Stent implantation for superior vena cava syndrome of. Superior vena cava syndrome in thoracic malignancies. Svcs is a medical emergency if associated with laryngeal or cerebral edema. The etiologies and management of svcs have evolved over time. It transports blood from our arms, chest, neck, and head to our hearts. In these instances, svcs results due to the obstruction of the superior vena cava by a tumor or thrombosis. Superior vena cava syndrome svcs is obstruction of blood flow through the superior vena cava svc. Hypercalcemia of malignancy, which is associated with poor outcomes. Review of evolving etiologies, implications and treatment strategies. Malignant causes of svc syndrome include advanced lung cancer 67%, mediastinal tumors 20%, and metastatic solid malignancy 5%. 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.
Nonmalignant svcs is typically caused by infectious etiologies or by. Primary or metastatic cancer in the upper lobe of the right lung can compress the superior vena cava. Although rarely fatal, may sometimes present as lifethreatening upper airway ob. Endovascular today issn 15511944 print and issn 2689792x online is a publication dedicated to bringing you comprehensive coverage of all the latest technology, techniques, and developments in the endovascular field. Lymphoma or other tumors located in the mediastinum can also cause compression of the superior vena cava less often, the superior vena cava can become blocked with a blood clot from within. 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. Thromboembolic events in patients with malignant superior vena cava syndrome and the role of anticoagulation. Obstruction of the svc in the pediatric age group is rare and has a different etiologic spectrum.
There can be many causes, including lung cancer, metastatic breast cancer, and nonhodgkins lymphoma. Superior vena cava syndrome in thoracic malignancies philipp m lepper md, sebastian r ott md, hanno hoppe md, christian schumann md, uz stammberger md, antonio bugalho md, steffen frese md, michael schmu. May 03, 2007 superior vena cava syndrome with malignant causes. Svcs is a significant disorder affecting up to 10 % of small cell lung cancer sclc patients and 24 % of all lung cancer patients. It is considered an ominous complication of the malignancy. A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other conditions. In some patients, treatment of the superior vena cava syndrome and their malignant conditions results in the cure of both. 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. Treatment of malignant superior vena cava obstruction. Malignancy is the most common cause of this syndrome.
Superior vena cava svc syndrome results from the obstruction of blood flow through the svc, having distinct pathophysiological underlying mechanisms. The treatment of the benign svc syndrome is focused on achieving longterm durability and patency of the superior. Apr 05, 2020 the cause of the disease is thought to be autoimmune in nature and heavily linked to tobacco use in patients with buergers as primary disease. Superior vena cava syndrome is a form of vessel obstruction that occurs as a result of mechanical compression or due to thrombosis. Cardiac 1 atrial myxomas 2 intrapericardial band 3 pericarditist. Superior vena cava endoprostheses have proven effective for treating benign causes of superior vena cava obstruction, even in the presence of a cardiac pacemaker. Feb 03, 2009 although the exact incidence of superior vena cava syndrome remains unknown, the condition affects about 15 000 patients in the united states each year. The superior vena cava is one of the primary veins within our bodies. Superior vena cava syndrome svcs occurs when a persons superior vena cava is partially blocked or compressed. The superior vena cava syndrome svcs involves a group of symptoms deriving from obstruction or compression of the superior vena cava. Superior vena cava syndrome svcs results from compression of the superior vena cava. Superior vena cava syndrome is caused by gradual compression of the superior vena cava where it enters the right atrium, leading to edema and retrograde flow. Superior vena cava syndromea journal of thoracic oncology. Superior vena cava syndrome definition of superior vena.
Know what is superior vena cava syndrome, its causes, symptoms, treatment and prognosis. Cancer is associated with an increased risk of thromboembolism that varies according to patient, tumor, and treatmentrelated factors. 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. Thrombectomy, thrombosis, superior vena cava syndrome, superior vena cava obstruction doi. Superior vena cava syndromecausessymptomstreatmentprognosis. In the case of a malignancy causing svc obstruction, management issues include treatment of the. Benign superior vena cava svc obstruction is an uncommon entity. Superior vena cava syndrome with malignant causes nejm. Superior vena cava syndrome svcs is a relatively common sequela.
