UAMS IM Residency Program

Sandra Susanibar

ACD – 07.25

WHAT IS THE DIAGNOSIS?

SUPERIOR VENA CAVA SYNDROME• Superior vena cava (SVC) syndrome results from obstruction of blood flow in the SVC.

• The majority of cases are due to malignancy, and approximately 95 percent of these are due to lung cancer or non-Hodgkin lymphoma (NHL).

• Can also be caused by benign causes such as fibrosing mediastinitis and thrombosis associated with intravascular devices.

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GENERAL PRINCIPLES

• Malignancy-related SVC syndrome was previously considered a potentially life-threatening medical emergency requiring immediate radiation therapy (RT) to rapidly relieve the obstruction.

• Emergency RT is no longer considered necessary for most patients.

• Current management guidelines stress the importance of accurate histologic diagnosis prior to starting therapy, and the upfront use of endovascular stents in severely symptomatic patients to provide more rapid symptom relief than can be achieved using RT.

• Important exceptions to this general approach are patients who present with stridor due to central airway obstruction or severe laryngeal edema causing respiratory compromise, and those with depressed central nervous system status from cerebral edema. These situations represent true medical emergencies, and these patients require both endovascular stenting and emergent RT to decrease the risk of sudden respiratory failure and death. (See 'Need for emergent treatment' above.)

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TREATMENT RECOMMENDATIONS • Clinical SVC syndrome who present with stridor, respiratory compromise, or depressed

central nervous system function, we recommend emergent treatment with endovascular stenting followed by radiation therapy (RT) (Grade 1B).

• For patients receiving RT on an emergency basis for severe airway obstruction, we suggest a short course of high-dose corticosteroids to minimize the risk of central airway obstruction (Grade 2C).

• For other cases, if the imaging studies are consistent with a malignancy, a histologic diagnosis is required prior to initiating specific antitumor therapy.

• When thrombus is present, systemic anticoagulation should be considered to limit extension of thrombus.

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