Diagnosis and management of superior vena cava (SVC) syndrome

CT: computed tomography; MRI: magnetic resonance imaging; RT: radiation therapy; DVT: deep vein thrombosis; SCLC: small cell lung carcinoma; GCT: germ cell tumor; NSCLC: non-small cell lung carcinoma.
* Risk factors include known malignancy, central venous access, and prior DVT.
¶ Grading symptoms:

• Grade 1: Mild; edema in the head or neck (vascular distention), cyanosis, plethora.
• Grade 2: Moderate; edema in the head or neck with functional impairment (mild dysphagia, cough, mild or moderate impairment of the head, jaw or eyelid movements, visual disturbances caused by ocular edema).
• Grade 3: Severe; mild or moderate cerebral edema (headache, dizziness), mild/moderate laryngeal edema, or diminished cardiac reserve (syncope after bending).
• Grade 4: Life threatening; significant cerebral edema (confusion, obtundation), significant laryngeal edema (stridor), or significant hemodynamic compromise (syncope without precipitating factors, hypotension, or renal insufficiency).

Δ Options include catheter-based venography, with possible endovenous thrombus removal and endovenous stenting as needed, or CT venography, depending on the severity of initial symptoms (refer to UpToDate text) and institutional resources.
◊ Initial chemotherapy is appropriate for chemotherapy-sensitive malignancies (SCLC, lymphoma, GCT). Initial RT is appropriate for radiosensitive tumors; patients with NSCLC may achieve more rapid palliation with endovascular stenting followed by RT for disease control. Selected patients with thymoma/thymic carcinoma or a residual mass after treatment of GCT may benefit from surgical resection.