Aggressive angiomyxoma of the vulva: a case report

Background: Aggressive angiomyxoma is a rare mesenchymal tumor with extensive local invasion. It often presents with a lump in the perineal area and pelvic. Since it often does not involve adjacent organs such as the urethra and anus, does not cause obstructive symptoms. Its incidence is 6 times higher in women than men. This tumor grows slowly and has a slight tendency to metastasize. However, the rate of local recurrence is high. MRI is the most widely used diagnostic method. Ultrasound and CT scan are also used for diagnosis. Due to the rarity of this tumor, it is difficult to diagnose before surgery and pathology assessment. Due to the penetrating nature of the tumor and the lack of a clear capsule, incomplete surgical resection is common. Local recurrence is common even after complete mass resection. Therefore, patients need a long follow-up. The main treatment is surgery but non-surgical interventions such as hormonal therapy, radiotherapy, arterial embolization, etc. have been associated with variable success rates. Case Presentation: A 54-year-old female patient presented with a vulvar mass in February 2021. The patient mentioned that the mass had existed for ten years and had increased in size in the last two years. After discussion on the tumor board, she underwent surgery. The pathology of the mass was reported to be aggressive angiomyxoma. The patient was treated with a GNRH agonist after surgery. The patient is currently under follow-up and has not had a recurrence so far (March 2020). Conclusion: Aggressive angiomyxoma is a rare mesenchymal tumor. It has extensive local invasion and a high recurrence rate, but distant metastasis is rare. Estrogen receptors or Progesterone receptors are commonly positive in aggressive angiomyxoma. The best treatment for aggressive angiomyxoma remains unknown. Extensive local resection of the tumor has been reported as an important therapeutic measure. In cases of mass recurrence, reoperation and hormone therapy have been effective. It is important for gynecologists to consider this tumor as a differential diagnosis when dealing with vulvar masses.