Malignant Superior Vena Cava Syndrome: State of the Art

Abstract

Superior vena cava syndrome (SVCS) is a clinical entity characterized by signs and symptoms arising from the obstruction or occlusion of the thin-walled superior vena cava (SVC) and can result in significant morbidity and mortality. Despite the rise of benign cases of SVCS, as a thrombotic complication of intravascular devices, it is most commonly seen secondary to malignancy as a consequence of thrombosis, direct invasion of tumor cells inside the vessel, or external compression. SVCS can be the initial presentation of a previously undiagnosed tumor in up to 60% of cases. Lung cancer and non-Hodgkin lymphoma (NHL) are responsible for up to 85%-90% of malignancy-related SVCS, while metastatic cancers account for approximately 10%. Herein, we review the pathophysiology, etiology, clinical presentation, diagnosis, and management of malignancy-related SVCS.

Keywords: endovascular therapy; lung cancer; malignancy-related superior vena cava syndrome; non-hodgkin lymphoma (nhl); superior vena cava obstruction; superior vena cava syndrome; thoracic oncology.

Figure 1. CT axial and coronal images of a patient with diffuse large B-cell lymphoma causing severe mass effect on SVC and right brachiocephalic vein

Figure 2. Venography depicting extensive stenosis of the SVC together with thrombosis (left) and recanalization of the lesion with combined thromboaspiration and stent placement technique (right)