Aggressive angiomyxoma (AA) is a distinctive, locally aggressive, fibromyxoid tumor of the pelvic and genital soft tissues. AA is of unknown histogenesis but the cytologically bland spindled tumor cells, which surround characteristic variegated blood vessels, show fibroblastic or myofibroblastic features. AA may be related to angiomyofibroblastoma (AMF), another cytologically bland fibromyxoid genital spindle cell tumor with variable myoid differentiation that does not, as a rule, recur.
Synopsis
tumoral cells: wavy and fibrillar, spindled, stellate
polygonal fibroblast-like CD34+ dendritic cells
+/- nuclear and cytoplasmic staining for FXIIIa
Differential diagnosis
angiomyofibroblastoma
Localization
vulva, vagina
hypodermis
larynx (#8868184#)
Cytogenetics
chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva (#11550285#)
12q13-15 rearrangement with HMGA2 abnormal expression
See also
CD34+ primitive fibroblasts
factor XIIIa+ dendritic histiocytes
fibrovascular tumors
fibrohistiocytic tumors
myxoid soft tissue tumors
microvascular unit (tissue responsible for stromal repair and remodeling and angiogenesis)
CD34+ fibroblasts
FXIIIa+ histiocytes (FXIIIa+ dendritic cells or FXIIIa+ dendritic histiocytes)
See also