Obstruction of the superior vena cava svc, a thinwalled vessel that carries blood at low pressure, may result from compression by tumor or intraluminal thrombus formation. Covered stent placement for the treatment of malignant. An unusual case of superior vena cava syndrome caused by. In this manuscript, we describe an extremely rare case of a transudative chylothorax secondary to superior vena cava syndrome svc as well as a description of five previously reported cases of transudative chylothoraces associated with svc syndrome published in the english literature between 1960 and 2018 via a medline search using the search terms. The majority of cases of superior vena cava svc syndrome result from neoplasm, particularly lung cancer. Superior vena cava syndrome svcs is a relatively common sequela of mediastinal malignancies and may cause significant patient distress. Nonmalignant superior vena cava syndrome in a patient. Bilyeu, md approximately 15,000 cases of superior vena cava svc obstruction are diagnosed in the united states annually. Superior vena cava svc syndrome is a collection of clinical signs. Clinical findings in a patient with the superior vena cava syndrome, including facial edema, plethora, jugular venous distention, and prominent superficial vascularity of neck and upper chest, are shown in panel a. Superior vena cava obstruction evaluation with mdct. Although the exact incidence of superior vena cava syndrome remains unknown, the condition affects about 15 000 patients in the united states each year.
Treatment of malignant superior vena cava syndrome by endovascular stent insertion. Its diagnosis is symptomsbased, the most common symptom being dyspnea, along with findings on. Clinical practice from the new england journal of medicine superior vena cava syndrome with malignant causes. Malignant obstructions of the inferior vena cava ivc are a common cause of the ivc syndrome. Endovascular treatment of malignant superior vena cava. A reassessment of the clinical applications of the superior vena cava syndrome. Superior vena cava syndrome is a pathological condition in which the superior vena cava gets obstructed or impinged. In superior vena cava syndrome svcs, this vein has. Today, this syndrome is most commonly seen secondary to malignancy although. It has been reported 3 that svc syndrome occurs in approximately 4% of lung cancer patients at diagnosis and may develop during the disease course. Korean j radiol 151, janfeb 2014 87 introduction superior vena cava svc syndrome is a common covered stent placement for the treatment of malignant superior vena cava syndrome. Stent implantation for superior vena cava syndrome of.
In case of a malignant cause, superior vena cava syndrome paraschiv et al 42 vol. In over 90% of cases, superior vena cava syndrome is the result of a malignant tumor that is causing displacement or is growing in an infiltrative manner in the thorax. Superior vena cava syndrome in thoracic malignancies. Superior vena cava syndrome case history bmj best practice. Detterbeck, joachim yahalom a 58yearold man presents with a. Endovascular treatment of superior vena cava syndrome. Superior vena cava syndrome svcs entails severe symptoms due to blood flow obstruction of the superior vena cava svc towards the right auricle, caused by either extrinsic compression or invasion of the vena cava. Although thymomas are wellknown causes of svcs, cases of svcs caused by malignant thymomas protruding into adjacent vessels draining the svc with thrombosis have. Lymphoma, either hodgkin disease or nonhodgkin lymphoma, tends to compress the vessel. The primary reason behind this compression or impingement is believed to be malignant tumors especially of the lungs. Malignancies primarily lung cancer are the underlying cause of 8085% of cases, leaving 1520% caused by. A 58yearold man presents with a 2week history of progressive dyspnea on exertion, neck swelling, decreased appetite, and fatigue.
