Unexpected inferior vena cava syndrome: leiomyosarcoma

Leiomyosarcomas arising directly from the blood vessels are rare. These tumours are formed from the muscular wall of either a major vein or artery. The authors describe the case of an 84-year-old woman with lower back pain with bilateral abdominal irradiation and marked peripheral oedema, who was diagnosed with leiomyosarcoma of the inferior vena cava after biopsy of an infrahepatic mass. An endovascular prosthesis was placed as the patient was proposed for palliative care. Leiomyosarcomas of the inferior vena cava may present with non-specific symptoms. Therefore, the authors aim to draw attention to the diagnosis process, as well as imaging findings related to this pathology.

Inferior vena cava-syndrome

Summary: Inferior vena cava syndrome (IVCS) is caused by agenesis, compression, invasion, or thrombosis of the IVC, or may be associated with Budd-Chiari syndrome. Its incidence and prevalence are unknown. Benign IVCS is separated from malignant IVCS. Both cover a wide clinical spectrum reaching from asymptomatic to highly symptomatic cases correlated to the underlying cause, the acuity, the extent of the venous obstruction, and the recruitment and development of venous collateral circuits. Imaging is necessary to determine the underlying cause of IVCS and to guide clinical decisions. Interventional therapy has changed the therapeutic approach in symptomatic patients. This article provides an overview over IVCS and focuses on interventional therapeutic methods and results.

Inferior Vena Cava Syndrome: A Rare Complication

Inferior vena cava syndrome is rare and often difficult to diagnose because of its rarity and consequent low suspicion. We describe the case of a 28-year-old female patient with a history of nephroblastoma of the right kidney, stage IV, with a favourable histology with epidural metastasis (D5-D9), diagnosed at 3 years of age. The patient underwent treatment with surgery, chemotherapy and radiotherapy. The patient suffered from sudden low back pain worsening over 2 weeks, with progressive inability to walk. The pain radiated to the front of the thighs. Concomitantly, oedema of the lower limbs with cephalocaudal progression was observed. At admission to our institution, the physical examination showed peripheral oedema, abdominal wall venous collaterals, an inability to walk due to low back pain in the supine position, with no neurological deficits. Lumbar MRI showed exuberant epidural venous congestion. The hypothesis of inferior vena cava thrombosis (IVCT) was considered and confirmed by angio-CT. IVCT is prevalent in patients with congenital anomalies of the inferior vena cava, occurring in approximately 60–80% of these cases, and most published series on inferior vena cava syndrome refer to thrombotic complications in this subgroup of patients. There are currently no guidelines defined or validated to guide the diagnosis and approach to IVCT. With this case, we would like to draw attention to a rare disease that should be suspected in all patients with inferior vena cava disease, whether resulting from congenital disease or after surgical procedures.