Korean J Radiol.  2012 Dec;13(6):803-807. 10.3348/kjr.2012.13.6.803.

Immunoglobulin G4-Related Sclerosing Disease Involving the Urethra: Case Report

• Choi JW
• Kim SY
• Moon KC
• Cho JY
• Kim SH

• KMID: 1397512
• DOI: http://doi.org/10.3348/kjr.2012.13.6.803

Abstract

Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.

Keyword

Immunoglobulin G4-related sclerosing disease; Inflammatory pseudotumor; Urethra; IgG4

MeSH Terms

Aged
Autoimmune Diseases/diagnosis
Female
Granuloma, Plasma Cell/*diagnosis
Humans
Immunoglobulin G/*immunology
Pancreatitis/diagnosis/immunology
Sclerosis
Urethral Diseases/*diagnosis/immunology

Figure

• 

  Fig. 1 CT, MR, US, and histologic findings in 72-year-old woman with IgG4-related inflammatory pseudotumor in urethra. A. Coronal reformatted CT image reveals a rim-enhancing soft tissue around urethra (arrows). B. Axial T1-weighted MR image (TR/TE, 790/14) reveals isointense soft tissue (arrows) around urethra. C. Sagittal T2-weighted MR image (TR/TE, 4800/95) reveals iso- to slightly hyperintense mass (arrows) around urethra. D. Axial diffusion weighted MR image (TR/TE, 4800/79) demonstrates intense high signal intensity in urethral mass (arrowheads). E. Axial apparent diffusion coefficient (ADC) map demonstrates concordant low ADC values of urethral mass (arrowheads). F. On gdolinium-enhanced fat-saturation T1-weighted MR images (TR/TE, 2.9/1.2), mass (arrowheads) shows rim-enhncement in arterial phase. G. On 5-minute delayed fat-saturation T1-weighted MR image, mass (arrowheads) shows diffuse enhancement. Note that central portion of mass is enhanced in delayed phase, in comparison with arterial phase image. H. After steroid therapy for three months, sagittal T2-weighted MR image (TR/TE, 4550/107) reveals marked interval decrease in size of urethral mass (arrows). I. Transvaginal ultrasonography image in 72-year-old woman with IgG4-related inflammatory pseudotumor in urethra. transvaginal ultrasonography scan in color Doppler mode reveals heterogeneously low echoic mass (arrows) encasing urethra. Note that vascularity in urethral mass is poor. J. Microscopic exam shows linear spindle cell (arrowheads) proliferation and lymphocyte (arrow) infiltration (Hematoxylin & Eosin, × 200). K. Immunohistochemical staining reveals positivity (brown color) for marker of proliferated spindle cells and smooth muscle actin, suggesting that specimen is compatible with IPT. L. Immunohistochemical staining for IgG4 demonstrates positivity (brown color) in some inflammatory cells and spindle cells, suggesting that specimen is associated with IgG4-related sclerosing disease. IgG4 = immunoglobulin G4, IPT = inflammatory pseudotumor.

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