Aggressive Angiomyxoma of the Vulva or Perineum: Report of Three Patients

Background: Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumour that chiefly involves the vulvoperineal region of young female patients. Treatment is wide surgical excision. Frequent relapses are common. Cases: We present three patients who each had an aggressive angiomyxoma treated initially for another presumed diagnosis. Two patients required immediate repeat surgical procedures for incomplete tumour excision. The third patient developed a recurrence two years after the initial surgery. In one patient, hormonal therapy was used postoperatively because the evaluation of the tumour margins was uncertain. Harpoon markers were used to aid in tumour localization in another patient prior to resection. Conclusion: Clinicians should consider the diagnosis of aggressive angiomyxoma when a patient presents with an atypical vulvoperineal mass, as an incorrect diagnosis may lead to repeated surgical procedures.