Background:

Primary tumors of the inferior vena cava (IVC) are rare. They most commonly arise from the smooth muscle of the IVC walls, known as leiomyomas.

Case report:

A 49-year-old male patient presented to the emergency department with right leg swelling. He had undergone varicose vein extraction surgery one month before presentation. Doppler ultrasound revealed thrombosis of the right iliac and femoral veins. Permeability of the IVC was presumed, since symptoms were unilateral. The patient was discharged with anticoagulation therapy, rivaroxaban 15mg bid.

During follow-up, complaints of right leg swelling and discomfort persisted, raising the suspicion of post-thrombotic syndrome. A computed tomography angiography scan revealed a 12 cm-long thrombus in the infrarenal IVC. Despite ongoing anticoagulation, symptoms persisted with intermittent improvements and worsening. Consequently, additional investigation with phlebography, magnetic resonance, and positron emission tomography (PET) scan was performed, which raised a high suspicion of a leiomyoma of the IVC. An endovascular biopsy with a Cordis ® biopsy catheter confirmed the diagnosis of an IVC leiomyoma.

Open surgical resection of the tumor was proposed and accepted by the patient.

After the operation, the patient spent three days in intensive care and was discharged from the hospital after one week. At the one-month follow-up appointment, the patient remained asymptomatic.

Conclusions:

While rare, it is crucial to keep in mind the existence of IVC leiomyomas, which often occur with nonspecific symptoms. In this clinical case, the sole symptom was thrombosis of the femoral, iliac, and inferior vena cava, refractory to anticoagulation therapy. To confirm the diagnosis, biopsy is mandatory, with endovascular biopsy being an efficient and safe method.

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