Aggressive angiomyxoma
Updates to Article Attributes
Aggressive angiomyxomas are rare tumours that arise in the pelvis and typically cross the levator ani muscles. Despite its name, it is essentially a benign tumour and the term "aggressive" is given due to a predilection for local recurrence. Only rarely does it metastasise.
Epidemiology
It is seen predominantly in women (>90%) in the second through fourth decades of life 3.
Pathology
Aggressive angiomyxoma is a mesenchymal tumour that arises from connective tissue.
Radiographic features
Classically shows involvement of both pelvic and perineal compartments.Rarely, if it happens in males, it involves the inguinoscrotal region 4.
CT
On CT, a well-defined mass with attenuation less than or equal to that of adjacent skeletal muscle is typically seen, often with swirled enhancing tissue internally 3.
MRI
Reported signal characteristics of the lesion include:
- T1: tends to be iso to low signal
- T2: predominantly high signal and typically gives a swirling appearance. Due to the presence of collagen fibrils in myxoid tissue, on T2W images and post contrast T1 images, laminated pattern is seen with alternating hyper and hypointense linear areas 4.
- cystic degeneration and intratumoral vessels can rarely be seen 4.
Treatment
Surgical resection with long term follow up is treatment of choice. As this tumour has local infiltration, so chances of recurrence are high 4.
In ER and PR positive cases, hormonal therapy is also found to be helpful 4.
-<p><strong>Aggressive </strong><strong>angiomyxomas</strong> are rare tumours that arise in the <a href="/articles/pelvis-1">pelvis</a> and typically cross the <a href="/articles/levatore-ani-muscules">levator ani muscles</a>. Despite its name, it is essentially a benign tumour and the term "aggressive" is given due to a predilection for local recurrence. Only rarely does it metastasise.</p><h4>Epidemiology</h4><p>It is seen predominantly in women (>90%) in the second through fourth decades of life <sup>3</sup>.</p><h4>Pathology</h4><p>Aggressive angiomyxoma is a mesenchymal tumour that arises from connective tissue.</p><h4>Radiographic features</h4><p>Classically shows involvement of both pelvic and perineal compartments.</p><h5>CT</h5><p>On CT, a well-defined mass with attenuation less than or equal to that of adjacent skeletal muscle is typically seen, often with swirled enhancing tissue internally <sup>3</sup>.</p><h5>MRI</h5><p>Reported signal characteristics of the lesion include:</p><ul>- +<p><strong>Aggressive </strong><strong>angiomyxomas</strong> are rare tumours that arise in the <a href="/articles/pelvis-1">pelvis</a> and typically cross the <a href="/articles/levatore-ani-muscules">levator ani muscles</a>. Despite its name, it is essentially a benign tumour and the term "aggressive" is given due to a predilection for local recurrence. Only rarely does it metastasise.</p><h4>Epidemiology</h4><p>It is seen predominantly in women (>90%) in the second through fourth decades of life <sup>3</sup>.</p><h4>Pathology</h4><p>Aggressive angiomyxoma is a mesenchymal tumour that arises from connective tissue.</p><h4>Radiographic features</h4><p>Classically shows involvement of both pelvic and perineal compartments.Rarely, if it happens in males, it involves the inguinoscrotal region <sup>4</sup>.</p><h5>CT</h5><p>On CT, a well-defined mass with attenuation less than or equal to that of adjacent skeletal muscle is typically seen, often with swirled enhancing tissue internally <sup>3</sup>.</p><h5>MRI</h5><p>Reported signal characteristics of the lesion include:</p><ul>
-<strong>T2</strong>: predominantly high signal and typically gives a swirling appearance </li>-</ul>- +<strong>T2</strong>: predominantly high signal and typically gives a swirling appearance. Due to the presence of collagen fibrils in myxoid tissue, on T2W images and post contrast T1 images, laminated pattern is seen with alternating hyper and hypointense linear areas <sup>4</sup>.</li>
- +<li>cystic degeneration and intratumoral vessels can rarely be seen <sup>4</sup>.</li>
- +</ul><p> </p><h4>
- +<strong>T</strong><strong>reatment</strong>
- +</h4><p>Surgical resection with long term follow up is treatment of choice. As this tumour has local infiltration, so chances of recurrence are high <sup>4</sup>. </p><p>In ER and PR positive cases, hormonal therapy is also found to be helpful <sup>4</sup>.</p>
References changed:
- 4. Surabhi V, Garg N, Frumovitz M, Bhosale P, Prasad S, Meis J. Aggressive Angiomyxomas: A Comprehensive Imaging Review With Clinical and Histopathologic Correlation. American Journal of Roentgenology. 2014;202(6):1171-1178. href="https://www.ajronline.org/doi/full/10.2214/AJR.13.11668"
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