Difference between revisions of "Fibromatoses"

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'''Fibromatoses''' are a group of benign stromal lesions that may be seen in [[pediatric pathology]] and may or may not be associated with [[syndromes]].
[[Image:Palmar fibromatosis - alt -- high mag.jpg|thumb|right|Micrograph showing a [[palmar fibromatosis]]. [[H&E stain]].]]
'''Fibromatoses''' are a group of benign stromal lesions that may or may not be associated with [[syndromes]].
 
''Plantar fibromatosis'' redirects here.


==Syndromes associated with fibromatoses==
==Syndromes associated with fibromatoses==
*[[Familial adenomatous polyposis]].<ref>{{Ref PBoD8|1092}}</ref>
*[[Familial adenomatous polyposis]].<ref name=Ref_PBoD8_1092>{{Ref PBoD8|1092}}</ref>
**[[Gardner syndrome]].
**[[Gardner syndrome]].
***[[Desmoid-type fibromatosis]].
*Hereditary desmoid syndrome.
*Hereditary desmoid syndrome.
==Types==
===Superficial===
*Palmar ([[Dupuytren's contracture]]).
*Plantar (Ledderhose disease<ref>{{cite journal |authors=Mozena JD, Hansen EK, Jones PC |title=Radiotherapy for Plantar Fibromas (Ledderhose Disease) |journal=J Am Podiatr Med Assoc |volume=112 |issue=1 |pages= |date=March 2022 |pmid=35324461 |doi=10.7547/19-008 |url=}}</ref><ref name=pmid35274715>{{cite journal |authors=Schoenfeld JD, Agaram NP, Lefkowitz RA, Kelly CM, Healey JH, Gounder MM |title=Sorafenib in Dupuytren and Ledderhose Disease |journal=Oncologist |volume=27 |issue=3 |pages=e294–e296 |date=March 2022 |pmid=35274715 |pmc=8914480 |doi=10.1093/oncolo/oyab050 |url=}}</ref>).
*Penile ([[Peyronie's disease]]).
===Deep===
*[[Desmoid-type fibromatosis]].
*Others.
==Gross==
*Firm.
*Infiltrative borders.
Image:
*[http://www.flickr.com/photos/kcrazypathgirl/5858963062/ Desmoid tumour (flickr.com)].
*[http://www.flickr.com/photos/lunarcaustic/5334047938/ Mesenteric fibromatosis (flickr.com)].


==Microscopic==
==Microscopic==
Features:
Features:
*Bland spindle cells.
*Bland spindle cells - typically in fascicles.
**Pale eosinophilic cytoplasm.<ref>URL: [http://www.histopathology-india.net/Fibromatosis.htm http://www.histopathology-india.net/Fibromatosis.htm]. Accessed on: 18 September 2012.</ref>
**Nucleus may be ovoid.<ref name=pmid19926768/>
*Small thin blood vessels that are parallel to one another.
*Small thin blood vessels that are parallel to one another.
**Nuclei of [[blood vessel]]s are typically darker staining than those of the lesion.
DDx:
*Metaplastic carcinoma, e.g. [[metaplastic breast carcinoma]].
*[[Nodular fasciitis]].
*[[Dermatofibroma]].
*[[GIST]].
*[[Desmoplastic fibroblastoma]] - esp. shoulder region.
Images:
*[http://www.sarcomaimages.com/index.php?v=Plantar-Fibromatosis Plantar fibromatosis (sarcomaimages.com)].
*[http://www.flickr.com/photos/lunarcaustic/5333412517/in/photostream/ Mesenteric fibromatosis (flickr.com)].
*[http://radiographics.rsna.org/content/29/7/2143/F12.expansion.html Plantar fibromatosis (rsna.org)].<ref name=pmid19926768/>
*[http://radiographics.rsna.org/content/29/7/2143/F17.expansion.html Plantar fibromatosis (rsna.org)].<ref name=pmid19926768>{{Cite journal  | last1 = Murphey | first1 = MD. | last2 = Ruble | first2 = CM. | last3 = Tyszko | first3 = SM. | last4 = Zbojniewicz | first4 = AM. | last5 = Potter | first5 = BK. | last6 = Miettinen | first6 = M. | title = From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. | journal = Radiographics | volume = 29 | issue = 7 | pages = 2143-73 | month = Nov | year = 2009 | doi = 10.1148/rg.297095138 | PMID = 19926768 }}</ref>
==IHC==
*Beta-catenin +ve -- nuclear stain.
*CD117 -ve.
*CD34 -ve.<ref name=pmid25349618>{{Cite journal  | last1 = Li Destri | first1 = G. | last2 = Ferraro | first2 = MJ. | last3 = Calabrini | first3 = M. | last4 = Pennisi | first4 = M. | last5 = Magro | first5 = G. | title = Desmoid-type fibromatosis of the mesentery: report of a sporadic case with emphasis on differential diagnostic problems. | journal = Case Rep Med | volume = 2014 | issue =  | pages = 850180 | month =  | year = 2014 | doi = 10.1155/2014/850180 | PMID = 25349618 }}</ref>
Image:
*[http://pathinfo.wikia.com/wiki/File:Beta-catenin.fibromatosis.1.jpg Fibromatosis (wikia.com)].
==Sign out==
===Deep===
<pre>
SOFT TISSUE LESION, LEFT SHOULDER, CORE BIOPSY:
- FIBROMATOSIS, SEE COMMENT.
COMMENT:
The lesion was evaluated with immunostains:
Positive: vimentin, SMA (rare), beta-catenin.
Negative: pankeratin, S-100, desmin, CD34 (stains vessels).
</pre>
===Plantar===
<pre>
Left Foot, Plantar Fascia, Fasciectomy:
- Plantar fibromatosis.
</pre>
===Micro===
The sections show a lesion with bland spindle cells. There is no apparent nuclear atypia.  Mitotic figures are not identified.  No myxoid areas are apparent.  No necrosis is identified.


