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Address reprint requests and correspondence to Udaya B. S. Prakash, MD, Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, 200 First St SW, Rochester, MN 55905
Affiliations
Adviser to fellow and Consultant in Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, Rochester, Minn
A 59-year-old man presented with the chief complaint of epistaxis (approximately 100 mL) of 1 day's duration. He had no previous history of epistaxis or nasal trauma. One week before presentation, he had an episode of hemoptysis, 5 mL of bright red blood without concurrent epistaxis. Two weeks before presentation, the patient experienced dry cough, diffuse pressurelike headache, fullness of the neck, and a brief syncopal spell. He had no history of dyspnea or constitutional symptoms. His medical history included subclinical hypothyroidism and mild hypercholesterolemia. He had a 60-pack-year history of smoking but had stopped smoking 15 years before presentation. His only medication was pravastatin.
Physical examination revealed normal vital signs, rightsided epistaxis, facial plethora, swelling of the neck, engorged neck veins, and a firm right supraclavicular lymph node with a diameter of 1 cm. Prominent superficial venous dilatation was visible over the thorax. The thyroid was mildly diffusely enlarged, with no palpable nodules. His lungs were clear on auscultation, pupils were equal in size and reaction, and arms were not swollen. Findings on the rest of the examination were normal.
Results of laboratory tests, including a complete blood cell count, serum electrolytes, creatinine, calcium, sensitive thyroid-stimulating hormone, and bilirubin level, were normal. The alkaline phosphatase level was 376 U/L (reference range; 98–251 U/L), and elevation was predominantly of hepatic origin. The aspartate aminotransaminase level was 33 U/L (reference range, 12–31/U/L), and the erythrocyte sedimentation rate was 76 mm in 1 hour (reference range, 0–22 mm in 1 hour). Oxygen saturation, measured by transcutaneous pulse oximeter, was 95%.
1.
Which one of the following symptoms is most commonly encountered in the clinical syndrome previously described?
a.
Dyspnea
b.
Headache
c.
Dysphagia
d.
Cough
e.
Hoarseness
This patient's clinical features are consistent with superior vena cava syndrome (SVCS). The most common symptom reported with SVCS is dyspnea (71%), followed by headache (11%), dysphagia (9%), cough (4%), and visual disturbance (4%).
Other less common symptoms are hoarseness, epistaxis, dizziness, nasal congestion, syncopal attacks, chest pain, lethargy, tongue swelling, and hemoptysis.
Recumbency or bending over may exacerbate symptoms. The most common physical sign in SVCS is swelling of the face or upper extremities or both. Other physical findings include jugular venous distention (27%–67%), plethora (13%–20%), chest or shoulder swelling (7%), distention of thoracic veins (67%), or cyanosis (13%).
Less common findings are proptosis, glossal or laryngeal edema, conjunctival suffusion, mental status changes, decreased visual acuity, or Homer syndrome. The severity of symptoms depends on the degree of superior vena caval narrowing and rapidity of onset of obstruction. Among patients with SVCS secondary to an underlying malignancy, symptoms appear within 2 weeks of patient presentation in 31% of cases.
Recent onset of symptoms is generally suggestive of a malignant cause, and chronic symptoms are more suggestive of a nonmalignant cause. The exception is thrombosis of the superior vena cava, which is usually rapidly symptomatic. Our patient's relatively quick onset of symptoms and signs suggests an underlying malignant cause of superior vena caval obstruction. His long-term smoking history led us to consider primary lung cancer as the most likely cause of SVCS. Chest radiography was performed.
2.
Which one of the following chest radiographic findings is most likely to be seen in our patient?
a.
Superior mediastinal widening
b.
Pleural effusion
c.
Diffuse lung infiltrates
d.
Normal findings
e.
Right hilar mass
The most common radiographic finding in SVCS is superior mediastinal widening, which occurs in 59% of patients.
Hilar adenopathy and venous congestion secondary to superior vena caval obstruction can contribute to mediastinal widening. Superior vena cava syndrome occasionally leads to development of pleural effusion or diffuse lung infiltrates.
In the early stages of SVCS, chest radiographic findings can be normal, but normal findings would not be expected in this patient with multiple clinical signs of SVCS. A right hilar mass, evident in 19% of such patients, suggests underlying bronchogenic cancer. Tumors on the right side of the lung are 4 times more likely than tumors on the left to cause SVCS because of the site of the superior vena cava.
Chest radiography showed a mass in the apical segment of the right upper lobe of the lung and elevation of the right hemidiaphragm, with apparent pleural thickening (Figure 1).
Figure 1chest radiograph showing right apical mass and elevation of the right hemidiaphragm, with apparent pleural thickening.
Which one of the following is the most likely underlying cause of this patient's clinical presentation?
a.
Non-small cell lung cancer
b.
Small cell lung cancer
c.
Lymphoma
d.
Metastatic carcinoma
e.
