Aggressive angiomyxoma

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

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

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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