Uperior vena cava syndrome (svcs) was first described in l757 in a patient with a syphilitic

Hospital Physician  January 1999

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Table 1. Malignant Causes of Superior Vena Cava

Syndrome

Malignancy

Histologic Subtypes

Lung cancer

Small cell

Large cell

Adenocarcinoma

Undifferentiated

Lymphoma Lymphoblastic

Lymphocytic

Mixed

Nodular

Non-Hodgkin’s

Metastatic tumor

Breast

Testicular

Other malignancy

Kaposi’s sarcoma

Table 2. Benign Causes of Superior Vena Cava

Syndrome

Infectious

Tuberculosis

Histoplasmosis

Actinomycosis

Syphilis

Pyogenic

Tumors

Cystic hygroma

Substernal goiter

Teratoma

Thymoma

Dermoid cyst

Cardiac

Atrial myxoma

Pericarditis

Intrapericardial band

Mitral stenosis

Complication of central catheter

Complication of congenital heart surgery

Complication of total parenteral nutrition line

Vascular

Aortic aneurysm

Arteriovenous fistula

Polycythemia 

Idiopathic thrombosis 

Other causes

Sarcoidosis 

Postirradiation 

Mediastinal hematoma 

Pneumothorax 

Behçet’s disease

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is minimal, the physical findings may not be prominent

and the diagnosis may be more difficult to establish.

Today, establishing the underlying diagnosis and etiolo-

gy of SVCS has become more important because cer-

tain disorders that cause SVCS may be more amenable

to specific treatment regimens. For example, small cell

lung carcinoma and lymphoma respond dramatically to

chemotherapy/irradiation, whereas thrombosis from a

central line catheter does not respond to this treat-

ment.

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Laboratory Studies

Chest radiography. 

The initial diagnostic test for sus-

pected SVCS is chest radiography. Although this test is

not specific for SVCS, chest radiography may be helpful

in identifying the cause of the disorder. Findings on chest

radiography that may be helpful include widening of the

superior mediastinum, pleural effusions, and a hilar or

mediastinal mass, usually on the right side (Table 3).

These radiologic findings usually suggest an underlying

malignancy, whereas calcified lymph nodes may be more

predictive of granulomatous disease. However, the results

of chest radiography may appear normal despite an

obstruction in the superior vena cava. In the absence of

previous catheterization or surgery, a normal result on

chest radiography in a patient with SVCS is almost

pathognomonic of chronic fibrous mediastinitis.

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