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General
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Epidemiology
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Clinical features
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Case reports
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Treatment
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Clinical images
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Site distribution

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Calf, courtesy of Dr. Mark R. Wick

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Paraspinous subcutaneous mass

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Various images

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CT scan, right chest wall, courtesy of Dr. Mark R. Wick

Gross description
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Gross images
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Courtesy of Dr. Mark R. Wick

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Extra-abdominal fibromatosis

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Extraabdominal fibromastosis: thoracic tumor

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Firm, white lobulated fusiform mass

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Operative specimen

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Intraabdominal fibromatosis: gray-glossy cut surface

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Mass in mesentery of small bowel

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Well-defined margins, lesion with infiltrative margins

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Tumor infiltrates thoracic wall

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Trunk tumor

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Thigh tumor

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Gray-white tumor of Gardner syndrome

Micro description
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Micro images
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Images courtesy of Dr. Mark R. Wick:

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Beta cantenin



Extra-abdominal fibromatosis - AFIP:

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Low, paucicellular fibrous proliferation in long fascicles, numerous slit-like vessels

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Fibroblasts, spindled, dense, wavy nuclei and minimal cytoplasm

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Margin: infiltration of skeletal muscle

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Atrophic and regenerating muscle fibers, edge of the lesion, may resemble rhabdomyoblasts

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Some tumors are myxoid

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mMyxoid tumors are uniformly bland, unlike those in malignant myxoid tumors

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Cellular tumor, scattered mitotic figures, none atypical

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Keloid-type pattern, observed focally in deep tumors



Extra-abdominal fibromatosis - other sources:

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Paraspinal tumor

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Thigh mass

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Predominantly cellular with relatively less fibrosis

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Predominantly fibrotic with less cellularity

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Breast tumor, smooth muscle actin+



Intra-abdominal (mesenteric) fibromatosis - AFIP:

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Extrinsic to bowel muscularis propria, which distinguishes it from GIST

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Paucicellular and composed of relatively uniform, bland spindle cells

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Bland, mitotically active, uniform cells in myxoid stroma

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Less commonly bland spindle cells are within collagen bundles

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Pelvic tumor

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Trichrome stain highlights collagen in the fibromatosis, distinguishes it from overlying normal muscularis propria



Intra-abdominal fibromatosis - other sources:

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Uniform spindle cells with moderate collagen



Abdominal fibromatosis:

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Extension into adjacent muscle

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Fascicles of fibroblastic spindle cells

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Immunostains

Cytology description
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Cytology images
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FNA, coherent clusters of uniform spindle cells

Positive stains
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Negative stains
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Electron microscopy description
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Molecular / cytogenetics description
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Molecular / cytogenetics images
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Trisomy 20 and 8

Differential diagnosis
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End of Soft Tissue > Fibroblastic / myofibroblastic tumors > Fibromatosis - deep (desmoid type)



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