Deep Aggressive Angiomyxoma of Vulva: A Rare Entity

Background: Deep aggressive angiomyxoma (DAA) is a rare soft tissue neoplasm occurring mainly in young females. Due to its rarity and presentation mainly as a labial cyst, it is often misdiagnosed. It is a low-grade neoplasm with high local recurrence following incomplete excision because of its aggressive infiltrative nature. We report nine cases of vulval DAA, describing the clinical, radiological, histopathological, and immunohistochemical features to raise awareness of this rare entity so as to avoid its misdiagnosis. Materials and Methods: A retrospective observational study was conducted from 2012 to 2023. Patient details were obtained from the hospital database. All the slides and blocks were reviewed. Results: The mean age of presentation was 36 years. All the cases presented as a vulval mass ranging in size from 5 to 26 cm and showed a classic type of morphology. Myoid differentiation was identified in six out of nine cases. Estrogen receptors were positive in all cases, while the progesterone receptor was negative in one case. All the patients were alive on follow-up, with one case showing recurrence after 70 months. Conclusion: DAA should always be kept in mind while dealing with vulval masses. MRI is an important tool for its preoperative diagnosis. Due to the tendency of DAAs for recurrence, complete surgical excision is the treatment of choice. Both estrogen and progesterone receptor status of excised specimens are required, as the administration of a gonadotropin-releasing hormone agonist is helpful. Moreover, further molecular studies on DAA should be conducted for a better understanding of this entity and to develop targeted therapy.