Unmasking a Rare Pelvic Masquerader: Aggressive Vulvovaginal Angiomyxoma With Perineal Herniation - Case Report

Sep 28, 2026

Aggressive angiomyxoma (AAM) is a rare, locally invasive, slow-growing low-grade mesenchymal tumor that commonly affects reproductive-age females and has a high recurrence tendency. Its indolent course and nonspecific imaging often lead to misdiagnosis as more common conditions such as Bartholin cyst, lipoma, or hernia. Accurate preoperative identification is crucial to reduce incomplete resection and recurrence. A 47-year-old female case with perineal herniation showed typical MRI findings and histopathological correlation, remaining recurrence-free 12 months after resection.

LanguageEnglish

CategoryHealthcare

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Reading Time18 min

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Abstract

Introduction

Case Presentation

Imaging and clinical evaluation

Discussion

Review began 11/13/2025 Review ended 11/25/2025 Published 12/01/2025 © Copyright 2025 Kumar et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited. DOI: 10.7759/cureus.98244 Open Access Case Report Unmasking a Rare Pelvic Masquerader: Aggressive Vulvovaginal Angiomyxoma With Perineal Herniation Niranjan Kumar 1 , Pradosh Kumar Sarangi 2, Kondaveeti Nikhileswar 2, Mona Lisa 3, Amiy Arnav 4, Satya Ranjan Patra 1 1. General Surgery, All India Institute of Medical Sciences, Deoghar, Jharkhand, IND 2. Radiodiagnosis, All India Institute of Medical Sciences, Deoghar, Jharkhand, IND 3. Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Deoghar, Jharkhand, IND 4. Surgical Oncology, All India Institute of Medical Sciences, Deoghar, Jharkhand, IND Corresponding author: Pradosh Kumar Sarangi, [drpksarangi@gmail.com](drpksarangi@gmail.com) Abstract Aggressive angiomyxoma (AAM) is a rare, locally invasive, slow-growing low-grade mesenchymal tumor most frequently found in reproductive-age females with a high propensity for recurrence. AAM poses diagnostic challenges due to its indolent course, non-specific imaging appearance, and frequent misdiagnosis as more common entities like Bartholin cyst, lipoma, hernia, leiomyoma, etc. Accurate diagnosis before surgery is crucial to prevent recurrence. We present a case ofAAM in a 47-year-old female with perineal herniation showing typical imaging features, including T2 swirling or laminated pattern, and correlated with histopathology post-surgical resection. The patient remained recurrence-free 12 months post-resection. This case reinforces the pivotal role of imaging, especially MRI, in preoperative diagnosis and surgical planning to minimize the likelihood of incomplete resection and recurrence. Categories: General Surgery, Radiology, Pathology Keywords: aggressive angiomyxoma, histology, mri, pelvic mass, perineal herniation, swirling pattern, vulvovaginal mass Introduction Aggressive angiomyxoma is a rare, benign, slow-growing, locally infiltrative, low-grade mesenchymal tumor commonly seen in reproductive-age women. Steeper and Rosai first described it in 1983 [1] . Although described typically in reproductive-age females, a few cases in the scrotum or spermatic cord in males were reported [2] . Aggressive angiomyxoma often has a large pelvic component located deep in relation to the pelvic diaphragm, which makes it difficult to detect during physical examination and is often mistaken for Bartholin’s cysts, vaginal cysts, lipomas, leiomyoma, or perineal hernias [3] . In all cases, diagnostic certainty can be achieved only by histology [4] . Surgical resection is the mainstay of treatment. The infiltrative nature of the tumor makes it difficult for surgeons to achieve a negative surgical margin, leading to frequent recurrences in up to 35% of patients [5] . Estrogen and progesterone receptor-positive tumors can show treatment response to GnRH agonists both preoperatively and after recurrence [5,6] . We present a case of aggressive vulvovaginal angiomyxoma with perineal herniation with classic imaging features and correlating with histopathological examination. Case Presentation A 47-year-old female presented with a complaint of swelling in the perineal region for 6 years (Figure 1) . The patient was apparently asymptomatic six years back, then she noticed swelling in the posterior aspect of the vagina, which was insidious in onset and gradually progressive. The patient had no history of urinary or bowel complaints. No significant weight loss, pain, itching, or redness was noted. Excision of the swelling was done three years ago. Two months later, she noticed swelling in the anterior region of the vagina. Two years back, it was re-

FAQs

Why is aggressive angiomyxoma difficult to diagnose before surgery?

It follows an indolent course and often has nonspecific imaging features, leading to frequent misdiagnosis as other more common pelvic or vulvovaginal conditions.

What role does MRI play in this case?

MRI helps identify characteristic imaging patterns preoperatively, supporting accurate diagnosis and surgical planning to minimize incomplete resection and recurrence.

What treatment strategy is emphasized for aggressive angiomyxoma?

Surgical resection is the mainstay, but the infiltrative tumor nature can make achieving a negative margin difficult, which is why recurrence risk is significant.

Sep 28, 2026

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