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Emergencies in patients with cancer may be classified into three groups: pressure or obstruction caused by a space-occupying lesion, metabolic or hormonal problems (paraneoplastic syndromes, Chap. 100), and treatment-related complications.

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Superior Vena Cava Syndrome

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Superior vena cava syndrome (SVCS) is the clinical manifestation of superior vena cava (SVC) obstruction, with severe reduction in venous return from the head, neck, and upper extremities. Malignant tumors, such as lung cancer, lymphoma, and metastatic tumors, are responsible for the majority of SVCS cases. With the expanding use of intravascular devices (e.g., permanent central venous access catheters, pacemaker/defibrillator leads), the prevalence of benign causes of SVCS is increasing now, accounting for at least 40% of cases. Lung cancer, particularly of small cell and squamous cell histologies, accounts for approximately 85% of all cases of malignant origin. In young adults, malignant lymphoma is a leading cause of SVCS. Hodgkin's lymphoma involves the mediastinum more commonly than other lymphomas but rarely causes SVCS. When SVCS is noted in a young man with a mediastinal mass, the differential diagnosis is lymphoma vs primary mediastinal germ cell tumor. Metastatic cancers to the mediastinum, such as testicular and breast carcinomas, account for a small proportion of cases. Other causes include benign tumors, aortic aneurysm, thyromegaly, thrombosis, and fibrosing mediastinitis from prior irradiation, histoplasmosis, or Behçet's syndrome. SVCS as the initial manifestation of Behçet's syndrome may be due to inflammation of the SVC associated with thrombosis.

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Patients with SVCS usually present with neck and facial swelling (especially around the eyes), dyspnea, and cough. Other symptoms include hoarseness, tongue swelling, headaches, nasal congestion, epistaxis, hemoptysis, dysphagia, pain, dizziness, syncope, and lethargy. Bending forward or lying down may aggravate the symptoms. The characteristic physical findings are dilated neck veins; an increased number of collateral veins covering the anterior chest wall; cyanosis; and edema of the face, arms, and chest. More severe cases include proptosis, glossal and laryngeal edema, and obtundation. The clinical picture is milder if the obstruction is located above the azygos vein. Symptoms are usually progressive, but in some cases they may improve as collateral circulation develops.

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Signs and symptoms of cerebral and/or laryngeal edema, though rare, are associated with a poorer prognosis and require urgent evaluation. Seizures are more likely related to brain metastases than to cerebral edema from venous occlusion. Patients with small cell lung cancer and SVCS have a higher incidence of brain metastases than those without SVCS.

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Cardiorespiratory symptoms at rest, particularly with positional changes, suggest significant airway and vascular obstruction and limited physiologic reserve. Cardiac arrest or respiratory failure can occur, particularly in patients receiving sedatives or undergoing general anesthesia.

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Rarely, esophageal varices may develop. These are "downhill" varices based on the direction of blood flow from cephalad to caudad (in contrast to "uphill" varices associated with caudad to cephalad flow from portal hypertension). If the obstruction to the SVC is proximal ...

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