vulvovaginal aggressive angiomyxoma
Tuesday 7 September 2010
JRC:15102 : Vulvar angiomyxoma, angiomyofibroblastoma variant.
Definition: Aggressive angiomyxoma is a rare mesenchymal neoplasm, which almost always occurs in the vulvovaginal region and which exhibits a marked tendency for local recurrence after excision.
A wide range of other mesenchymal lesions occur in this region, which potentially mimic aggressive angiomyxoma to a variable extent.
The HMGA2 gene has been overexpressed in a significant percentage of aggressive angiomyxomas.
HMGA2 is positive in most aggressive angiomyxomas and is useful in diagnosis as most mesenchymal lesions which closely mimic this are negative.
However, caution should be exercised as some other vulvovaginal mesenchymal lesions, especially, but not exclusively leiomyomas can be HMGA2-positive.
As well as being of value in primary diagnosis, HMGA2 is useful in evaluating margins and in reexcision specimens of aggressive angiomyxoma in identifying foci of residual or recurrent tumor.
HMGA2 is a sensitive but not specific immunohistochemical marker of vulvovaginal aggressive angiomyxoma. McCluggage WG, Connolly L, McBride HA. Am J Surg Pathol. 2010 Jul;34(7):1037-42. PMID: 20551826