Aggressive angiomyxoma of the female perineum in pregnancy

A 32-year-old Caucasian first gravidae, primiparae in the 32nd gestational week, was transferred by her attending physician because of suspected massive condylomata accuminata of the vulva to plan an elective caesarean section. The structure was detected and had grown during the pregnancy. Her medical history and physical examination were otherwise normal. Physical examination of the vulva showed a soft proliferation of 3 × 4 cm of the commissura posterior (Fig. 1) that was not differentiated from the surrounding tissue. Inspection was therefore untypical for condylomata and the smear for human papilloma virus was negative. Excision of the mass was performed in the 36th gestational week under local anesthesia when the structure had grown further. Histopathology showed an aggressive angiomyxoma (AAM). Nuclear staining for estrogen receptors in immunohistochemistry was positive, and negative for progesterone (Fig. 2). The patient was able to deliver spontaneously. Nine months after delivery there was no sign of a recurrence. Aggressive angiomyxoma of the perineum. Positive nuclear staining for estrogen receptors in immunohistochemistry (negative for progesterone). Aggressive angiomyxoma was first described in nine female patients 1983 by Steeper and Rosai (1). It tends to occur more often in white young to middle-aged women. It is a rare form of a histologically characteristic tumor of the female pelvis and perineum with a locally infiltrative growth pattern. A high tendency for local recurrence has been observed. Invasion through the pelvic diaphragm into the retroperitoneal tissues has been reported (2, 3), but never metastases. To our knowledge this is the third report of an AAM that was detected during pregnancy. Depending on the exact location of the tumor, characteristic differential diagnoses have been made: Bartholin's gland cyst or abscess, labial cyst, polyp, Gartner's duct cyst, pedunculated soft tissue tumors, or perineal herniation (4). Tumors that originated in the vaginal wall presented as cysts or a mass following delivery (5). In this case the initial diagnosis was condylomata accuminata (a comparison that has not been described before). Failure to make a correct preoperative diagnosis is very common. A computed tomography (CT) scan can be useful to mark the extension of the tumor if growth is suspected inside the pelvis. The tumor is typically composed of myxoid stroma with prominent vessels of variable, capillaries and medium-sized arteries with a thick muscle layer, the vessels typically clustered in some areas. The bulk of the tumor is composed of fibroblasts and myofibroblasts. Mitotic figures are absent. As AAM occurs mainly in women of reproductive age and seems to grow considerably during pregnancy there might be a hormonal dependency. Fishman et al. reported a weak estrogen receptor positivity and a negative progesterone receptor activity in the AAM diagnosed of a 37-year-old black woman who was not pregnant at the first diagnosis but with a recurrence during pregnancy (2). Htwe et al. found positive staining for progesterone and negative for estrogen in the histology of a 41-year-old pregnant black woman (5). Wide local excision is the treatment of choice. Recurrences are attributed to incomplete tumor excision. However, because of the proliferation into the adjacent soft tissues the AAM often obscures the normal planes and makes surgical evaluation and resection difficult. If the tumor is extended into the pelvis a laparotomy might be necessary. The recurrence rate is nevertheless high. Long-term follow-up is advisable because recurrences can occur several years after the first diagnosis. Author for correspondence: Christiane A. Wolf Department of Obstetrics Humboldt-University Berlin Charité, Campus Virchow Augustenburger Platz 1 13353 Berlin Germany e-mail: tannawolf@aol.com