Intravenous leiomyomatosis manifesting as saddle embolism

Abstract

A 51-year-old, otherwise well woman, presented with progressive severe dyspnoea. CT pulmonary angiogram (CTPA) demonstrated a large filling defect within the right main pulmonary artery with evidence of right heart strain. She was anticoagulated and discharged home; however, was readmitted with progression of symptoms and hypotension within 1 month. Repeat CTPA demonstrated progression of the filling defect. Formal surgical thrombectomy was performed with removal of an unusual cream-coloured, rubber-like material. Histological analysis revealed intravenous leiomyomatosis (IVL). IVL is a rare benign neoplasm, characterised by smooth muscle cell proliferation in vascular structures that can act aggressively. This case describes the workup, recognition and management of IVL.

Keywords: radiology (diagnostics); vascular surgery; venous thromboembolism.

Figure 1

Coronal CT-thorax abdomen and pelvis displaying a filling defect extending from the left common iliac vein into the intrahepatic inferior vena cava (arrowed).

Figure 2

Axial CT pulmonary angiogram displaying saddle-filling defect.

Figure 3

Radiological features of the filling defect that suggest an underlying complexity. Mixed signal intensity within the left common iliac vein on axial T2 MRI (A) and similar heterogeneity within the inferior vena cava on axial CT (B) may represent internal vascularity of the intravenous leiomyomatosis.