Difference between revisions of "Aggressive angiomyxoma"

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#redirect [[Soft_tissue_lesions#Aggressive_angiomyxoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Aggressive_angiomyxoma_-_very_high_mag.jpg
| Width      =
| Caption    = Aggressive angiomyxoma. [[H&E stain]].
| Synonyms  =
| Micro      = thick blood vessels that meld into the surrounding stroma, [[myxoid stroma]], small stellate cells/spindle cells without significant nuclear atypia
| Subtypes  =
| LMDDx      = [[angiomyofibroblastoma]], [[neurofibroma]], [[myxoma]]
| Stains    =
| IHC        = desmin +ve, vimentin +ve, ER +ve, PR +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = poorly circumscribed lesion with a "rubbery" or "gelatinous" consistency
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]] - typically [[vulva]], [[scrotum]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      = mass lesion
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign, locally aggressive
| Other      =
| ClinDDx    = [[Bartholin cyst]], [[angiomyofibroblastoma]]
| Tx        = excision
}}
'''Aggressive angiomyxoma''', also '''deep aggressive angiomyxoma''', is a rare benign lesion, typically of the [[vulva]], with a locally aggressive behaviour.
 
==General==
*Classically a [[vulva]]r mass or (less commonly) a [[scrotum|scrotal]] mass.
**Case report of a thigh lesion.<ref name=pmid18338163>{{Cite journal  | last1 = Heffernan | first1 = EJ. | last2 = Hayes | first2 = MM. | last3 = Alkubaidan | first3 = FO. | last4 = Clarkson | first4 = PW. | last5 = Munk | first5 = PL. | title = Aggressive angiomyxoma of the thigh. | journal = Skeletal Radiol | volume = 37 | issue = 7 | pages = 673-8 | month = Jul | year = 2008 | doi = 10.1007/s00256-008-0465-0 | PMID = 18338163 }}</ref>
*Benign - no metastatic potential.
*"Aggressive" as it has a high recurrance.
 
==Gross==
*Poorly circumscribed.
*"Rubbery" or "gelatinous".
 
Location:
*[[Vulva]] - classic location.
 
Clinical DDx:
*[[Bartholin cyst]].
 
==Microscopic==
Features:<ref name=Ref_WMSP624>{{Ref WMSP|624}}</ref>
#Thick blood vessels that meld into the surrounding stroma - '''key feature'''.
#[[Myxoid stroma]] - '''key feature'''.
#Small stellate cell/spindle cells without significant nuclear atypia.
 
DDx:
*[[Angiomyofibroblastoma]].<ref name=pmid1314521>{{Cite journal  | last1 = Fletcher | first1 = CD. | last2 = Tsang | first2 = WY. | last3 = Fisher | first3 = C. | last4 = Lee | first4 = KC. | last5 = Chan | first5 = JK. | title = Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma. | journal = Am J Surg Pathol | volume = 16 | issue = 4 | pages = 373-82 | month = Apr | year = 1992 | doi =  | PMID = 1314521 }}</ref>
*[[Neurofibroma]].
*[[Myxoma]].
 
===Images===
<gallery>
Image:Aggressive_angiomyxoma_-_intermed_mag.jpg | Aggressive angiomyxoma - intermed. mag. (WC/Nephron)
Image:Aggressive_angiomyxoma_-_high_mag.jpg | Aggressive angiomyxoma - high mag. (WC/Nephron)
Image:Aggressive_angiomyxoma_-_very_high_mag.jpg | Aggressive angiomyxoma - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.webpathology.com/image.asp?n=1&Case=99 Aggressive angiomyxoma - low mag. (webpathology.com)].
*[http://www.webpathology.com/image.asp?case=99&n=2 Aggressive angiomyxoma - high mag. (webpathology.com)].
*[http://path.upmc.edu/cases/case538.html Aggressive angiomyxoma - several images (upmc.edu)].
 
==IHC==
Features:<ref name=Ref_WMSP624>{{Ref WMSP|624}}</ref>
*Desmin +ve.
*Vimentin +ve.
*ER +ve.
*PR +ve.
 
==See also==
[[Soft tissue lesions]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Soft tissue lesions]]

Latest revision as of 00:12, 17 February 2014

Aggressive angiomyxoma
Diagnosis in short

Aggressive angiomyxoma. H&E stain.
LM thick blood vessels that meld into the surrounding stroma, myxoid stroma, small stellate cells/spindle cells without significant nuclear atypia
LM DDx angiomyofibroblastoma, neurofibroma, myxoma
IHC desmin +ve, vimentin +ve, ER +ve, PR +ve
Gross poorly circumscribed lesion with a "rubbery" or "gelatinous" consistency
Site soft tissue - typically vulva, scrotum
Signs mass lesion
Prevalence rare
Prognosis benign, locally aggressive
Clin. DDx Bartholin cyst, angiomyofibroblastoma
Treatment excision

Aggressive angiomyxoma, also deep aggressive angiomyxoma, is a rare benign lesion, typically of the vulva, with a locally aggressive behaviour.

General

  • Classically a vulvar mass or (less commonly) a scrotal mass.
    • Case report of a thigh lesion.[1]
  • Benign - no metastatic potential.
  • "Aggressive" as it has a high recurrance.

Gross

  • Poorly circumscribed.
  • "Rubbery" or "gelatinous".

Location:

  • Vulva - classic location.

Clinical DDx:

Microscopic

Features:[2]

  1. Thick blood vessels that meld into the surrounding stroma - key feature.
  2. Myxoid stroma - key feature.
  3. Small stellate cell/spindle cells without significant nuclear atypia.

DDx:

Images

  • Aggressive angiomyxoma - intermed. mag. (WC/Nephron)

  • Aggressive angiomyxoma - high mag. (WC/Nephron)

  • Aggressive angiomyxoma - very high mag. (WC/Nephron)

www:

IHC

Features:[2]

  • Desmin +ve.
  • Vimentin +ve.
  • ER +ve.
  • PR +ve.

See also

Soft tissue lesions.

References

  1. ↑ Heffernan, EJ.; Hayes, MM.; Alkubaidan, FO.; Clarkson, PW.; Munk, PL. (Jul 2008). "Aggressive angiomyxoma of the thigh.". Skeletal Radiol 37 (7): 673-8. doi:10.1007/s00256-008-0465-0. PMID 18338163.
  2. ↑ 2.0 2.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 624. ISBN 978-0781765275.
  3. ↑ Fletcher, CD.; Tsang, WY.; Fisher, C.; Lee, KC.; Chan, JK. (Apr 1992). "Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma.". Am J Surg Pathol 16 (4): 373-82. PMID 1314521.
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