Eric L. Smith and Jeffrey S. Groeger
Definition
• Compression or invasion of the SVC causing increased venous pressure resulting classically in face & arm swelling, vein distension, & dyspnea (see below)
Epidemiology
• Approx. 15000 new cases diagnosed in US annually
• 60–80% a/w malignancy
Differential Diagnosis
• Thrombus: Esp in presence of indwelling catheter (Chest 2003;123:809)
• Infxn: TB, syphilis, fungal
• Fibrosing mediastinitis
• Postradiation fibrosis
• Aortic aneurysm
Diagnosis
• CT + contrast, or MRI if cannot give contrast, will determine thrombosis vs. extrinsic compression
• If extrinsic compression will need bx to confirm malignancy & obtain dx in many cases. Bx of distant LN or pleural effusion cytology may suffice.
Management
• Determined by: Severity, rapidity of onset, & underlying cause
• In general, obtain bx before XRT or chemotherapy (Clin Oncol 1997;9:83)
Exception: Sev. stridor or elevated ICP requires immediate intervention
• Tx options:
Elevate head of bed
XRT
± steroids: Dexamethasone 4 mg q6h
± loop diuretics
± chemo: If thought to be rapidly responsive (see below)
often chemo can be only tx
• Intravascular stent: Can be placed urgently in pts requiring tissue dx (Vascular 2007;15:314)
Also recommended in all pts w/malignancy thought unlikely to respond to XRT or chemo (see below) or in thrombosis
• Surgical bypass graft infrequently used, can be part of attempted curative multimodality tx if caused by thymoma
• If thrombus: Fibrinolytic + anticoagulation, consider removing any associated indwelling catheter
• SVC syndrome does not change the likelihood of cure of an underlying malignancy & should not compromise the choice of curative tx