J Korean Soc Radiol.  2015 Dec;73(6):418-423. 10.3348/jksr.2015.73.6.418.

Multi-Organ Involvement of an Immunoglobulin G4-Related Inflammatory Pseudotumor of the Urogenital Tract: A Case Report

• Chun KY
• Jung YY
• Kim YJ
• Joo JE
• Yoo TK
• You MW
• Choi YS

• KMID: 2130950
• DOI: http://doi.org/10.3348/jksr.2015.73.6.418

Abstract

Inflammatory pseudotumor (IPT) is a rare solid tumor of unknown etiology that can arise in most organs. IPT usually presents as a single, benign lesion. In the urogenital tract, IPT frequently occurs in the bladder, but in rare instances, IPT may originate in the kidney, prostate, or ureter. We describe a highly unusual case of multi-organ IPT that included the periureteral area, paravesical space, and prostate. The diagnosis was confirmed by computed tomography imaging, and by pathology testing that detected prominent immunoglobulin G4-positive plasma cells.

MeSH Terms

Diagnosis
Granuloma, Plasma Cell*
Immunoglobulins*
Kidney
Pathology
Plasma Cells
Prostate
Ureter
Urinary Bladder
Immunoglobulins

Figure

• 

  Fig. 1 A 50-year-old man with multi-organ involvement of IgG4-related inflammatory pseudotumor of the urogenital tract. A. Axial contrast-enhanced CT image showing three ill-defined heterogeneous, delayed enhancing masses in the right paravesical space and bilateral periureteral areas (asterisks). Each bilateral periureteral mass encases a distal ureter, and the left periureteral mass abuts the inferior bladder wall (white arrow). Infiltration of the bilateral obturator muscles is shown (black arrows). B. Coronal reformatted contrast-enhanced CT image showing bilateral periureteral masses (asterisks) and irregular wall thickening and luminal narrowing of the bilateral distal ureters (black arrows) from tumor involvement. C. Axial contrast-enhanced CT image demonstrating bilateral hydroureteronephrosis (arrows). D. Retrograde pyelography image showing right hydronephrosis with double-J catheter insertion in the right ureter, and segmental dilatation and stenosis (arrows) of the left distal ureter. IgG4 = immunoglobulin G4 E. Microscopic photomicrography of the paravesical mass showing spindle cell proliferation with diffuse marked fibrosis, accompanying marked infiltration of plasma cells, lymphocytes, histiocytes, and eosinophils with lymphoid follicles (hematoxylin and eosin stain, × 100). F. A follow-up axial contrast-enhanced CT scan after surgery, radiation therapy, and steroid hormone therapy, showing marked decreases in the size of the bilateral periureteral masses (asterisks). G. The last follow-up axial contrast-enhanced CT image revealing a soft tissue lesion encasing the left proximal ureter (arrow). H. The additional immunohistochemistry stain on high power field microscopy performed after suspicion of IgG4-related IPT, showing a high volume of IgG4-positive plasma cells (arrow). IgG4 = immunoglobulin G4, IPT = inflammatory pseudotumor

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