Retrosternal goiter
The most likely diagnosis in this patient with hemoptysis, a lengthy smoking history, and a lung mass is lung cancer. Primary intrathoracic malignancy causes 87% to 97% of cases of SVCS.
Most bronchogenic carcinomas that cause SVCS are non-small cell cancers, including squamous cell carcinomas (21%), adenocarcinoma (6%–16%), and large cell or other carcinomas (11%).
In our patient, the presence of a peripherally located lung mass strongly suggests non-small cell lung cancer. It is very uncommon for a small cell lung cancer to manifest with a peripherally located lung mass.
The second most common malignancy causing SVCS is non-Hodgkin lymphoma (13%); Hodgkin lymphoma is a rare cause (1%).
thrombosis of the superior vena cava (de novo or secondary to intravascular lines or pacemaker wires), mediastinal fibrosis (including infections like histoplasmosis), granulomatous disease, retrostemal goiter, iatrogenic (after a Mustard operation in which systemic and pulmonary venous returns are redirected in patients with transposition of the great vessels), pneumothorax, mediastinal emphysema, silicosis, and benign mediastinal masses (such as dermoid cysts).
Our patient's relatively quick onset of symptoms (within 2 weeks), lengthy history of smoking, hemoptysis, and right hilar mass indicate that non–small cell lung cancer is the most likely cause of SVCS. He had no evidence of a large goiter, and no retrostemal extension of the thyroid was seen on chest radiography; thus, retrostemal goiter is an unlikely cause of his symptoms. Furthermore, a lymphoma is unlikely because he had no evidence of diffuse lymphadenopathy or a large mediastinal mass. Besides the intrathoracic findings and a single supraclavicular lymph node, there was no other evidence of a diffusely metastatic cancer, making this an unlikely cause of SVCS.
4.
Which one of the following is the next most appropriate step in the management of this patient?
a.
Emergent external beam radiation therapy
b.
Urgent administration of high-dose intravenous glucocorticoids
c.
Computed tomography (CT) of the chest
d.
Contrast venography of the superior vena cava
e.
Transesophageal echocardiography
External beam radiation therapy should not be recommended until a definitive diagnosis has been established. In the past, however, SVCS was considered an oncologic emergency requiring immediate treatment with intravenous corticosteroids and radiation therapy.
In patients with advanced disease and severe dyspnea, high-dose intravenous glucocorticoids (dexamethasone, 16 mg/d) and diuretics may be administered while a primary cause is being pursued, but documentation of efficacy is limited.
Because our patient was not experiencing severe respiratory symptoms, high-dose intravenous glucocorticoids were not urgently indicated. Further noninvasive imaging for a more accurate determination of the chest radiographic abnormality is the next most important step. Chest CT provides anatomical details of the intrathoracic and musculoskeletal structures of the chest, allows identification of the cause of the obstruction (extrinsic vs intraluminal thrombus), documents collateral circulation, and provides guidance for percutaneous biopsy.
In this patient, chest CT is the next most appropriate step in further delineating the extent of disease. Alternatively, nuclear magnetic resonance could be considered.
Contrast venography of the superior vena cava best defines the level and degree of obstruction, collateral circulation, and venous pressure, but it has the risk of an invasive procedure and is primarily used in planning surgical procedures.
Adjunctive studies include ultrasonography, transesophageal echocardiography, or radionuclide venography, but data are limited on their general use in SVCS.
Chest CT showed a large right hilar mass (Figure 2), a 3cm spiculated mass in the right upper lobe of the lung laterally, narrowing at the right upper lobe of the bronchus, obstruction of the superior vena cava, and postobstructive volume loss in the right upper and middle lobes of the lung. The most likely diagnosis is lung cancer. Our patient was admitted to the hospital, the head of his bed was raised, and he was given high-dose intravenous glucocorticoids.
Figure 2Chest computed tomogram showing right hilar mass.
Which one of the following is the most appropriate diagnostic test in this patient?
a.
Thoracentesis
b.
Mediastinoscopy
c.
Thoracotomy
d.
Biopsy of palpable lymph node
e.
Bronchoscopy with biopsy
Diagnostic yield for thoracentesis (if pleural effusion is present) is 33%; mediastinoscopy (with use of local or general anesthesia), up to 100%; thoracotomy, 100%; biopsy of palpable lymph node, 85% to 87%; bronchoscopy with biopsy, 46% to 60%; and sputum cytology, 33% to 68% (higher yields in large central tumors).
However, risks of thoracotomy include general anesthesia, acute airway obstruction, severe bronchospasm, uncontrollable bleeding from extensive venous collaterals, and cardiovascular collapse.
Furthermore, if the clinical suspicion of lung cancer has been confirmed, surgery is not an option because involvement of the supraclavicular lymph node and superior vena cava indicates unresectability. Generally, in patients with SVCS and a mediastinal mass on CT, a histological diagnosis is first attempted via biopsy of a palpable node, bronchoscopy, thoracentesis (if pleural effusion is present), or CT-guided needle aspiration of the lung.
