Simultaneous presentation of vulvar aggressive angiomyxoma and ipsilateral subcutaneous lipoma: a case report

BackgroundAggressive angiomyxoma (AAM) is a rare, locally infiltrative mesenchymal neoplasm of the pelviperineal region that is frequently misdiagnosed owing to its slow growth and nonspecific presentation. Synchronous presentation with a second, unrelated large soft-tissue tumor has not previously been described.CaseWe report a 53-year-old woman presenting with two sizeable, ipsilateral, left-sided masses: a pedunculated 13-cm vulvar lesion and a 30-cm multilobulated thigh mass. Ultrasound depicted the vulvar lesion as cystic; MRI favored AAM in the vulvar region and raised concern for liposarcoma in the thigh; initial biopsies were inconclusive. A multidisciplinary tumor board directed staged surgical excision. Histopathology confirmed vulvar AAM with estrogen receptor, progesterone receptor, and CD34 positivity and desmin and smooth muscle actin negativity, and a benign subcutaneous lipoma in the thigh, with no MDM2 amplification on fluorescence in situ hybridization. The postoperative course was uncomplicated.ConclusionThis unusual concurrent presentation illustrates the diagnostic challenges posed by AAM and underscores the value of advanced imaging, definitive histopathological confirmation, and coordinated multidisciplinary planning. Given the high risk of local recurrence, long-term surveillance remains essential.