Small intestine & ampulla
Benign tumors / tumor-like conditions
Idiopathic retroperitoneal fibrosis
Author: Hanni Gulwani, M.B.B.S.
Last author update: 1 August 2012
Last staff update: 19 November 2020
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed Search: Idiopathic retroperitoneal fibrosis small bowel
Table of Contents
Definition / general | Case reports | Gross description | Microscopic (histologic) descriptionCite this page: Gulwani H. Idiopathic retroperitoneal fibrosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/smallbowelIRF.html. Accessed December 18th, 2024.
Definition / general
- Fibrosis which develops in retroperitoneum, often at aortic bifurcation
- Associated with drug methylsergine, other fibrotic lesions (sclerosis of major bile ducts, Riedel 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
- 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
- Not well circumscribed
Microscopic (histologic) description
- 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)
