Objective: This study aimed to report the results of hormonal therapy in the management of a patient with recurrent aggressive angiomyxoma (AAM) and to propose a management strategy for AAM based on (1) the estrogen receptor (ER) and progestin receptor contents of the tumor (2) the extent of disease based on magnetic resonance imaging findings and (3) the patient’s menopausal status.
Materials and Methods: The chart of a patient with multiple pelvic recurrences of AAM managed surgically during a 16-year period followed by hormonal therapy was reviewed, and a literature search of pelvic, vaginal, and vulva AAM was performed.
Results: The patient presented in this report experienced 7 recurrences of AAM managed surgically during a 16-year period. She then was placed on leuprolide acetate for 3 monthly cycles, but the tumor recurred 6 months after the leuprolide acetate was discontinued. The patient was placed back on monthly leuprolide acetate for 5 years and has remained free of disease for more than 2 years after discontinuing the leuprolide acetate. A literature review suggest a role for hormonal therapy in the management of AAM based on the presence of ER/progestin receptor, the extent of the disease, and the menopausal status of the patient. Gonadotropin-releasing hormone analogs have been successfully used in premenopausal women as neoadjuvant therapy before surgery for previously untreated or recurrent disease, as adjuvant therapy after the initial surgical resection or after the resection of recurrent disease, and as the definitive treatment of AAM. Aromatase inhibitors may play a role in the treatment of ER-positive AAM occurring in postmenopausal women.
Conclusions: Aggressive angiomyxoma can be an extremely hormonally sensitive tumor. Hormonal therapy may have a significant role in the treatment of patients with extensive or recurrent AAM that is ER positive. The selection of hormonal agents used for treating AAM can be based on the patient’s menopausal status.
A paradigm for hormonal treatment in the management of primary and recurrent aggressive angiomyxoma based on steroid receptor content, extent of disease based on MRI findings, and menopause status is presented.
Departments of 1Obstetrics, Gynecology and Reproductive Sciences, 2Pathology, and 3Diagnostic Imaging, Yale University School of Medicine, New Haven
Reprint requests to: Peter Edward Schwartz, MD, Department of Obstetrics, Gynecology and Reproductive Sciences, Yale University School of Medicine, 333 Cedar St, New Haven, CT 06520. E-mail: email@example.com
The authors have declared they have no conflicts of interest.