In our patient, the least invasive procedure that would provide the greatest diagnostic yield is biopsy of a suspicious palpable lymph node. Mediastinoscopy or thoracotomy could be considered if the diagnosis was still inconclusive.
Reasons for obtaining a histological diagnosis before emergency radiation therapy include ruling out a benign cause and optimizing therapy (such as chemotherapy for small cell lung cancer or lymphomaj.
A lymph node biopsy is also a reasonable option; however, in our patient it was deferred because the palpable right supraclavicular lymph node was thought to be of questionable importance. Furthermore, the lung lesion detected on chest CT was believed to be easily accessible bronchoscopically. Thus, our patient underwent bronchoscopy with biopsy, which revealed squamous cell carcinoma of the lung, grade 4. After treatment with intravenous corticosteroids, our patient began a course of external beam radiation to the chest, delivered in 2-Gy fractions, for an anticipated total dose of 40 Gy. He experienced substantial improvement of his symptoms and signs of superior vena caval obstruction. Two weeks later, a course of chemotherapy, consisting of etoposide and cisplatin, was instituted. Approximately 1 month after initial presentation, the patient died of pneumococcal pneumonia and sepsis, likely exacerbated by immunosuppression.
Discussion
The superior vena cava is the major drainage channel for venous blood returning from the head, neck, and upper extremities. Anatomically, the right and left brachiocephalic veins unite to form the superior vena cava, which drains into the right atrium. The superior vena cava is 6 to 8 em in length and 1.5 to 2 cm in diameter.
The superior vena cava is susceptible to obstruction because it is a thin-walled, low-pressure vessel that lies in a nondistensible space in the mediastinum, where it can be compressed by fibrosis, tumor, lymphadenopathy, or a retrosternal goiter.
When the superior vena cava is obstructed, collateral circulation is diverted primarily via the internal mammary, vertebral, azygous, hemiazygous, lateral thoracic, and thoracoepigastric veins.
William Hunter first described SVCS in 1757, in a patient with syphilitic saccular aortic aneurysm. Before 1949, malignant tumors were estimated to cause only one third of cases of SVCS. In the pre-antibiotic era, many cases of SVCS were attributed to infectious causes.
Malignant causes may have been underrecognized because of the lack of sophisticated imaging technology. Primary intrathoracic malignancy is now responsible for most cases of SVCS.
Treatment of SVCS has 2 objectives—relieving symptoms and managing the underlying cause. Obtaining a histological diagnosis before treatment is important. Without a histological diagnosis, treatment should be initiated only in patients with rapidly progressive, life-threatening symptoms (cerebral or laryngeal edema) or in patients in whom multiple attempts to determine an underlying diagnosis have failed.
Radiation therapy is generally well tolerated, with the most common complication being esophagitis. Chemotherapy may yield a rapid response in patients with lymphoma; however, response to chemotherapy in patients with other tumors is likely to be delayed. Adjunctive therapy for symptomatic SVCS can include high-dose corticosteroids, but this type of treatment has adverse effects and may predispose to infection. Other adjunctive treatments include oxygen and elevation of the head of the patient's bed.
Recurrent superior vena caval obstruction after radiation therapy occurs in 10% to 32% of patients. Treatment options for recurrent malignant superior vena caval obstruction include balloon angioplasty, intravascular stenting, surgical bypass, or further radiation or chemotherapy.
Balloon angioplasty alone has a high rate of recurrence of superior vena caval obstruction, but it may be combined with intravascular stenting for improved results. Symptoms of superior vena caval obstruction generally improve within days to weeks after stenting. The role of anticoagulation after stenting is not clearly defined.
Complications of stenting include stent fracture or migration, local vessel trauma, and bleeding secondary to concurrent thrombolytics or anticoagulation.
Stenting of the superior vena cava is recommended in patients with recurrent malignant superior vena caval obstruction in whom conventional therapy (radiation or chemotherapy) has failed or in those who require urgent relief of symptoms. If stenting is not feasible or SVCS is secondary to benign disease, surgical bypass of the obstruction may be performed via thoracotomy, sternotomy, or subcutaneous jugular-femoral bypass.
In conclusion, this case illustrates several important points. First, the diagnosis of SVCS is primarily a clinical one that can usually be made based on the history and physical examination findings. Second, initial adjunctive studies of choice are chest radiography and chest CT with intravenous contrast (or nuclear magnetic resonance imaging). Third, the most likely underlying cause of SVCS is malignancy, usually bronchogenic cancer. However, a histopathologic diagnosis should be confirmed before radiation therapy is administered. The role of corticosteroids is not clearly defined in management of acute SVCS, and any potential benefits (which have not been clearly proved) should be weighed against risks such as immunosuppression. Finally, the prognosis of SVCS is primarily related to the prognosis of its underlying cause, and therapy should be based on the underlying disease.
References
Chen JC
Bongard F
Klein SR
A contemporary perspective on superior vena cava syndrome.
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