Abstract
Background: Aggressive angiomyxoma is a rare soft tissue mesenchymal tumor, locally infiltrative with a tendency to repeated local recurrence. The first description was in 1983. Normally it occurs in the vulvovaginal region, perineum and pelvis of females in reproductive age. Local wide excision of the tumor is the primary management. The local recurrence rate is high. The expression of estrogen and progesterone receptors in aggressive angiomyxoma suggests a hormone dependency of the tumor Case report: A 50-year-old woman reported first in 2013 with a painless swelling at the perineum. She underwent surgical excision. Pathologic findings reported the tumor as an aggressive angiomyxoma. The patient did not appear to the follow-up examinations. In 2016 she reported again with a painless swelling at the perineum and underwent a local wide resection. The perineal body had to be reconstructed because of the deep infiltration of the tumor. Conclusion: Aggressive angiomyxoma is a rare disease, but when treating women with a painless swelling in the vulvovaginal region, perineum or pelvis aggressive angiomyxoma should be considered as a differential diagnosis. There is no standardised therapy described, but complete resection seems to be important. Descriptions of pharmacologic treatment exist with gonadotropinreleasing hormone agonists.
Highlights
Aggressive angiomyxoma is a very rare soft tissue mesenchymal neoplasm [1], locally infiltrative with a tendency to repeated local recurrence
The first description was in 1983 [2]. It normally occurs in the vulvovaginal region, perineum and pelvis of females in reproductive age
This suggests that aggressive angiomyxoma is a local disease
Summary
Aggressive angiomyxoma is a very rare soft tissue mesenchymal neoplasm (only about 350 cases described in literature) [1], locally infiltrative with a tendency to repeated local recurrence. It normally occurs in the vulvovaginal region, perineum and pelvis of females in reproductive age It grows slowly as a painless solid mass with unclear boarders and the aggressive angiomyxoma often infiltrates the deep tissues. There are rare cases of aggressive angiomyxoma in the inguinoscrotal region of males and usually appear in older age [6]. This difference between males and females cannot be explained [7]. Descriptions of drug treatment exist with gonadotropin-releasing hormone (GnRH) agonists in premenopausal women [14,15,16,17] This locally aggressive tumor is a rare disease, the state of knowledge how to treat patients with this soft tissue mesenchymal neoplasm is low.
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