Superior vena cava syndrome . Superior vena cava syndrome is generally a sign of locally advanced bronchogenic carcinoma . Survival depends on the disease state of the patient. When small cell bronchogenic carcinoma is treated with chemotherapy, the average survival time with or without SVCS is almost identical (42 weeks or 40 weeks).
The 24-month survival is 9% in patients without SVCS and 3% in those with the syndrome. When the malignancy is treated with radiation therapy, 46% of patients with lung cancer non – small cell experience relief of symptoms compared with 62% of patients with bronchogenic carcinoma cells small. The 2-year survival of 5% is almost the same for both groups.
Summary
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- 1 Etiology
- 2 Clinical picture
- 3 Evaluation and Diagnosis
- 4 Treatment
- 5 Sources
Etiology
The obstruction of the venous drainage of the superior cava can be secondary to compression or infiltration by mediastinal tumors, generally bronchopulmonary (bronchogenic carcinoma). It may also be due to lymphoma, metastasis, and, more rarely, cardiac tumors originating from the atrium or pericardium. The syndrome can be caused by benign processes, such as the presence of a thoracic aortic aneurysm, mediastinitis, mediastinal fibrosis, constrictive pericarditis, retrosternal thyroid, venous thrombosis caused by central catheters, lymphadenopathy, Behçet’s disease, or trauma. The progression of venous thrombosis of the upper limb is a rare cause.
Clinical picture
The clinical signs are numerous and varied. They are commonly exacerbated by lying, chewing, or physical exertion.
The most frequent signs and symptoms, ordered from highest to lowest incidence, are: dilation of the jugular veins, facial and cervical edema, dyspnea, cough, flushing or cyanosis of the face and neck, dilation of the superficial veins of the upper limbs and upper chest, upper limb edema, nasal congestion, dysphonia, stridor, vertigo, headache, glottis edema, tongue edema, rhinorrhea, and dysphagia. The coexistence of facial, neck, upper thorax, and upper limb edema is classically referred to as edema in slaves.
It is exceptional that a pulmonary embolism occurs from a superior vena cava thrombosis. However, cases of massive pulmonary thromboembolism have been described.
Assessment and Diagnosis
Once superior vena cava syndrome (SVCS) is recognized, prompt clinical attention is important. A diagnosis should be established before starting therapy for the following reasons:
- 75% of patients have symptoms and signs for more than 1 week before seeking medical attention
- cancer patients diagnosed with SVCS die not from the syndrome itself but from the degree of their underlying disease, and
- 3% to 5% of patients diagnosed with SVCS do not have cancer.
In the absence of tracheal obstruction, SVCS is unlikely to be a life-threatening cancer emergency and treatment is not warranted before definitive diagnosis.
The patient’s initial evaluation should include a chest x- ray to look for mediastinal masses and related conditions such as pleural effusion, lobar collapse, or cardiomegaly. Computed tomography (CT) scan of the chest offers the most useful diagnostic information and can define the anatomy of the affected mediastinal nodes. Venous opening and thrombus presence are determined using contrast and rapid scanning techniques. Depending on local expertise, contrast or nuclear venography, magnetic resonance imaging, and ultrasound may be valuable in determining the site and nature of the obstruction.
If bronchogenic carcinoma is suspected, a sputum sample should be obtained. If the sputum sample is negative, a biopsy sample should be taken from the most accessible site that is clinically affected with disease. The biopsy strategy depends on the working diagnosis, the location of the tumor, the patient’s physiological state and the expertise available at the health institution. This may include a bronchoscopy, palpable cervical or supraclavicular lymph node biopsy, needle biopsy of a lung mass or mediastinal nodes guided by CT or ultrasound, mediastinoscopy, mediastinotomy, medial sternotomy, video-assisted thoracoscopy, and conventional thoracotomy. The biopsy results will help the clinician plan appropriate treatment.
Treatment
The limitations of chemotherapy and radiotherapy for the treatment of malignant tumors related to superior vena cava syndrome lead to palliative treatments. If symptoms are severe, vena cava obstruction will be considered. For this, endoluminal techniques offer great advantages with minimal risks. Fibrinolysis, if there is an added thrombus, followed by angioplasty and application of a stent.
Anticoagulant treatment must be instituted and maintained for a long time to avoid reocclusion. When the cause is benign or treatable, it must be corrected to avoid venous obstruction. Surgery has been used to repair, decompress or bypass the superior vena cava in benign processes. However, endoluminal techniques have become the treatment of choice due to their excellent results and low risk.