Superior vena cava syndrome with malignant causes request pdf. The insidious onset and slow progression of symptoms allow for development of an efficient collateral venous circulation compatible with longterm survival. An intrathoracic malignancy is responsible for 60 to 85 percent of cases of superior vena cava syndrome svc syndrome, and svc obstruction is the presenting overview of the risk factors, pathology, and clinical manifestations of lung cancer view in chinese. The most common cause of superior vena cava syndrome is cancer. Superior vena cava syndrome svcs is mechanical obstruction of the superior vena cava svc due to venous thrombi or extrinsic compression by intrathoracic tumours in most cases. Surgical treatment of benign superior vena cava syndrome. What are the malignant causes of superior vena cava syndrome. Although the exact incidence of superior vena cava syndrome remains unknown, the condition affects about 15 000 patients in the united states each year. The management of svc syndrome rests on the severity of symptoms, the cause of the obstruction, and the histological type of the tumor. The most common malignant cause of svc syndrome is bronchogenic carcinoma lung cancer and the most common type of small cell carcinoma. Definition the superior vena cava is the major vein in the chest that carries blood from the upper part of the body in to the heart. Radiation oncologypalliationsvc syndrome wikibooks, open. It is a medical emergency and most often manifests in patients with a malignant disease process within the thorax. However, it is important to recognize that a small percentage of svc syndromes are due to benign diseases such as mediastinal granulomas.
Early case series reported about equal distribution of cases due to aortic aneurysms and thoracic neoplasms 9,14. The superior vena cava syndrome caused by malignant. Pdf superior vena cava syndrome with malignant causes. Most data regarding management of the superior vena cava syndrome are from case series. It carries blood from the head, neck, upper chest, and arms to the heart. Superior vena cava syndrome an overview sciencedirect. First reported by hunter on a patient with a large syphilitic ascending aortic aneurysm compressing on the superior vena cava and innominate vein, superior vena cava obstruction svco syndrome is the constellation of signs and symptoms of impaired venous drainage of the head and neck and upper extremities as a result of external compression. The syndrome was originally described as being secondary to an infection, such as tuberculosis, or a syphilitic aortic aneurysm 24. Nonmalignant superior vena cava syndrome in a patient with. Svc compression can be caused by tumor masses in the middle or anterior mediastinum. Superior vena cava syndrome epidemiology bmj best practice.
However, in a small but significant number of patients the obstruction results from a benign, nonmalignant process, and for these patients the prognosis is usually. Epidemiology svcs develops in about 510% of patients with a malignant intrathoracic lesion 6. Nonmalignant causes can include aneurysm of the aorta, thromboses after implanted intravascular catheters or fibrosing mediastinitis. Stabilization is focused on vigorous rehydration, maintaining urine output, and lowering uric acid levels. Mar 27, 2020 superior vena cava syndrome svcs is obstruction of blood flow through the superior vena cava svc. Mid and longterm followup experience in patients with. Superior vena cava syndrome symptoms, diagnosis and. The primary reason behind this compression or impingement is believed to be malignant tumors especially of the lungs. The severity of the signs and symptoms presented depends on the. Review of evolving etiologies, implications and treatment. Differential diagnoses of malignant superior vena cava syndrome. Detterbeck, joachim yahalom a 58yearold man presents with a. The superior vena cava svc obstruction by malignant diseases is either by direct invasion and compression or by tumour thrombus of the svc. 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.
Malignant obstruction can be caused by direct invasion of tumor into the svc, or by external compression of the svc by an adjacent pathologic. The resulting increased venous pressure in the upper body may cause edema of the head, neck, and upper. Detterbeck, joachim yahalom a 58yearold man presents with a 2week history of progressive. A contemporary perspective on superior vena cava syndrome. Request pdf superior vena cava syndrome with malignant causes. It was first described by william hunter in 1757, in a patient with a large syphilitic aortic aneurysm compressing the svc3,5. Superior vena cava svc obstruction is often caused by a malignant process such as bronchogenic carcinoma or lymphoma.
Diagnosis superior vena cava syndrome due to thrombotic obstructiona complication of a cardiac procedure. The superior vena cava is a major vein in a persons body. If the cancer spreads to the lymph nodes around the superior vena cava, they can enlarge and press on or cause a blockage in the vein. Sao paolo, 1999 brazil abstract neoadjuvant chemotherapy vs radiotherapy alone for superior vena cava syndrome svcs due to nonsmall cell lung cancer nsclc. Clinical practice from the new england journal of medicine superior vena cava syndrome with malignant causes. Superior vena cava syndrome is a pathological condition in which the superior vena cava gets obstructed or impinged. Superior vena cava syndrome affects 15,000 patients each year in the united states, and its incidence continues to grow as the prevalence of intravascular devices increases. 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. Right sided masses are more likely to cause svcs, due to the anatomic location of the svc. Feb 29, 2016 superior vena cava syndrome svcs is a relatively common sequela of mediastinal malignancies and may cause significant patient distress. A prospective study looking at the results of treating malignant superior vena cava obstruction by means of percutaneous selfexpanding stent insertion or by means of radiation therapy. With the increasing use of intravascular devices, the incidence of the svc syndrome arising from benign etiologies is increasing. The majority of cases of superior vena cava syndrome arises with malignancies in the mediastinum. Symptoms include facial edema, dyspnea, cough, neck distension, hoarseness and dysphagia, while severe cases may present with coma and severe respiratory distress.
