Colon tumor
Mesenchymal tumors
Retroperitoneal fibrosis of colon

Author: Raul S. Gonzalez, M.D. (see Authors page)

Revised: 4 January 2017, last major update December 2016

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: retroperitoneal fibrosis [title] colon
Cite this page: Retroperitoneal fibrosis of colon. PathologyOutlines.com website. http://pathologyoutlines.com/topic/colontumorirf.html. Accessed July 26th, 2017.
Definition / general
  • Nonneoplastic fibrosis that develops in retroperitoneum and may encroach upon colon
Essential features
  • Benign fibrosing process of the retroperitoneum that can rarely involve the gastrointestinal tract secondarily
  • Some cases belong to the spectrum of IgG4 related disease
Terminology
  • Also called Ormond's disease, idiopathic retroperitoneal fibrosis
Sites
  • Arises in retroperitoneum, often at aortic bifurcation
  • Gastrointestinal tract involvement is rare
Etiology
  • IgG4 positive plasma cells implicated as a causative factor (Am J Surg Pathol 2009;33:1833); can also be caused by methysergide or lymphoma, or may be idiopathic
Clinical features
Laboratory
  • Increased serum IgG and IgG4 in patients with IgG4 related disease
Case reports
Treatment
Clinical images

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Retroperitoneal mass enveloping aorta

Gross description
  • Poorly circumscribed fibrotic mass
Gross images

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Fibrosis involving ureter

Microscopic (histologic) description
  • Storiform fibrotic process infiltrated by eosinophils and IgG4 positive plasma cells (which may be sparse)
  • May show obliterative phlebitis, fat necrosis
  • Similar findings may be observed in lymph nodes (Am J Clin Pathol 1996;105:430)
Microscopic (histologic) images

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Contributed by Raul S. Gonzalez, M.D.



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Fibrosis with inflammatory cells - reference


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Fibrosis with inflammatory cells - reference

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Fibrosis with inflammatory cells and fat necrosis

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Fibrosis involving ureter

Positive stains
  • IgG4 (in plasma cells)
Differential diagnosis