Difference between revisions of "Desmoid-type fibromatosis"

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DDx:
DDx:
*[[Hypertrophic scar]]-like lesion, see ''[[dermal scar]]''.
*[[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>
*[[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>
- 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/>
*[[Retroperitoneal fibrosis]] - no beta-catenin staining.<ref name=pmid23020601/>
*Other [[fibromatoses]].
*Other [[fibromatoses]].

Revision as of 18:35, 9 November 2013

Desmoid-type fibromatosis
Diagnosis in short

Desmoid-type fibromatosis. H&E stain.
LM "sweeping fascicles"/bundles, spindle cells with small slender nuclei, solid dark eosinophilic cytoplasm, +/-mitoses, long thin-walled vessels - parallel to one another
LM DDx hypertrophic scar, gastrointestinal stromal tumour, retroperitoneal fibrosis, other fibromatoses,nodular fasciitis
IHC beta-catenin +ve (nuclear), SMA +ve/-ve, CD117 -ve
Site soft tissue
Syndromes familial adenomatous polyposis - esp. Gardner syndrome
Prevalence uncommon
Prognosis benign but locally aggressive
Clin. DDx trauma/hematoma

Desmoid-type fibromatosis is a benign soft tissue lesion in the fibroblastic/myofibroblastic group of tumours.

It is also known as desmoid tumour and desmoid fibromatosis.

General

Gross

Features:[2]

  • 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:[2][3]

  • "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:

Images

  • Desmoid tumour. (WC)

www:

IHC

Features:[2]

  • Beta-catenin +ve (nuclear[4]) - important.
  • SMA +ve ~50% of lesions.

Others:

  • CD117 -ve.

Sign out

LESION, ABDOMINAL WALL, BIOPSY:
- DESMOID-TYPE FIBROMATOSIS.

COMMENT:
The tumour stains strongly with beta-catenin and weakly with SMA. It is negative for CD117.

See also

References

  1. ↑ URL: http://www.dtrf.org/dtrf_aboutdesmoids.htm. Accessed on: 15 April 2011.
  2. ↑ 2.0 2.1 2.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 609. ISBN 978-0781765275.
  3. ↑ URL: http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196. Accessed on: 4 October 2011.
  4. ↑ 4.0 4.1 4.2 Huss, S.; Nehles, J.; Binot, E.; Wardelmann, E.; Mittler, J.; Kleine, MA.; Künstlinger, H.; Hartmann, W. et al. (Jan 2013). "β-catenin (CTNNB1) mutations and clinicopathological features of mesenteric desmoid-type fibromatosis.". Histopathology 62 (2): 294-304. doi:10.1111/j.1365-2559.2012.04355.x. PMID 23020601.
  5. ↑ Thalheimer, A.; Meyer, D.; Gattenlöhner, S.; Timmermann, W.; Thiede, A. (Jul 2004). "[Gastrointestinal stromal tumor of the abdominal wall. An unusual localization of a rare tumor].". Chirurg 75 (7): 708-12. doi:10.1007/s00104-003-0696-5. PMID 15257404.
  6. ↑ URL: http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html. Accessed on: 4 October 2011.
  7. ↑ Ma, JH.; Ma, ZH.; Dong, XF.; Yin, H.; Zhao, YF. (Jun 2013). "Abdominal wall desmoid tumors: A case report.". Oncol Lett 5 (6): 1976-1978. doi:10.3892/ol.2013.1297. PMID 23833679.
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