Multimodality treatment of malignant superior vena caval. The drawing below shows where the superior vena cava is located in the body. One of the benign causes of svc obstruction is the longterm use of indwelling catheters and wires, increasing the chances of svc thrombosis. Superior vena cava syndrome is most often caused by compression of the vein the superior vena cava, that returns blood from the upper body back to the right atrium of the heart by tumor. 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.
Superior vena cava syndromea proposed classification. Superior vena cava syndrome comprises various symptoms and signs resulting from the obstruction of the superior vena cava and. Infectious causes especially syphilitic aortic aneurysm and tuberculosis accounted for the majority of cases 50 years ago but are now rare, especially in the developed countries. The difficulties of a randomized study in superior vena caval obstruction. 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. Lung cancer is the most common etiology, nonsmall cell lung cancer accounting for 50% of cases of malignant svc syndrome and small cell lung cancer for 25% cases. Malignancy is the most common cause of the superior vena cava svc syndrome.
Endoprostheses are also used to bypass malignant obstructions of the superior vena cava because they do not preclude further oncologic treatment with surgery or chemotherapy, and they. An individualized clinical approach is important to pursue the accurate diagnosis of the. These signs and symptoms together constitute the superior vena cava syndrome svc syndrome, first described by william hunter in 1757 as a complication of a saccular aortic aneurysm. The diagnosis can be made clinically, but imaging studies are recommended for confirmation. Non malignant svcs is typically caused by infectious etiologies or by. Superior vena cava syndromecausessymptomstreatment. Superior vena cava syndromea proposed classification system. Jun 01, 2018 tumor lysis syndrome is a metabolic emergency that presents as severe electrolyte abnormalities. Malignant causes represents the majority of all cases of svcs. A 58yearold man presents with a 2week history of progressive dyspnea on exertion, neck swelling, decreased appetite, and fatigue. Pleural fluid sample demonstrating milky, pink fluid. Lung cancer, including both nonsmall cell lung cancer and small cell lung cancer, is the most common extrinsic cause of superior vena cava syndrome.
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. Superior vena cava syndrome svcs is an obstructive emergency. Superior vena cava svc syndrome occurs in approximately 15,000 people in the united states each year. 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 can also be caused by a blood clot in the vein. Sep 28, 2018 superior vena cava syndrome is a pathological condition in which the superior vena cava gets obstructed or impinged. 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. Mar 21, 2020 adelstein dj, hines jd, carter sg, et al. Since some malignancies causing superior vena cava syndrome svcs are only sensitive to a specific treatment regimen, it is crucial to diagnose the underlying pathology in such cases. Superior vena cava syndrome american journal of medicine. Currently, svc syndrome is generally due to cancer or thrombotic events. Superior vena cava syndrome etiology bmj best practice.
Lung cancer, particularly masses located in the upper lobe of the right lung, can impede blood flow. Superior vena cava syndrome an overview sciencedirect topics. Symptoms include swelling of the face and arms associated with shortness of breath. Clinical condition that occurs due to obstruction of the superior vena cava.
Management of the superior vena cava syndrome associated with malignant conditions involves both treatment of the cancer and relief of the symptoms of obstruction. The other major malignant cause, although much less common, is nonhodgkins lymphoma. The obstruction of blood flow through the superior vena cava svc into the right atrium may present as a severe clinical syndrome. 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. Svcs is an emergency requiring immediate diagnosis and treatment. Description superior vena cava syndrome is a partial occlusion of the. Superior vena cava obstruction evaluation with mdct. Malignancyrelated superior vena cava syndrome uptodate. Superior vena cava syndrome caused by a malignant tumor. A restriction of the blood flow occlusion through this vein can cause superior vena cava syndrome svcs. Although rarely fatal, may sometimes present as lifethreatening upper airway o. Superior vena cava syndrome history and exam bmj best. In the case of a malignancy causing svc obstruction, management issues. Sep 01, 2017 tumors in the chest may press on or grow into the superior vena cava.
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