Superior Vena Cava Syndrome

Superior vena cava (SVC) syndrome results from obstruction of the SVC. The most common malignant causes of SVC syndrome are non-small-cell lung cancer, small-cell lung cancer, non-Hodgkin lymphoma, and metastatic lesions. Propagated thrombosis around a central line should also be considered. The diagnosis of SVC syndrome is suspected based on classic symptoms and is confirmed by imaging with contrast-enhanced CT, MR venography, ultrasound, or catheter-based venography.

Decreased venous return may result in hemodynamic compromise. Although presentation of SVC syndrome can be impressive, it is rarely an emergency. Many cases have a relatively benign course, and some patients may improve without emergent therapy. Treatment of SVC syndrome is twofold: management of symptoms and management of the malignancy.

Presenting Signs and Symptoms

Patients with SVC syndrome present with edema of the head, neck, and arms, often with cyanosis, and distended subcutaneous vessels. SVC syndrome may also cause the following signs and symptoms:

• cough

• hoarseness

• dyspnea

• stridor

• syncope

• headache

• confusion

The following table lists various symptoms of SVC syndrome and their frequency:

(Source: Superior Vena Cava Syndrome with Malignant Causes. N Engl J Med 2007.)

(Source: Superior Vena Cava Syndrome with Malignant Causes. N Engl J Med 2007.)

Grading of SVC Syndrome

SVC syndrome is graded on a scale of 0 to 5:

Treatment

Symptomatic treatment of SVC syndrome:

• For supportive care, raise the head of the bed and provide supplemental oxygen for dyspnea.

  • Patients with life-threatening SVC syndrome may require intensive care for airway protection or blood pressure support.

• Endovascular stenting has demonstrated high success rates and is the most commonly used procedural intervention for SVC syndrome.

• Although glucocorticoids (i.e., dexamethasone 4 mg every 6 hours) are sometimes used, their benefit is unclear in this setting, and tumors that respond strongly to glucocorticoids, such as lymphoma, can be more difficult to diagnose.

Treatment of malignancy: Patients require radiation therapy or chemotherapy (small-cell lung cancer and lymphoma are very chemosensitive, and rapid initiation of effective systemic therapy is often preferred in these malignancies). Tissue diagnosis is needed prior to radiation therapy or systemic therapy and should be expedited (especially in the presence of airway compromise) with rushed pathology.

An algorithm for treating malignant causes of SVC syndrome can be found here, and a table comparing the various treatment options can be found here.