6
Cortazar FB, Stone JH. IgG4-related disease and the kidney. Nat Rev Nephrol 2015;11:599-609.  Back to cited text no. 7
Li P, Chen H, Deng C, Wu Z, Lin W, Zeng X, et al. Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related disease in Chinese population. Mod Rheumatol 2016;26:583-7.  Back to cited text no. 8
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. 9
Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet 2015;385:1460-71.  Back to cited text no. 10
Khosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol 2011;23:67-71.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]


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