Aggressive angiomyxoma

Last revised by Dr Dalia Ibrahim on 11 Sep 2021

Aggressive angiomyxomas are rare tumors that arise in the pelvis and typically cross the levator ani muscles. Despite its name, it is essentially a benign tumor and the term "aggressive" is due to a predilection for local recurrence. Only rarely does it metastasize.

It is seen predominantly in women (>90%) in the second through fourth decades of life 3.

Aggressive angiomyxoma is a mesenchymal tumor that arises from connective tissue.

Classically shows involvement of both pelvic and perineal compartments. In males, it may involve the inguinoscrotal region 4.

On CT, it usually presents as a well-defined mass with attenuation less than or equal to that of adjacent skeletal muscle. It often has swirled enhancing tissue internally 3.

Reported signal characteristics of the lesion include:

  • T1: tends to be iso to low signal
  • T2: predominantly high signal and typically has a swirled appearance

Due to the presence of collagen fibrils in the myxoid tissue, a laminated pattern may be seen on T2W images and post contrast T1 images, with alternating hyper- and hypointense linear areas 4. Rarely, cystic degeneration and intratumoral vessels may be seen 4.

Surgical resection with long term follow up is the treatment of choice. The "aggressive" tumor is infiltrative, so chances of local recurrence are high 4

In ER and PR positive cases, hormonal therapy is also found to be helpful 4.

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Cases and figures

  • Case 1
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  • Case 2
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  • Case 3: para-testicular aggressive angiomyxoma
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  • Case 4
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