==See also==
==See also==
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[[Category:Weird stuff]]
[[Category:Weird stuff]]
[[Category:Soft tissue pathology]]

Latest revision as of 17:29, 24 April 2023

Micrograph showing a palmar fibromatosis. H&E stain.

Fibromatoses are a group of benign stromal lesions that may or may not be associated with syndromes.

Plantar fibromatosis redirects here.

Syndromes associated with fibromatoses

Types

Superficial

Deep

Gross

  • Firm.
  • Infiltrative borders.

Image:

Microscopic

Features:

  • Bland spindle cells - typically in fascicles.
    • Pale eosinophilic cytoplasm.[4]
    • Nucleus may be ovoid.[5]
  • Small thin blood vessels that are parallel to one another.
    • Nuclei of blood vessels are typically darker staining than those of the lesion.

DDx:

Images:

IHC

  • Beta-catenin +ve -- nuclear stain.
  • CD117 -ve.
  • CD34 -ve.[6]

Image:

Sign out

Deep

SOFT TISSUE LESION, LEFT SHOULDER, CORE BIOPSY:
- FIBROMATOSIS, SEE COMMENT.

COMMENT:
The lesion was evaluated with immunostains:
Positive: vimentin, SMA (rare), beta-catenin.
Negative: pankeratin, S-100, desmin, CD34 (stains vessels).

Plantar

Left Foot, Plantar Fascia, Fasciectomy:
	- Plantar fibromatosis.

Micro

The sections show a lesion with bland spindle cells. There is no apparent nuclear atypia. Mitotic figures are not identified. No myxoid areas are apparent. No necrosis is identified.

See also

References

  1. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1092. ISBN 978-1416031215.
  2. ↑ Mozena JD, Hansen EK, Jones PC (March 2022). "Radiotherapy for Plantar Fibromas (Ledderhose Disease)". J Am Podiatr Med Assoc 112 (1). doi:10.7547/19-008. PMID 35324461.
  3. ↑ Schoenfeld JD, Agaram NP, Lefkowitz RA, Kelly CM, Healey JH, Gounder MM (March 2022). "Sorafenib in Dupuytren and Ledderhose Disease". Oncologist 27 (3): e294–e296. doi:10.1093/oncolo/oyab050. PMC 8914480. PMID 35274715. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8914480/.
  4. ↑ URL: http://www.histopathology-india.net/Fibromatosis.htm. Accessed on: 18 September 2012.
  5. ↑ 5.0 5.1 5.2 Murphey, MD.; Ruble, CM.; Tyszko, SM.; Zbojniewicz, AM.; Potter, BK.; Miettinen, M. (Nov 2009). "From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation.". Radiographics 29 (7): 2143-73. doi:10.1148/rg.297095138. PMID 19926768.
  6. ↑ Li Destri, G.; Ferraro, MJ.; Calabrini, M.; Pennisi, M.; Magro, G. (2014). "Desmoid-type fibromatosis of the mesentery: report of a sporadic case with emphasis on differential diagnostic problems.". Case Rep Med 2014: 850180. doi:10.1155/2014/850180. PMID 25349618.

External links

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