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Case Reports
. 2024 Jun 8;16(6):e61968.
doi: 10.7759/cureus.61968. eCollection 2024 Jun.

A Rare Case of Retroperitoneal Tumefactive Fibroinflammatory Lesion Related to IgG4-Sclerosing Disease

Spyridon Nikas  1 , Sotiria Zonitsa  1 , Pavlos Anastasiadis  1 , Kalliopi Veniadou  1 , Sofia Roumelioti  1 , Angelos C Mitsas  2 , Georgios Gerasopoulos  2 , Panagiotis Gkouvis  1
Affiliations
Case Reports

A Rare Case of Retroperitoneal Tumefactive Fibroinflammatory Lesion Related to IgG4-Sclerosing Disease

Spyridon Nikas et al. Cureus. .

Abstract

We present a unique case of a retroperitoneal tumefactive fibroinflammatory lesion related to IgG4-sclerosing disease; it is a rare manifestation of the IgG4-related disease, which usually causes diffuse fibrosis when located in the retroperitoneum, rather than mass-like lesions. A 49-year-old man presented to the emergency department complaining of abdominal pain and vomiting. Subsequent testing with abdominal ultrasound, CT, and MRI revealed a large retroperitoneal mass of unknown origin, heterogenous, with a concentric circles pattern best visualized in MRI. The lesion was resected, and the histological and immunohistochemical studies revealed an IgG4-related tumefactive fibroinflammatory lesion of the retroperitoneum.

Keywords: fibroinflammatory tumor; igg4-related disease; retroperitoneal mass; retroperitoneum; tumefactive lesions.

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Conflict of interest statement

Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Ultrasound appearance of the lesion
Note the gross calcifications and the heterogeneity of the lesion. The deep borders of the mass are indiscernible
Figure 2
Figure 2. Axial CT appearance of the retroperitoneal mass
The red arrow shows the lesion, which displays central foci of calcification, air, and areas of necrosis. The green arrow shows the left psoas muscle infiltration, and the blue arrow shows the strangulation of the left ureter by the lesion in the retroperitoneal space CT: computed tomography
Figure 3
Figure 3. Coronal CT appearance of the retroperitoneal mass
Central rings of calcification, with foci of air, are noted, probably due to direct infiltration of adjacent bowel loops CT: computed tomography
Figure 4
Figure 4. Axial MRI T1-weighted image, with intravenous contrast infusion
Note the heterogeneity, the infiltration of the left psoas muscle, and the peripheral signal enhancement after intravenous contrast infusion MRI: magnetic resonance imaging
Figure 5
Figure 5. Coronal MRI T1-weighted image, with intravenous contrast infusion
Note the peripheral signal enhancement after intravenous contrast infusion MRI: magnetic resonance imaging
Figure 6
Figure 6. Coronal MRI T2-weighted image
MRI: magnetic resonance imaging
Figure 7
Figure 7. Axial MRI T2-weighted image
MRI: magnetic resonance imaging
Figure 8
Figure 8. Coronal MRI T2-weighted image (different level compared to Figure 6)
The concentric circles-like pattern (like “onion layers”) is best appreciated in the T2 images MRI: magnetic resonance imaging
Figure 9
Figure 9. Intraoperative photograph of the lesion
Note the bulging of the mass in the retroperitoneal space, next to the large vessels of the abdomen
See this image and copyright information in PMC

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