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Aetiology

Superior vena cava syndrome is related to cancer in almost 90% of cases and especially to lung cancers (among them small cell carcinoma).

Other frequently responsible cancers are: lymphoma, breast cancer, mediastinal geminal tumours, thymoma and various others.

Due to the anatomical configuration, superior vena cava syndrome is observed four times more frequently on the right.

Differential diagnoses are rare: idiopathic mediastinal fibrosis, histoplasmosis, venous thrombosis related to a catheter or surgery. In fact, all pathological processes which invade or destroy the lymph structures of the superior mediastinum may provoke an obstruction to the return of venous blood and thrombosis.

Clinical presentation

Its onset is generally insidious.

Its severity depends on:

• rapidity of the obstructive process,
• thrombosis syndrome,
• precise location of the obstruction,
• possibility and rapidity of the constitution of collateral circulation.

Venous hyperpressure of the entire upper body is observed with venous distension, laryngeal oedema, increased intracranial pressure, cerebral oedema. All of these symptoms, if not treated (or if treated too late), may be fatal.

The revealing patient complaint is dyspnea, coughing, headache and facial swelling. Then, neck, chest and upper limb swelling is observed. All of these symptoms are worsened when the patient bends forwards. Diagnosis is evident as soon as this is evoked.

In reality, the development of superior vena cava syndrome is rarely acute (most often neglected). Half of the patients reveal their cancer through this syndrome. Chest radiography and thoracic scan are the best imaging procedures before a diagnostic biopsy.

You can view a typical case of superior vena cava with thrombosis and important collateral circulation.

Treatment

Most cancers, which are discovered via this syndrome, are more and less chemosensitive (small cell lung cancer, lymphoma, germ tumours). Chemotherapy is therefore the choice treatment. In less chemosensitive tumours, radiotherapy may be a good palliative option.

Other palliative measures are semi-seated position, rest, oxygen and short courses of corticosteroids.

A poor response to aetiological and palliative treatment suggests concurrent thrombosis which may require further anticoagulant treatment.

Unfortunately, most superior vena cava syndromes relapse since underlying cancer is not cured by treatment (lung cancer). The use of intravenous stents has been suggested in order to avoid acute complications.