Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30-40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases.

Aggressive angiomyxoma is a rare mesenchymal tumour of the genital tract with a high incidence in women of reproductive age. The aim of our work is to understand which is the best management strategy for this condition, starting from the description of a rare case report up to the performance of a narrative review of the literature.

We report a case of a 46-year-old woman who came to our attention because of the growth of a 10-centimetre pedunculated, non-soft, non-tender mass of the left labium majus. She underwent surgical excision, and the histologic diagnosis was aggressive angiomyxoma. Due to a lack of tumour-free margins, radicalization surgery was carried out after three months. A review of the literature of the last ten years was performed following the PRISMA statement on MEDLINE (PubMed). We obtained data from twenty-five studies describing thirty-three cases.

Aggressive angiomyxoma is characterized by a high recurrence rate of between 36 and 72% after surgery. There is no universal consensus about hormonal therapy, and most studies (85%) describe surgical excision followed by only clinical and radiological follow-up.

Wide surgical excision is the gold-standard treatment for aggressive angiomyxoma, succeeded by either clinical or radiological (ultrasound or MRI) follow-up.

Aggressive angiomyxoma is a benign, slow-growing, locally aggressive tumor of mesenchymal origin primarily occurring in the pelvic-perineal regions of reproductive age group women and displays a high risk of local recurrence. Lack of specific symptomatology and overlap with other benign and malignant vulval masses makes it a diagnostic challenge. We describe the case of a 32-year-old nulliparous woman with a history of recurrent vulval abscess requiring multiple incision and drainage procedures before she presented to us with an actively draining abscess on the upper third of the left labia majora. She underwent excisional biopsy at our center, the histopathology of which revealed aggressive angiomyxoma with secondary pyogenic slough. The preoperative diagnosis of vulval aggressive angiomyxoma becomes challenging due to the absence of diagnostic features. It ought to be considered a differential in every perineal-pelvic region mass in adult women.

Aggressive angiomyxoma is a rare locally invasive mesenchymal neoplasm of unknown pathogenesis arising predominantly in pelvic and perineal tissues of adult women. Surgical excision is the classical management but is associated with the risk of recurrence, especially with incomplete excision. There is a proposed role for adjuvant hormonal therapy. We report a very rare case of urethral aggressive angiomyxoma, managed by surgical excision, in a 40-year-old woman who presented with severe hematuria with clot retention.

Background  Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor predominantly affecting the pelvis and perineum of females. Due to its variable clinical presentation, it is often misinterpreted and treated as other pathologies like Bartholin gland cyst, vulvar abscess, and hernia. The tumor is locally infiltrative, making its complete surgical removal difficult. Aim  The aim of the study is to highlight and illustrate the imaging and pathological features of AAM. Materials and Methods  After obtaining clearance from the Institutional Ethics Committee, a retrospective study was conducted on patients with histopathological diagnosis of AAM, presenting to the dedicated sarcoma clinic between 2016 and 2019. The demographic, clinical, radiological, pathological, and treatment details were obtained from the institute's database. The magnetic resonance imaging (MRI) and computed tomography images were reviewed to assess different imaging parameters. Follow-up imaging was assessed for residual or recurrent mass in the pelvis or perineum and distant metastasis. Results  A total of four patients were included with an age range of 28 to 50 years. The baseline MRI of these four patients shows pelvic mass extending into the perineum, infiltrating along the surrounding fascial planes with the characteristic laminated appearance on T2-weighted image and gradual intense enhancement following contrast administration. All patients had residual disease post-surgery and were put on hormonal therapy. Conclusion  AAMs are locally aggressive, rarely metastasizing mesenchymal tumor that has a specific predilection for the perineum and pelvis of females. MRI features like laminated or striated appearance, post-contrast enhancement, and finger-like infiltrating projections should raise the suspicion of the diagnosis on baseline imaging.

Aggressive angiomyxomas are rare infiltrative mesenchymal neoplasms that commonly recur locally. The purpose of this study was to conduct a retrospective review of imaging findings of aggressive angiomyxomas with clinicopathologic correlation in 16 patients.

CT and MRI studies and clinical data of 16 patients with histopathologic evidence of aggressive angiomyxoma who had been referred to our institutions from January 2002 through January 2012 were retrospectively reviewed. The tumors were evaluated with respect to location, morphology, attenuation or signal intensity, and enhancement characteristics.

The most common location was the pelvis and perineum with the mass on either side of the pelvic diaphragm (12/16, 75%). The characteristic "laminated" appearance was seen in 10 of 12 patients on MRI. Aggressive angiomyxomas showed only mild diffusion restriction and mild (18)F-FDG avidity in both of the two patients who underwent DWI and PET/CT, in keeping with histologic low-mitotic activity. Imaging features, such as collateral vessels and fingerlike growth pattern, were seen in seven of 16 (44%) aggressive angiomyxomas. Internal cystic degeneration was seen in three of 16 (19%) aggressive angiomyxomas.

The finding of a large multicompartmental tumor with a characteristic internal laminated morphology or extension on either side of the pelvic diaphragm should alert the radiologist to the possible diagnosis of aggressive angiomyxoma. Imaging features, such as large peripheral vessels and cystic degeneration are less common, but presence of these features in the background of laminated morphology should not deter the radiologist from suggesting a diagnosis of aggressive angiomyxoma.