Individualized managing strategies of aggressive angiomyxoma of female genital tract and pelvis

Abstract

Aims: To investigate and evaluate the clinical management strategies of aggressive angiomyxoma (AA) in female genital tract and pelvis.

Methods: A cohort of 13 patients with AA diagnosed and treated in Peking Union Medical College Hospital in the last 12 years was reported focusing on the results of the managements and prognosis.

Results: The mean age at initial presentation was 36.9 years. The commonest site of tumor was perineum. Only two cases were accurately diagnosed as AA preoperatively by biopsy and fine needle aspiration of the tumors respectively. MRI helpfully reveals the location, relationship and degree of infiltration between tumors and pelvic organs. Surgery is the mainstay treatment. 11 of 12 patients had complete resection and majority of the operations were finished successfully through trans-perineum and trans-vagina approaches. Three cases with positive expression of ERs and PRs in the tumors received GnRHa injections which were useful preoperatively but not postoperatively. One repeatedly-recurrent case was treated with radiotherapy effectively. The recurrence rate in our study was 41.7% (5/12), with a median recurrence interval of 20.9 months. No patient developed distant metastases and died of the disease.

Conclusions: AA preferentially involves the pelvic and perineal regions of women in reproductive age. Tumor biopsy and fine-needle aspiration cytology are conducive to the preoperative diagnosis. The individualized operative strategy and awareness to protect and rebuild structure and function of the organs should be emphasized during the management of AA. Long-term follow-up is mandatory because of the high rate of recurrence.

Keywords: AA; Aggressive angiomyxoma; Female; Genital tract; Pelvis; Soft tissue tumor.