Presentation
Acute left-sided pleuritic chest pain; epigastric and left upper quadrant pain. Previous bilateral lower limb deep venous thromboses. Elevated D-dimer. Pulmonary embolus?
Patient Data
There is a left upper zone nodule, projected between the anterior ends of the first and second ribs.
There are further smaller nodules within both lower zones.
No pulmonary thromboembolism.
There are however numerous intrapulmonary nodules, multiple hypoattenuating masses throughout the imaged liver, and sclerotic lesions within the T12 vertebral body and the right scapula.
There is a large, lobulated, heterogeneous and minimally calcified retroperitoneal soft tissue mass centered on the infrarenal inferior vena cava. Much of the infrarenal inferior vena cava is imperceptible; tumor thrombus is seen extending cranially through the lumen of the inferior vena cava up to the level of the liver. The renal veins and right atrium are spared.
Marked pelvic venous congestion, presumably consequent on impaired drainage into the iliocaval system.
Numerous hypoattenuating liver masses and basal pulmonary nodules in keeping with metastases.
Partially captured soft tissue nodule within the anterior musculature of the right thigh; intraperitoneal soft tissue nodule within the left iliac fossa, deep to the transversus aponeurosis; subcutaneous soft tissue mass overlying the left-sided lumbar paraspinal musculature; all in keeping with metastases.
Sclerotic lesions within inferior right scapula, within the T12 vertebral body, and within the left ilium in keeping with osseous metastases.
Ultrasound-guided core biopsy of the mass overlying the left-sided paraspinal musculature was performed.
Histopathological analysis revealed leiomyosarcoma of intermediate grade.
Case Discussion
Inferior vena cava leiomyosarcoma is a rare, slow-growing malignancy of the smooth muscle cells of the inferior vena cava 1.
The majority of cases occur in women 2. Many patients present with abdominal pain 4. A minority, as in this case, presents with (or have a history of) lower limb deep venous thrombosis 4.
Metastatic disease is present in up to 50% of cases at presentation, usually involving the liver, followed by the lung 3,4.
Treatment is typically via surgical resection 4. Prognosis is poor however, with recurrence affecting roughly 50% of patients 4. The 5-year survival is 33% 4. The disease in this case is inoperable, and the patient was offered chemotherapy.
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