Thrombosis of the superior vena cava, however, is less common when it is not related to central venous catheters in the subclavian vein or the superior vena cava. The superior vena cava svc obstruction by malignant diseases is either by direct invasion and compression or by tumour thrombus of the svc. Malignant obstruction can be caused by direct invasion of tumor into the svc, or by external compression of the svc by an adjacent pathologic. Malignant thrombosis of the superior vena cava caused by. The syndrome is almost invariably secondary to a malignant process. The management of svc syndrome rests on the severity of symptoms, the cause of the obstruction, and the histological type of the tumor. Superior vena cava syndrome can be diagnosed on physical examination alone, but studies must be performed in order to establish a cause. Pact paclitaxel coated balloon omer iftikhar 1, eileen gajo, westby fisher, jonathan gilbert1 and michael h. Superior vena cava syndrome in thoracic malignancies philipp m lepper md, sebastian r ott md, hanno hoppe md. At present, 35% of svc obstructions are caused by the increased use of intravascular devices such as catheters and pacemaker wires. Review of evolving etiologies, implications and treatment. Endovascular management of malignant inferior vena cava. Superior vena cava syndrome with malignant causes nejm.
Pdf diagnostics and treatment of superior vena cava. Tbe svc syndrome that ensues may be the cause of significant morbidity. Thrombectomy, thrombosis, superior vena cava syndrome, superior vena cava obstruction doi. Obstruction of the superior vena cava svc causes symptoms that commonly include a. Superior vena cava syndrome svcs is a constellation of clinical symptoms and signs caused by either extrinsic compression or svc thrombosis. Know what is superior vena cava syndrome, its causes, symptoms, treatment and prognosis. In the current study, the authors report an unusual case in which the initial presentation of the alcl was that of superior vena cava svc syndrome.
Symptoms of the syndrome include shortness of breath. Although rarely fatal, may sometimes present as lifethreatening upper airway o. Blood clot thrombus formation that causes superior vena cava syndrome is a complication of pacemaker wires,dialysis, and other intravenous catheters that are threaded into the superior vena cava. Superior vena cava syndrome secondary to an angiotropic. Small cell lung cancer and non hodgkin lymphoma are the most common culprits, though intravascular devices. Superior vena cava syndrome symptoms, diagnosis and.
Superior vena cava syndrome svcs occurs when a persons superior vena cava is partially blocked or compressed. When vessel compression is associated with tracheal compression, it is termed superior mediastinal syndrome sms. Advances in knowledge n use of endovascular expanded polytetrafluoroethylene eptfecovered stents appears to be a safe and effective method to treat patients with malignant superior vena cava syndrome. Superior vena cava syndrome svcs, is a group of symptoms caused by obstruction of the superior vena cava svc, a short, wide vessel carrying circulating blood into the heart. Malignancyrelated superior vena cava syndrome uptodate.
Salinger1 1department of cardiology, northshore university health system, usa case report american journal of cardiovascular and thoracic surgery open access. Historically, and in other parts of the world, infection syphilis and tuberculosis is another cause of superior vena cava syndrome. The superior vena cava is one of the primary veins within our bodies. Superior vena cava syndrome possibilities of intervention therapy beran s. Pdf superior vena cava syndrome with malignant causes. Percutaneous management of non malignant superior vena.
Request pdf superior vena cava syndrome with malignant causes a 58yearold man presents with a 2week history of progressive dyspnea on exertion, neck swelling, decreased appetite, and fatigue. Superior vena cava syndrome is diagnosed by ultrasound, chest xray, ct scan, and in some cases biopsy. Superior vena cava syndrome is caused by gradual compression of the superior vena cava where it enters the right. Superior vena cava syndromecausessymptomstreatment. Whatever is its cause, obstruction of the svc causes elevated pressure in the veins draining into the svc and increased or reversed blood flow through collateral vessels. Small cell lung cancer and nonhodgkin lymphoma are the most common culprits, though intravascular devices. Lung cancer, either nonsmall cell or small cell cancer, can directly invade into the superior vena cava. Review of evolving etiologies, implications and treatment strategies. Our patient underwent a chest ct scan to assess the superior vena cava syndrome, which is caused by malignant conditions, such as lung cancer or lymphoma in more than 90% of cases. Later, malignant diseases were the predominant cause of svc obstruction, in 90% of the cases. Superior vena cava syndrome svcs, a clinical manifestation arising from compression of the thinwalled superior vena cava svc, was first described by william hunter in 1757 and can be caused by a variety malignancies hunter and johnston 1757. A transudative chylothorax associated with superior vena. Infections such as tuberculosis, and extrinsic compression such as syphilitic aortic aneurysm, thymoma or thyroid goitre, are also known to cause superior vena cava syndrome.
