Aggressive angiomyxoma: an unusual presentation

Abstract

Aggressive angiomyxoma is an uncommon mesenchymal myxoid tumor that is characterized by slow growth and frequent local recurrence. It is currently regarded as a nonmetastasizing tumor. We describe a case of recurrent aggressive angiomyxoma with invasion into the veins including the inferior vena cava and the right atrium and with pulmonary metastases. Our case, together with those unusual cases documented in previous reports, may lead to a reappraisal of the nature of aggressive angiomyxoma.

Keywords: Aggressive angiomyxoma; Computed tomography; Histopathology; Inferior vena cava; Pulmonary metastases.

Fig. 1

Aggressive angiomyxoma in 37-year-old woman. A. Unenhanced CT image shows large abdominal mass (stars) being hypo-attenuated in relation to surrounding muscular tissue. B. Contrast-enhanced CT image shows large homogeneous abdominal mass (stars). It directly invades inferior vena cava (long arrow), causing obstruction of inferior vena cava and formation of periaortic venous collaterals (short arrow). C. Coronal reconstruction image from contrast-enhanced CT shows mass encasing colon (long arrow) and causing portion of loop of small bowel to deviate superiorly (short arrow). D. Coronal reconstruction image from contrast-enhanced CT shows inferior vena cava filling defect (long black arrow) extending superiorly to level of right atrium (short black arrow). Image also shows prominent enhancing nodules within inferior vena cava (black arrowheads) and collateral vessels around aorta (white arrow). E. Contrast-enhanced CT image shows heterogeneously enhancing mass in left lower lobe (long arrow) and apparent inferior vena cava filling defect (short arrow). F. Low-power view shows vascular appearance of tumor, against myxoid, hypocellular background. G. Medium-power view shows bland cytological appearance of spindle cells.