Aggressive angiomyxoma—report of three cases

Abstract

Aggressive angiomyxoma, occurring predominantly in adult, fertile female pelvic and perineal structures, is a connective tissue-originating tumour and seems to be hormonally regulated. Many of the tumours are estrogen (ER)-receptor and progesterone (PR)-receptor positive and medical therapy with gonadotropin-releasing hormone agonists (GnRH) showed to be effective in a few cases. Aggressive angiomyxoma has a high tendency of local recurrence, but it usually does not develop metastases. These special features of aggressive angiomyxoma have resulted in different treatment modalities in different centres: some preferring treatments with minimal morbidity and some speaking for aggressive surgical therapy. During the years 2002–2003, we operated on three women with aggressive angiomyxoma. The first patient was a 27-year-old woman who noticed that her belly was unusually large during the 12th week of pregnancy. In a local hospital, a large tumour next to the uterus was verified with ultrasonography. A laparotomy was undertaken and the tumour was biopsied. Histology revealed an aggressive angiomyxoma and the patient was sent to our university hospital. In MRI, a large, jelly-like retroperitoneal tumour dislocating the rectum, sigmoidal colon, vagina, uterus and urinary bladder and extending below the levators near to the skin in the perineal region was seen. The patient was willing to continue her pregnancy. At laparotomy during the 16th week of pregnancy, most part of the tumour weighing 2 kg could be removed. Only some leaf-like structures, which were attached to the levator structures, were left. She overcame the operation well and had an intensive outpatient policlinic follow-up during the rest of her pregnancy. She presented in spontaneous labour at term and gave birth to a healthy male infant with a birth weight of 3,655 g. The Apgar score was 9 and the umbilical artery pH 7.37. Histologic examination from the tumour showed an ER-positive and PR-positive aggressive angiomyxoma. In follow-up MRI, a residual mass was still seen in the pelvic wall extending below the levators to the ischiorectal fossa. In the re-laparotomy, the tumour located diffusely in the posterior wall of the bladder, in the anterior wall of the uterus and in the lower pelvis. The operation was macroscopically radical but uncertain because of the diffuse growth pattern of the tumour. The patient recovered well. In follow-up MRI 6 months after the operation, a minor 6×2 cm residual tumour was seen in the low lateral pelvic wall extending to the ischiorectal fossa and 1 year after the operation, the residual tumour showed marginal growth. The patient was symptomless. A further follow-up MRI displayed no change in the residual tumour 1 year later. The second patient was a 47-year-old woman who was operated on because of a local breast cancer. She received radiochemotherapy post-operatively, but no anti-estrogenic Int J Colorectal Dis (2007) 22:1545–1546 DOI 10.1007/s00384-006-0263-x