October 2021 · Volume 10 · Issue 10 Page 3979 International Journal of Reproduction, Contraception, Obstetrics and Gynecology Devi TR et al. Int J Reprod Contracept Obstet Gynecol. 2021 Oct;10(10):3979-3982 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789 Case Report Aggressive angiomyxoma T. Ramani Devi*, D. Sangavi, A. Deepika INTRODUCTION Angiomyxoma is a rare benign mesenchymal tumour which is slow-growing, low-grade neoplasm mostly occurring in the vulvovaginal (perineal) region which has a tendency for recurrence and infiltrates into the surrounding skeletal muscle and fat. 1,2 Presenting complaints include dull aching pain, urinary and gastrointestinal symptoms such as dysuria, urinary retention and dyspareunia. Most commonly occurs in the women of reproductive age group and was first described by Steeper and Rosai in 1983. They reported a case series of 9 female patients, who presented with benign-appearing myxoid and vascular tumour that was infiltrative and had a tendency for local recurrence and hence the term aggressive. 3 Recurrence often occurs after many years. 4 Incidence of angiomyxoma based on gender is seen in the ratio of female:male-6.6:1. Fibroblastic or myo-fibroblastic origin seems most likely. 5 WHO classifies aggressive angiomyxoma as a ‘Tumour of uncertain differentiation’. 6 Initially, angiomyxoma was thought to be a tumour with no metastatic potential, but this is no more acceptable, as few case reports of metastasis has been reported. 7 Liver, lungs, larynx, orbit and supraclavicular fossa are other rare sites for angiomyxoma. 8 The reported age of presentation ranges from 1 to 82 years, with a median age of 33 years. 9,10 Literature evidence are quite sparse, almost limited to 250 cases. There is lack of consensus on the clinical presentation, treatment protocols and follow up pattern because of its rarity. We are presenting our case as DOI: https://dx.doi.org/10.18203/2320-1770.ijrcog20213874 Department of Obstetrics and Gynecology, Ramakrishna Medical Centre LLP and Janani Fertility Centre, Trichy, Tamil Nadu, India Received: 28 July 2021 Accepted: 01 September 2021 *Correspondence: Dr. T. Ramani Devi, E-mail: ramanidevidr@yahoo.co.in Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Angiomyxoma is a rare slow-growing, benign low-grade tumor occurring in women of reproductive age group which is known for its recurrence. The symptoms are variable. Mrs. K. aged 33 years, reached our outpatient department (OPD) with complaints of painless swelling on the right labial region which she was feeling uncomfortable while sitting. Mass was found to be mobile with no evidence of inflammatory change. The location of the mass made us think about, Bartholin’s cyst and hence surgical excision of the mass was done. The whole mass was found to be lying below the fat in the right labial region and wide excision was completed. The histopathology of the mass was reported as angiomyxoma. Angiomyxoma arises from the mesenchymal tissue and it is locally invasive with high recurrence rate. It is more common in females. Mostly seen in the vulvovaginal, pelvic cavity and perineum. The lesion can reach huge size. It has to be differentiated from gynecologic malignancies, cyst, abscess and hernia. Histology along with immune- histochemistry can confirm the lesion. Wide excision is the mode of treatment. Incomplete excision can lead to relapse. Relapse can happen in 35-72% of the cases. Local recurrence may occur between 2 months to 15 years following initial diagnosis. Hence, follow up is essential. Angiomyxoma is a very rare condition and only around 250 cases have been reported in the world literature. It has to be differentiated from other benign conditions. Diagnosed by non-invasive techniques like ultrasound sonography (USG), magnetic resonance imaging (MRI) and computed tomography (CT). Wide excision is the treatment of choice. Long term follow up is needed as recurrences are high. Keywords: Angiomyxoma, Bartholin’s cyst, Reproductive age, Wide excision, Follow-up for recurrence