Leiomyosarcoma of the inferior vena cava

Summary:  Work-up included laboratory evaluation for hypercoagulable state along with imaging workup for occult malignancy as a possible cause of Trousseau syndrome. Initial ultrasonographic abdominal survey reveals right hydronephrosis (not shown) and CT was recommended (figure 1). Figure 2 is a vena cavagram at the time of IVC filter placement and figure 3 is microscopic section of an aspiration biopsy.

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Diagnosis
Leiomyosarcoma of the inferior vena cava (IVC) (IVC sarcoma)

Findings
The IVC is patent at the level of the renal vein. Right hydronephrosis is demonstrated (figure 1A). More inferiorly, a large 5 cm heterogeneously enhancing retroperitoneal mass extends from the renal hilar level to the iliac bifurcation (figure 1B), centered within and obliterating the IVC. The intraluminal filling defect surrounded by contrast above the iliac bifurcation (figure 1C) could represent an intraluminal component of the tumor mass or bland thrombus.

The patient underwent successful biopsy of the mass percutaneously with CT guidance. The aspirates and cell block (figure 3) demonstrate pleomorphic and cohesive groups of spindle cells, which are positively stained with vimentin and muscle specific actin (special stains not shown) indicating spindle cell neoplasm of smooth muscle differentiation, highly suspicious for leiomyosarcoma.

Laboratory evaluation confirmed antiphospholipid antibody syndrome, which, in combination with the IVC sarcoma, contributed to his recurrent and progressive DVT despite appropriate coumadin therapy. Because of retroperitoneal bleeding related to the biopsy procedure and the concern of possible pulmonary embolism, a suprarenal Greenfield stainless steel IVC filter was inserted. The vena cavogram. at the time of filter placement (figure 2) shows complete occlusion of the IVC just below the renal vein orifices.

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