Svcs is a significant disorder affecting up to 10 % of small cell lung cancer sclc patients and 24 % of all lung cancer patients. Lung cancer, including both nonsmall cell lung cancer and small cell lung cancer, is the most common extrinsic cause of superior vena cava syndrome. Percutaneous management of non malignant superior vena cava syndrome using in. Causes of superior vena cava include lung cancer, lymphoma, other cancers in the chest, blood clots in the superior vena cava, or. We present an uncommon case of malignant svcs presenting with syncope. Diagnostics and treatment of superior vena cava syndrome caused by malignant tumours. Superior vena cava syndrome is a form of vessel obstruction that occurs as a result of mechanical compression or due to thrombosis. To demonstrate the effectiveness of endovascular treatment evt with selfexpandable bare stents for malignant superior vena cava syndrome svcs and to analyze predictive factors of evt efficacy. Superior vena cava svc syndrome is a complication that occurs when malignancies, particularly in advanced lung cancer and mediastinal tumors, cause obstruction to the svc 1, 2. Hepatocellular carcinoma hepatocellular carcinoma usually invades the portal venous system, but the invasion of the inferior vena cava and the suprahepatics may occur in up to 4% of patients 11. Here, for the first time as far as we are aware, we report the case of a nonsmallcell lung cancer patient with a massive svc malignant thrombosis who was treated with thoracic irradiation and erlotinib. In most published studies, cancer is the most common underlying cause of superior vena cava obstruction. An intrathoracic malignancy is responsible for 60 to 85 percent of cases of superior vena cava syndrome. The majority of cases are caused by malignant tumors within the mediastinum, most commonly lung cancer and nonhodgkins lymphoma, directly compressing or invading the svc wall.
The medical care includes anticoagulants, glucocorticoids, diuretics, as well as radio and chemotherapy. About 50 years ago, the etiology of most superior vena cava svc obstructions was infectious in nature. Approach to the patient who presents with superior vena. The superior vena cava syndrome svcs comprises various symptoms due to occlusion of the svc, which can be easily obstructed by pathological conditions eg, lung cancer, due to the low internal venous pressure within rigid structures of the thorax trachea, right bronchus, aorta. The majority of cases are caused by malignant tumors within the mediastinum. Most cases of superior vena cava svc syndrome resulting from neoplasm, especially from lung cancer, remain a serious challenge to treat. Superior vena cava syndrome that develops when the superior vena cava is displaced or stenosed is now one of the main application areas in the venous system. Superior vena cava syndrome svcs is an acute or subacute oncologic emergency with typical clinical features. Symptoms include facial edema, dyspnea, cough, neck distension, hoarseness and dysphagia, while severe cases may present with coma and severe respiratory distress.
Clinical condition that occurs due to obstruction of the superior vena cava. The resulting increased venous pressure in the upper body may cause edema of the head, neck, and upper. As reports on interventional treatment of malignant inferior caval obstructions are very sparse, the purpose of this study was to retrospectively assess the outcome of endovascular treatment of symptomatic, malignant ivc syndromes. Approach to the patient who presents whh superior vena cava obstruction anthony abner, m. It transports blood from our arms, chest, neck, and head to our hearts. Superior vena cava syndrome statpearls ncbi bookshelf. In superior vena cava syndrome svcs, this vein has become obstructed or compressed to some degree. A case of malignant superior vena cava syndrome presenting. Superior vena cava svc syndrome is a clinical condition that occurs as a result of obstruction of the svc, leading to interrupted venous return from the head, thorax, and upper extremities to the right atrium. Nonmalignant causes can include aneurysm of the aorta, thromboses after implanted intravascular catheters or fibrosing mediastinitis. The superior vena cava syndrome caused by malignant. Angiotropic large cell lymphoma alcl is characterized by the intravascular proliferation of malignant lymphoid cells in small and medium.
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