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]].
***[[Desmoid-type fibromatosis]].
*Hereditary desmoid syndrome.
*Hereditary desmoid syndrome.
*[[Gardner 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==
Features:
*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.
**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