Superior Vena Cava Syndrome

James Metz, MD
Updated by: Lara Bonner Millar, MD
Abramson Cancer Center of the University of Pennsylvania
Last Modified: November 2, 2011

Superior vena cava (SVC) syndrome is a situation that requires emergency evaluation and treatment. The syndrome is caused by compression of the superior vena cava, which is the major vein that drains blood from the head, neck, upper chest and arms. This may cause shortness of breath, facial and neck swelling, arm swelling and cough. It can progress very rapidly and can require the use of a respirator to assist with breathing.

Cancer is the most common cause of SVC syndrome. Lung cancer, lymphoma, and germ cell tumors of the chest are the cancers most likely to cause SVC syndrome. Any cancer that has spread to the lymph nodes in the chest may cause compression of the superior vena cava. Some patients present with SVC syndrome as their first symptom of cancer. It can also be caused by some non-cancerous conditions including goiter, aortic aneurysm, and inflammation of the mediastinum (area in the center of the chest). Rarely, central venous catheters can cause a blood clot to form and contribute to a SVC syndrome.

How is SVC syndrome diagnosed & treated?

Your physician will perform a complete history and physical exam. They will look specifically for signs associated with SVC syndrome. A chest x-ray and CT scan of the chest may be recommended. A CT scan can show compression of the SVC very clearly.

SVC syndrome caused by cancer requires prompt initiation of treatment. This condition can progress rapidly. Patients with a diagnosis of cancer may be placed on steroids to help relieve the swelling. Radiation therapy directed at the mass causing the obstruction may give relief rapidly. Chemotherapy may be utilized for the treatment of SVC when the tumor is considered responsive to specific medications. A stent may be placed by interventional radiology to open the superior vena cava in certain situations. Rarely, surgery may be considered as primary management of SVC syndrome. If the SVC syndrome is caused by a blood clot, blood thinners are utilized.

For those patients without a cancer diagnosis who present with a mass in the chest and clinical SVC syndrome, a biopsy must be obtained to confirm a malignancy. If treatment with steroids or radiation is initiated before a biopsy, it can be very difficult to obtain an accurate diagnosis. This could dramatically impact subsequent cancer management.

SVC syndrome is a true cancer emergency. If you are a cancer patient, any new swelling in the face and arms associated with shortness of breath should be reported to your oncologist immediately.

See OncoLink's Nurses' Notes article on SVC syndrome.

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