Superior vena cava syndrome
Encyclopedia
Superior vena cava syndrome (SVCS), or superior vena cava obstruction (SVCO), is usually the result of the direct obstruction of the superior vena cava
by malignancies such as compression of the vessel wall by right upper lobe tumors or thymoma
and/or mediastinal lymphadenopathy
. The most common malignancies that cause SVCS is bronchogenic carcinoma. Cerebral edema is rare, but if it occurs it may be fatal.
Following are frequent symptoms:
Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.
(rare), and other acute leukemias. Syphilis
and tuberculosis
have also been known to cause superior vena cava syndrome. SVCS can be caused by invasion or compression by a pathological process or by thrombosis in the vein itself, although this latter is less common (approximately 35% due to the use of intravascular devices).
, transbronchial needle aspiration
at bronchoscopy
and mediastinoscopy
. CXRs provide the ability to show mediastinal widening and may show the presenting primary cause of SVCS. CT scans should be contrast enhanced and be taken on the neck, chest, lower abdomen and pelvis. They may also show the underlying cause and the extent to which the disease has progressed.
or methylprednisolone
) decrease the inflammatory response to tumor invasion and edema surrounding the tumor. Glucocorticoids are most helpful if the tumor is steroid-responsive, such as lymphomas. In addition, diuretics (such as furosemide
) are used to reduce venous return to the heart which relieves the increased pressure.
within one month of treatment. However, even with treatment, 90% of patients die within two and a half years. This relates to the cancerous causes of SVC that are 90% of the cases. The average age of onset of disease is 54 years of age.
Superior vena cava
The superior vena cava is truly superior, a large diameter, yet short, vein that carries deoxygenated blood from the upper half of the body to the heart's right atrium...
by malignancies such as compression of the vessel wall by right upper lobe tumors or thymoma
Thymoma
Thymoma is a tumor originating from the epithelial cells of the thymus. Thymoma is an uncommon tumor, best known for its association with the neuromuscular disorder myasthenia gravis. Thymoma is found in 15% of patients with myasthenia gravis. Once diagnosed, thymomas may be removed surgically...
and/or mediastinal lymphadenopathy
Mediastinal lymphadenopathy
Mediastinal lymphadenopathy or mediastinal adenopathy is an enlargement of the mediastinal lymph nodes.-Causes:*Tuberculosis*Sarcoidosis*Cystic fibrosis*Histoplasmosis*Acute lymphoblastic leukemia*Coccidioidomycosis*Lymphoma...
. The most common malignancies that cause SVCS is bronchogenic carcinoma. Cerebral edema is rare, but if it occurs it may be fatal.
Signs and symptoms
Shortness of breath is the most common symptom, followed by face or arm swelling.Following are frequent symptoms:
- DyspneaDyspneaDyspnea , shortness of breath , or air hunger, is the subjective symptom of breathlessness.It is a normal symptom of heavy exertion but becomes pathological if it occurs in unexpected situations...
- HeadacheHeadacheA headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
- Facial edemaEdemaEdema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
- Venous distention in the neck and distended veins in the upper chest and arms
- Upper limb edema
- Lightheadedness
- Cough
- Edema of the neck, called the collar of Stokes
Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.
Cause
Approximately 90% of cases are associated with a cancerous tumor that is compressing the superior vena cava, such as bronchogenic carcinoma including small cell and non-small cell lung carcinoma, Burkitt lymphoma, lymphoblastic lymphomas, pre-T-cell lineage acute lymphoblastic leukemiaAcute lymphoblastic leukemia
Acute lymphoblastic leukemia is a form of leukemia, or cancer of the white blood cells characterized by excess lymphoblasts.Malignant, immature white blood cells continuously multiply and are overproduced in the bone marrow. ALL causes damage and death by crowding out normal cells in the bone...
(rare), and other acute leukemias. Syphilis
Syphilis
Syphilis is a sexually transmitted infection caused by the spirochete bacterium Treponema pallidum subspecies pallidum. The primary route of transmission is through sexual contact; however, it may also be transmitted from mother to fetus during pregnancy or at birth, resulting in congenital syphilis...
and tuberculosis
Tuberculosis
Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...
have also been known to cause superior vena cava syndrome. SVCS can be caused by invasion or compression by a pathological process or by thrombosis in the vein itself, although this latter is less common (approximately 35% due to the use of intravascular devices).
Diagnosis
The main techniques of diagnosing SVCS are with chest X-rays (CXR), CT scansComputed tomography
X-ray computed tomography or Computer tomography , is a medical imaging method employing tomography created by computer processing...
, transbronchial needle aspiration
Needle aspiration biopsy
Needle aspiration biopsy , may refer to fine needle aspiration cytology , fine needle aspiration biopsy and fine needle aspiration , is a diagnostic procedure sometimes used to investigate superficial lumps or masses...
at bronchoscopy
Bronchoscopy
Bronchoscopy is a technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument is inserted into the airways, usually through the nose or mouth, or occasionally through a tracheostomy. This allows the practitioner to examine the patient's airways for...
and mediastinoscopy
Mediastinoscopy
Mediastinoscopy is a procedure that enables visualization of the contents of the mediastinum, usually for the purpose of obtaining a biopsy. Mediastinoscopy is often used for staging of lymph nodes of lung cancer or for diagnosing other conditions affecting structures in the mediastinum such as...
. CXRs provide the ability to show mediastinal widening and may show the presenting primary cause of SVCS. CT scans should be contrast enhanced and be taken on the neck, chest, lower abdomen and pelvis. They may also show the underlying cause and the extent to which the disease has progressed.
Treatment
Several methods of treatment are available, mainly consisting of careful drug therapy and surgery. Glucocorticoids (such as prednisonePrednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
or methylprednisolone
Methylprednisolone
Methylprednisolone is a synthetic glucocorticoid or corticosteroid drug. It is marketed in the USA and Canada under the brand names Medrol and Solu-Medrol. It is also available as a generic drug....
) decrease the inflammatory response to tumor invasion and edema surrounding the tumor. Glucocorticoids are most helpful if the tumor is steroid-responsive, such as lymphomas. In addition, diuretics (such as furosemide
Furosemide
Furosemide or frusemide is a loop diuretic used in the treatment of congestive heart failure and edema. It is most commonly marketed by Sanofi-Aventis under the brand name Lasix...
) are used to reduce venous return to the heart which relieves the increased pressure.
Prognosis
Symptoms are usually relieved with radiation therapyRadiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
within one month of treatment. However, even with treatment, 90% of patients die within two and a half years. This relates to the cancerous causes of SVC that are 90% of the cases. The average age of onset of disease is 54 years of age.