Aggressive angiomyxoma

Angiomyofibroblastoma (AMF) is usually separated from aggressive angiomyxoma (AAM) on morphologic grounds, but desmin is usually positive in the perivascular cells of AMF and is not found in AAM. 

Aggressive angiomyxoma (AAM) is a peculiar neoplasm of the pelvic and perineal soft tissues that most commonly affects women. It is composed of loosely arranged, bland spindled to stellate cells embedded in a myxoid matrix punctuated by numerous venule- and capillary-sized blood vessels reminiscent of myxoid liposarcoma (Fig. 4.8). The vascular pattern of AAM lacks the arborizing appearance that is seen in some overtly malignant myxoid tumors. Some morphologic and immunophenotypic features of aggressive AAM also are shared in part by intramuscular myxoma. 

Ockner DM, Sayadi H, Swanson SE, et al. Genital angiomyo-fibroblastoma Comparison with aggressive angiomyxoma and other myxoid neoplasms of skin and soft tissue. Am J Clin Pathol. 1997 107 36-44. 

Fletcher CDM, Tsang WTW, Fisher C, et al. Angiomyofibro-blastoma of the vulva A benign neoplasm distinct from aggressive angiomyxoma. Am J Surg Pathol. 1992 16 373-382. 

Granter SR, Nucci MR, Fletcher CD. Aggressive angiomyxoma Reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology. 1997 30 3-10. 

Recent study of the cytogenetics of aggressive angiomyxoma has identified that gene rearrangements in... 

Most entities, including aggressive angiomyxoma, express vimentin, ER, PR, desmin, actin, and CD34. 

Nucci MR, Weremowicz S, Neskey DM, et al. Chromosomal translocation t(8 12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva. Genes Chromosomes Cancer. 2001 32 172-176. 

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