Encompasses a constellation of symptoms and signs resulting from obstruction of the superior vena cava.
The increased venous pressure in the upper body results in edema of the head, neck, and arms, often with cyanosis, plethora, and distended subcutaneous vessels. Edema may cause functional compromise of the larynx or pharynx, manifested as cough, hoarseness, dyspnea, stridor, and dysphagia. Cerebral edema may lead to headache, confusion, and coma. The decreased venous return may result in hemodynamic compromise.
The most common malignant causes are non-small-cell lung cancer (50% of patients), small-cell lung cancer (25% of patients), lymphoma (approximately 10% of patients), and metastatic lesions (approximately 10% of patients).
Clinical diagnosis of obstruction of the superior vena cava is made on the basis of signs and symptoms. The most useful imaging study is computed tomography of the chest after the administration of contrast material.
1. Elevate the patient’s head to decrease the hydrostatic pressure and thereby the edema.
2. Glucocorticoid therapy: dexamethasone, 4 mg every 6 hours. Reduces the tumor burden in lymphoma and thymoma and is therefore more likely to reduce the obstruction in these patients than those with other types of tumor.
3. In patients with obstruction of the superior vena cava resulting from intravascular thrombus associated with an indwelling catheter, removal of the catheter should be considered.
4. Radiotherapy is often used to treat symptomatic patients with malignant obstruction of the superior vena cava.
5. Complete relief of symptoms of vena caval obstruction is achieved with chemotherapy in approximately 80% of patients with non-Hodgkin’s lymphoma or small-cell lung cancer and in 40% of those with non-small-cell lung cancer.
6. Placement of an intravascular stent: because the stent can be placed before a tissue diagnosis is available, it is a useful procedure for patients with severe symptoms, such as respiratory distress, that require urgent intervention. Should also be strongly considered for patients with mesothelioma, which tends not to respond well to chemotherapy or radiation, and may also be particularly useful when obstruction is caused by a thrombus associated with an indwelling catheter.
Wilson L, Detterbeck F, Yahalom J. Superior Vena Cava Síndrome with Malignant Causes. N Engl J Med 2007;356:1862-9.