Small bowel (small intestine) - Idiopathic retroperitoneal fibrosis

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Small bowel (small intestine)

Benign tumors and tumor-like conditions

Idiopathic retroperitoneal fibrosis

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 21 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Fibrosis which develops in retroperitoneum, often at aortic bifurcation
● Associated with drug methylsergine, other fibrotic lesions (sclerosis of major bile ducts, Riedel’s thyroiditis, inflammatory pseudotumor of orbit)
● IgG4 often involved in pathogenesis of idiopathic sclerosing lesion; associated with male gender
● Associated with lymphoma, which developed in 15% of patients

Case reports
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● 58 year old man with idiopathic sclerosing mesenteritis and retroperitoneal fibrosis (Korean J Gastroenterol 2011;58:221)
● With previous sclerosing mesenteritis and small bowel obstruction (Eur J Gastroenterol Hepatol 2006;18:1285)

Gross description
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● Not well circumscribed

Micro description
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● Widely scattered germinal centers and plasma cells in background of dense fibrosis
● Eosinophilic infiltration and oblitertaive phlebitis in IgG4 related cases (Am J Surg Pathol 2009;33:1833)

End of Small bowel (small intestine) > Benign tumors and tumor-like conditions > Idiopathic retroperitoneal fibrosis

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