aggressive angiomyxoma

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.

Epidemiology

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

Pathology

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

Radiographic features

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

CT

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 .

MRI

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 . Rarely, cystic degeneration and intratumoral vessels may be seen .

Treatment and prognosis

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

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

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