Malignant Superior Vena Cava Syndrome: State of the Art.

Vasileios Patriarcheas,Maria Grammoustianou,Andriani Charpidou,Ioannis Gkiozos,Nikolaos Syrigos,Minas Kostis,Elias Kotteas,Nikolaos Ptohis,Evangelos Dimakakos,Ioanna Thanou,Ioannis Trontzas

doi:10.7759/cureus.20924

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.

Highlights

BackgroundSuperior vena cava syndrome (SVCS), which William Hunter first described in 1757 [1], encompasses a collection of signs and symptoms arising from the obstruction of blood flow through the thin-walled superior vena cava (SVC)
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%
Notwithstanding the fact that during the last decades, the overall incidence of benign cases of SVCS has risen and accounted for up to 40% of all cases, the majority of SVCS cases are the result of mediastinal malignancies, such as lung cancer (up to 10% of patients with small cell lung cancer (SCLC) and 2%-4% of all patients with lung cancer), non-Hodgkin lymphoma (NHL), and metastatic tumors [4,5]

Summary

Introduction

Superior vena cava syndrome (SVCS), which William Hunter first described in 1757 [1], encompasses a collection of signs and symptoms arising from the obstruction of blood flow through the thin-walled superior vena cava (SVC). SVC obstruction is more common in patients with SCLC; the higher incidence of non-small cell lung cancer (NSCLC) makes it the most widespread cause (≈50% of the cases) of malignancy-related SVCS [15]. The widespread use of indwelling central venous catheters and implanted devices during the last decades has led to an increase in the incidence of nonmalignant (benign) causes of SVC obstruction. Thrombi associated with these catheters and parts of implanted devices (e.g., pacemaker leads) have emerged as a significant cause of SVCS and account for up to 30% of all cases [6,7]. This grading system assesses the degree of edema (laryngeal and/or cerebral) and hemodynamic status to categorize SVCS between life-threatening (grade 4), severe (grade 3), and non-life-threatening cases (grade 0-2)

Grade Findings

Conclusions

Disclosures

Hunter W

Nunnelee JD