Superior Vena Cava Syndrome Treatment & Management

The goals of superior vena cava syndrome (SVCS) management are to relieve symptoms and to attempt cure of the primary malignant process. Only a small percentage of patients with a rapid-onset superior vena cava (SVC) obstruction are at risk for life-threatening complications.^[17]

• Patients with clinical superior vena cava syndrome (SVCS) often gain significant symptomatic improvement from conservative treatment measures, including elevation of the head of the bed and supplemental oxygen.^[23]
• Emergency treatment is indicated when brain edema, decreased cardiac output, or upper airway edema is present. Corticosteroids and diuretics are often used to relieve laryngeal or cerebral edema, although documentation of their efficacy is questionable.
• Radiotherapy has been advocated as a standard treatment for most patients with superior vena cava syndrome (SVCS). It is used as the initial treatment if a histologic diagnosis cannot be established and the clinical status of the patient is deteriorating; however, recent reviews suggest that superior vena cava syndrome (SVCS) obstruction alone rarely represents an absolute emergency that requires treatment without a specific diagnosis.^{[3, 25]}

  • The fractionation schedule of radiation usually includes 2-4 large initial fractions of 300-400 cGy, followed by conventional fractionation of 150-200 cGy daily, to a total dose of 3000-5000 cGy. The radiation dose depends on tumor size and radioresponsiveness. The radiation portal should include a 2-cm margin around the tumor.
  • During irradiation, patients improve clinically before objective signs of tumor shrinkage are evident on chest radiography. Radiation therapy palliates superior vena cava (SVC) obstruction in 70% of patients with lung carcinoma and in more than 95% with lymphoma.
• In patients with superior vena cava syndrome (SVCS) secondary to non–small-cell carcinoma of the lung, radiotherapy is the primary treatment. The likelihood of patients benefiting from such therapy is high, but the overall prognosis of these patients is poor.^[26]
• Chemotherapy may be preferable to radiation for patients with chemosensitive tumors.^[26]

  • In 1983, Maddox and associates reported on 56 patients with small-cell lung cancer who presented with superior vena cava syndrome (SVCS). Correction of superior vena cava syndrome (SVCS) was obtained in 9 (56%) of 16 patients treated with radiation therapy alone, in 23 (100%) of 23 given chemotherapy, and in 5 (83%) of 6 who received combined therapy.^[27]
  • The most extensive experience in superior vena cava syndrome (SVCS) management secondary to non-Hodgkin lymphoma is reported from the M.D. Anderson cancer center. Patients were treated with chemotherapy alone, chemotherapy combined with radiation therapy, or radiation therapy alone. All patients achieved complete relief of superior vena cava syndrome (SVCS) symptoms within 2 weeks of the institution of any type of treatment. No treatment modality appeared to be superior in achieving clinical improvement.^[28]
• When superior vena cava syndrome (SVCS) is due to thrombus around a central venous catheter, patients may be treated with thrombolytics (eg, streptokinase, urokinase, recombinant tissue-type plasminogen activator) or anticoagulants (eg, heparin, oral anticoagulants). Removal of the catheter, if possible, is another option, and it should be combined with anticoagulation to avoid embolization.^{[7, 21]} . They are most effective when patients are treated within 5 days after the onset of symptoms.
• In a 1988 report, Adelstein et al discuss prophylaxis against embolic events in the presence of a superior vena cava (SVC) obstruction in the management of 25 patients with malignant superior vena cava syndrome (SVCS).^[29]
• Ten patients were retrospectively reviewed after having been diagnosed clinically without venography and treated without anticoagulation. Five thromboembolic complications occurred, 2 of which proved fatal.
• Fifteen patients were prospectively evaluated by means of angiography and then treated with anticoagulants. Angiographic evidence of intraluminal subclavian vein or superior vena cava (SVC) thrombosis was found in 5 of these patients, and no thromboembolic complications occurred.
• Of the 20 patients who were ultimately given anticoagulation therapy, 2 had fatal intracranial hemorrhages.
• The authors suggested the need for randomized prospective trials if the role of venography and anticoagulation in this syndrome is to be determined.^[29]

• Surgical bypass of the superior vena cava (SVC) may be a useful way to palliate symptoms in carefully selected patients.

  • Indications to proceed with such procedures are much less clear.
  • For the most part, these are patients with advanced intrathoracic disease amenable only to palliative therapy (ie, after failure of radiation therapy and chemotherapy).
  • Patients with benign disease appear to be the best candidates for bypass.^{[30, 31]}
• Superior vena cava (SVC) stenting can provide rapid symptomatic relief within few days in most patients with superior vena cava syndrome (SVCS). See the images below. Superior vena cava syndrome (case 1, cont'd). A Palmaz P308 stent mounted on a 12-mm balloon was deployed in the superior vena cava after it was predilated to 8 mm. The stent was subsequently dilated to 14 mm. Superior vena cava syndrome (case 1, cont'd). Venogram obtained after stenting shows a widely patent superior vena cava with no collateral drainage. Pressure measurements after stenting showed a 1- to 2-mm residual gradient. Superior vena cava syndrome (case 1, cont'd). Sonogram obtained 1 year after stenting shows near-normal venous pulsatility and respiratory phasicity. The patient experienced a complete resolution of symptoms.

  • Superior vena cava (SVC) stenting may provide relief of severe symptoms for patients while the histological diagnosis of the malignancy causing the obstruction is being actively pursued.^{[20, 25, 31]}
  • Stenting may also be indicated in patients in whom chemotherapy or radiation has failed.^{[32, 33, 34]}
  • Some literature recommends stenting as a first-line treatment to be performed early in the management of superior vena cava syndrome (SVCS).^{[32, 33, 34]}
  • Cases of excimer laser removal of pacemaker leads followed by venoplasty and stenting have been reported.^[35]

• Thoracic surgeon
• Hematologist/oncologist
• Radiation therapist
• Interventional radiologist