‘Aggressive’ angiomyxoma: a distinct clinical entity

Aim: An aggressive angiomyxoma is a mesenchymal tumour arising from connective tissues of the perineum or lower pelvis. They cause very little symptoms despite their large size. Methods: All patients with a diagnosis of aggressive angiomyxoma were studied retrospectively from a prospective database at the Royal Marsden Hospital between January 1990 and July 2001. Results: There were seven female patients with a diagnosis of aggressive angiomyxoma. Sites of involvement included the soft tissues of the perineum (n=4), vulva (n=2) and inguinal region (n=1). The common presenting feature was a mass. The size of the tumour was larger than 10 cm in three patients. Two patients had an intracapsular excision, two had marginal resection and in two patients wide excision was done. Four patients developed recurrent tumour with an interval ranging from 6 months to 97 months. Recurrence was not observed in the two patients after wide excision at intervals of 8 and 13 months. Conclusion: The tumour is aggressive in that it has a propensity for recurrence but usually in the long term. Operation can cure patients with aggressive angiomyxoma, but may result in significant morbidity due to the large size of the tumour at presentation and its frequent occurrence in the lower pelvis and perineum with proximity to genitourinary and anorectal structures. A period of watchful waiting to assess growth rate may be the most appropriate course in most patients. Surgery whenever offered for symptom control should be done with minimal morbidity.