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Small bowel (small intestine)

Benign tumors and tumor-like conditions

Fibromatosis


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 19 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Uncommon in small bowel, rarely adheres to or penetrates bowel wall
● Usually in mesentery or retroperitoneum
● Also called intraabdominal desmoid tumor

Clinical features
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● Mean age 34 years old (younger than GIST patients)
● May be associated with trauma, familial polyposis coli, Gardnerís syndrome, hormonal stimulation
● Characterized by mutations in beta-catenin gene, CTNNB1 or APC gene (Oncologist 2011;16:682)
● Benign, but may recur

Case reports
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● 73 year old woman with tumor in jejunal pouch (World J Surg Oncol 2006;4:27)

Treatment
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● Surgical excision, radiation therapy, possibly chemotherapy

Gross description
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● Firm, tan, homogenous
● Usually large (6 to 25 cm) with infiltrative margins

Micro description
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● Broad, sweeping fascicles of bland spindle cells with minimal mitotic activity (mean 4 mitoses/50 HPF), bland nuclear features, finely collagenous stroma
● Infiltrative borders, evenly spaced blood vessels
● May involve muscularis propria but no necrosis, no hemorrhage, no myxoid degeneration, no epithelioid cells, no pleomorphism, no foam cells, no inflammatory cells

Micro images
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Other images

Positive stains
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● Vimentin, smooth muscle actin, desmin (50%)

Negative stains
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● CD34, S100, CD117 (some antibodies give CD117+ results)

Electron microscopy description
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● Myofibroblastic/fibroblastic differentiation

Differential diagnosis
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GIST:: CD117+ with all antibodies, often CD34+, often malignant histologic features (Am J Surg Pathol 2000;24:947, Mod Pathol 2003;16:366)

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


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