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Aggressive angiomyxoma, an introspect of an enigmatically notorious cancer

Aggressive Angiomyxoma (AAM) is a rare locally invasive mesenchymal tumor that most commonly arises in the vulvovaginal region, perineum and pelvis of women. We present here 2 rare cases of AAM with varying presentations one arising from pelvis and other from the vulva. A 45-year-old lady referred from a private hospital with provisional diagnosis of cervical fibroid based on her MRI findings. On examination, a large mass was felt in the vagina which was firm, smooth, nontender and immobile. CT scan revealed a large (13x12x10 cms) mass displacing bowel, uterus, ureters, causing, hydroureteronephrosis and bilateral renal metastasis. Her chest x-ray too revealed probable metastasis. Biopsy of the mass was performed which confirmed diagnosis of Angiomyxoma and Immunohistochemistry further confirmed it. She opted for neoadjuvant GnRH therapy. Unfortunately, the patient expired soon after initiating the therapy. The second patient, 25-year-old, presented with mass growing on right side of vulva having increased massively in size during her recent pregnancy, now measuring 10x8 cms with a pedicle of 10x2 cms. Histopathology and immunohistochemistry confirmed AAM for which wide local excision was done, no evidence of recurrence noted till date. AAM has a very high risk for local relapse even though metastasis is very rare. Hence, we need to differentiate it from other mesenchymal tumors occurring in this region. Complete resection with negative margin is the mainstay of treatment. Since incidence of recurrence is high, follow-up is advised as long as 15 years.


Dr. Sarah Fatima Siddiqui completed her graduation (MBBS) from the prestigious Lady Hardinge Medical College and is currently pursuing her postgraduation (MS) in Obstetrics & Gynecology from the highly reputed Maulana Azad Medical College, New Delhi, India.


Sarah Fatima Siddiqui

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