Aggressive angiomyxoma (AAM) is a distinctive neoplasm seen in the female genital tract. We present the clinicopathological findings of 12 female patients with AAM. Immunohistochemical analysis for vimentin, desmin, smooth-muscle actin (SMA), muscle-specific actin (MSA), S-100, CD44, estrogen receptor (ER), and progesterone receptor (PR) was performed. Mean patient age was 39 years (range 20-77 years). Eight tumors arose in the vulva, two in the suburethral area, and two in the perirectal area. Three were pedunculated (two vulvar and one suburethral). Perineal tumors were locally excised, with limited removal of adjacent tissue or tissue surrounding the pedicle base of pedunculated tumors. Perirectal tumors were removed by wide excision. Tumors ranged 2.8-40.0 cm in size. Eleven patients were followed-up (mean 19 months). Recurrence occurred in one patient 48 months after tumor resection from perirectum and abdomen. Immunohistochemistry showed tumor positivity for vimentin (11/11), desmin (8/11), CD44 (8/11), ER (10/12), PR (11/12), and SMA (3/11). MSA and S-100 were negative. In summary, AAM in the perineum especially pedunculated tumors may possibly require only local excision for definitive treatment. Furthermore, the majority of AAM have CD44 expression.

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Aggressive angiomyxoma (AAM) is a distinctive neoplasm seen in the female genital tract. We present the clinicopathological findings of 12 female patients with AAM. Immunohistochemical analysis for vimentin, desmin, smooth-muscle actin (SMA), muscle-specific actin (MSA), S-100, CD44, estrogen receptor (ER), and progesterone receptor (PR) was performed. Mean patient age was 39 years (range 20–77 years). Eight tumors arose in the vulva, two in the suburethral area, and two in the perirectal area. Three were pedunculated (two vulvar and one suburethral). Perineal tumors were locally excised, with limited removal of adjacent tissue or tissue surrounding the pedicle base of pedunculated tumors. Perirectal tumors were removed by wide excision. Tumors ranged 2.8–40.0 cm in size. Eleven patients were followed-up (mean 19 months). Recurrence occurred in one patient 48 months after tumor resection from perirectum and abdomen. Immunohistochemistry showed tumor positivity for vimentin (11/11), desmin (8/11), CD44 (8/11), ER (10/12), PR (11/12), and SMA (3/11). MSA and S-100 were negative. In summary, AAM in the perineum especially pedunculated tumors may possibly require only local excision for definitive treatment. Furthermore, the majority of AAM have CD44 expression.