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Table of Contents
Definition / general | Essential features | Terminology | Sites | Etiology | Clinical features | Laboratory | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosis | Additional referencesCite this page: Retroperitoneal fibrosis of colon. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/colontumorirf.html. Accessed July 14th, 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
- Patients present with lower back or abdominal pain; bowel obstruction is rare
- IgG4 related cases usually seen in males
- Associated with other IgG4 related diseases, such as sclerosing cholangitis (World J Gastroenterol 2013;19:7661), Riedel's thyroiditis, inflammatory pseudotumor of orbit
- Can cause obstructive uropathy
Laboratory
- Increased serum IgG and IgG4 in patients with IgG4 related disease
Case reports
- 39 year old woman with bowel obstruction (Ann Surg 1972;176:199)
Treatment
- Options include steroids, azathioprine, tamoxifen and surgery (Nephrol Dial Transplant 2006;21:2485)
Clinical images
Images hosted on other servers:
Retroperitoneal mass enveloping aorta
Gross description
- Poorly circumscribed fibrotic mass
Gross images
Images hosted on other servers:
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
Positive stains
- IgG4 (in plasma cells)
Differential diagnosis
- Sclerosing mesenteritis: involves mesentery rather than retroperitoneum; can also be IgG4 related
- Inflammatory myofibroblastic tumor: spindle cells more prominent; may be positive for ALK1
Additional references
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