Difference between revisions of "Desmoid-type fibromatosis"

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Desmoid-type fibromatosis (view source)

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#redirect [[Fibroblastic/myofibroblastic tumours#Desmoid-type_fibromatosis]]
'''Desmoid-type fibromatosis''' is a benign [[soft tissue lesion]] in the [[fibroblastic/myofibroblastic tumours|fibroblastic/myofibroblastic group of tumours]].


It is also known as '''desmoid tumour''' and '''desmoid fibromatosis'''.
==General==
*Benign.
*One of many ''[[fibromatoses]]''.
*Locally aggressive.<ref>URL: [http://www.dtrf.org/dtrf_aboutdesmoids.htm http://www.dtrf.org/dtrf_aboutdesmoids.htm]. Accessed on: 15 April 2011.</ref>
*May be seen in the context of [[familial adenomatous polyposis]].
==Gross==
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Location:
**Abdominal wall, proximal extremities - classic for adolescents and women.
**Head and neck - classic for children.
*Circumscribed mass.
*May be quite large (>10 cm).
==Microscopic==
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref>URL: [http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196 http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196]. Accessed on: 4 October 2011.</ref>
*"Sweeping fascicles"/bundles.
*Spindle cells with:
**Small slender nuclei.
**Solid dark eosinophilic cytoplasm.
*+/-Mitoses - may be abundant.
*Long thin-walled vessels - parallel to one another - '''important feature'''.
DDx:
*[[Hypertrophic scar]]-like lesion, see ''[[dermal scar]]''.
*[[Gastrointestinal stromal tumour]]<ref name=pmid23020601>{{Cite journal  | last1 = Huss | first1 = S. | last2 = Nehles | first2 = J. | last3 = Binot | first3 = E. | last4 = Wardelmann | first4 = E. | last5 = Mittler | first5 = J. | last6 = Kleine | first6 = MA. | last7 = Künstlinger | first7 = H. | last8 = Hartmann | first8 = W. | last9 = Hohenberger | first9 = P. | title = β-catenin (CTNNB1) mutations and clinicopathological features of mesenteric desmoid-type fibromatosis. | journal = Histopathology | volume = 62 | issue = 2 | pages = 294-304 | month = Jan | year = 2013 | doi = 10.1111/j.1365-2559.2012.04355.x | PMID = 23020601 }}</ref>
- reported in abdominal wall.<ref name=pmid15257404>{{Cite journal  | last1 = Thalheimer | first1 = A. | last2 = Meyer | first2 = D. | last3 = Gattenlöhner | first3 = S. | last4 = Timmermann | first4 = W. | last5 = Thiede | first5 = A. | title = [Gastrointestinal stromal tumor of the abdominal wall. An unusual localization of a rare tumor]. | journal = Chirurg | volume = 75 | issue = 7 | pages = 708-12 | month = Jul | year = 2004 | doi = 10.1007/s00104-003-0696-5 | PMID = 15257404 }}</ref>
*[[Retroperitoneal fibrosis]] - no beta-catenin staining.<ref name=pmid23020601/>
*Other [[fibromatoses]].
*[[Nodular fasciitis]] - esp. with [[RBC extravasation]].
===Images===
<gallery>
Image:DesmoidFibromatosis.JPG | Desmoid tumour. (WC)
</gallery>
www:
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196 Desmoid tumour (surgicalpathologyatlas.com)].
*[http://www.cheapmedicinechest.com/wp-content/uploads/2010/10/Figure-4.-Desmoid-tumor-with-fibroblastic-proliferation.png Desmoid tumour (cheapmedicinechest.com)].<ref>URL: [http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html]. Accessed on: 4 October 2011.</ref>
*[http://radiographics.rsna.org/content/29/7/2143/F28.expansion.html Desmoid tumour (radiographics.rsna.org)].
*[http://www.ncbi.nlm.nih.gov.qe2a-proxy.mun.ca/pmc/articles/PMC3700980/figure/f3-ol-05-06-1976/ Desmoid-type fibromatosis (nih.gov)].<ref name=pmid23833679>{{Cite journal  | last1 = Ma | first1 = JH. | last2 = Ma | first2 = ZH. | last3 = Dong | first3 = XF. | last4 = Yin | first4 = H. | last5 = Zhao | first5 = YF. | title = Abdominal wall desmoid tumors: A case report. | journal = Oncol Lett | volume = 5 | issue = 6 | pages = 1976-1978 | month = Jun | year = 2013 | doi = 10.3892/ol.2013.1297 | PMID = 23833679 }}</ref>
==IHC==
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Beta-catenin +ve (nuclear<ref name=pmid23020601/>) - '''important'''.
*SMA +ve ~50% of lesions.
Others:
*CD117 -ve.
==Sign out==
<pre>
LESION, ABDOMINAL WALL, BIOPSY:
- DESMOID-TYPE FIBROMATOSIS.
COMMENT:
The tumour stains strongly with beta-catenin and weakly with SMA. It is negative for CD117.
</pre>
==See also==
*[[Fibroblastic/myofibroblastic tumours]].
==References==
{{Reflist|2}}
[[Category:Fibroblastic/myofibroblastic tumours]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
Michael
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