PMC3909864_01
57-year-old woman presenting with IgG4-related peritoneal disease.. A-D.Four selected contrast-enhanced axial CT views of hypogastric and pelvic areas show diffuse infiltration of pelvic organs by enhancing masses which encase distal ileum (white arrows) causing intestinal subocclusion (grey stars). Diffuse small round nodules are also dispersed within peritoneal spaces (black arrows). White stars show uterus, black stars small amount of fluid in Douglas pouch and grey arrows ovaries. IgG4 = immunoglobulin G4. E. Gross anatomy of resected organs including uterus (white star), ovaries and portion of incarcerated ileum (black arrow). Organs are massively infiltrated by numerous irregular whitish nodules mimicking diffuse carcinomatosis (white arrows). F. Gross anatomy of 30 cm of resected ileum terminale. Numerous white nodules (white arrows) infiltrating mesenteric fat tissue. G. Section through ileum (black star) showing pseudotumoral fibrosing tissue (black arrows) infiltrating full thickness of intestinal wall. IgG4 = immunoglobulin G4. H. Photomicrograph (hematoxylin-eosin [H-E] stain; magnification, x 10) of section of fatty peritoneal tissue showing area of dense storiform fibrosis (white arrow) separated by dense inflammatory lymphoplasmacytic infiltrate (black arrows) with germinal center formation (small black arrows). Black star shows normal fat tissue. I. Photomicrographic (H-E stain; magnification, x 40) details of typical storiform fibrosis intermingled with lymphoplasmacytic inflammatory infiltrate. J. Photomicrographic (orcein stain; magnification, x 40) details of obliterative phlebitis. K. Photomicrograph (IgG4 immunostain; magnification, x 40) showing numerous IgG4-positive plasma cells. IgG4 = immunoglobulin G4
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F1
Contrast-enhanced abdominal multidetector-row computed tomography (Fig. 1A-D) revealed a subocclusive intestinal syndrome. Gross anatomy (Fig. 1E, F) showed numerous whitish nodules which extensively covered and invaded the gynecologic structures, the wall of the ileum and the mesenteric fat tissue. On sections, some of these nodules were found to deeply penetrate the entire bowel wall from the serosa to the mucosa (Fig. 1G). Microscopically (Fig. 1 H-K), these nodules showed areas of dense storiform fibrosis separated by areas of dense lymphoplasmacytic inflammatory infiltrate containing numerous normal plasma cells.