Primary or metastatic cancer in the upper lobe of the right lung can compress the superior vena cava. Superior vena cava syndrome in thoracic malignancies. The obstruction of blood flow through the superior vena cava svc into the right atrium may present as a severe clinical syndrome. Differential diagnoses of malignant superior vena cava syndrome. Superior vena cava syndrome an overview sciencedirect topics. 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. Superior vena cava syndrome caused by a malignant tumor. 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 insidious onset and slow progression of symptoms allow for development of an efficient collateral venous circulation compatible with longterm survival. Jun 01, 2018 tumor lysis syndrome is a metabolic emergency that presents as severe electrolyte abnormalities. 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. 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. Detterbeck, joachim yahalom a 58yearold man presents with a 2week history of progressive. Know what is superior vena cava syndrome, its causes, symptoms, treatment and prognosis.
The treatment of the benign svc syndrome is focused on achieving longterm durability and patency of the superior. The superior vena cava syndrome caused by malignant. Symptoms include swelling of the face and arms associated with shortness of breath. Symptoms include facial edema, dyspnea, cough, neck distension, hoarseness and dysphagia, while severe cases may present with coma and severe respiratory distress. It was first described by william hunter in 1757, in a patient with a large syphilitic aortic aneurysm compressing the svc3,5.
Cancer is associated with an increased risk of thromboembolism that varies according to patient, tumor, and treatmentrelated factors. 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. Superior vena cava svc syndrome occurs in approximately 15,000 people in the united states each year. 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. Svc compression can be caused by tumor masses in the middle or anterior mediastinum.
Treatment of malignant superior vena cava obstruction. Right sided masses are more likely to cause svcs, due to the anatomic location 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. Svcs can also be caused by a blood clot in the vein. Surgical treatment of benign superior vena cava syndrome. Benign superior vena cava svc obstruction is an uncommon entity. Currently, svc syndrome is generally due to cancer or thrombotic events. 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 svcs is a relatively common sequela. Superior vena cava syndrome symptoms, diagnosis and. 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. Sao paolo, 1999 brazil abstract neoadjuvant chemotherapy vs radiotherapy alone for superior vena cava syndrome svcs due to nonsmall cell lung cancer nsclc. Superior vena cava syndromecausessymptomstreatment. Superior vena cava syndrome with malignant causes nejm.
Epidemiology svcs develops in about 510% of patients with a malignant intrathoracic lesion 6. Review of evolving etiologies, implications and treatment strategies. However, it is important to recognize that a small percentage of svc syndromes are due to benign diseases such as mediastinal granulomas. Sep 01, 2017 tumors in the chest may press on or grow into the superior vena cava. Superior vena cava endoprostheses have proven effective for treating benign causes of superior vena cava obstruction, even in the presence of a cardiac pacemaker. Superior vena cava syndrome svcs is an obstructive emergency. Pleural fluid sample demonstrating milky, pink fluid.
Superior vena cava syndromea journal of thoracic oncology. Superior vena cava syndrome svcs occurs when a persons superior vena cava is partially blocked or compressed. Superior vena cava syndrome definition of superior vena. Superior vena cava syndrome history and exam bmj best. Malignancy is the most common cause of this syndrome. In some patients, treatment of the superior vena cava syndrome and their malignant conditions results in the cure of both. Mar 27, 2020 superior vena cava syndrome svcs is obstruction of blood flow through the superior vena cava svc. Sep 28, 2018 superior vena cava syndrome is a pathological condition in which the superior vena cava gets obstructed or impinged. Nonmalignant superior vena cava syndrome in a patient with. Superior vena cava svc obstruction is often caused by a malignant process such as bronchogenic carcinoma or lymphoma. Svcs is an emergency requiring immediate diagnosis and treatment. Nonmalignant svcs is typically caused by infectious etiologies or by. Nonmalignant causes can include aneurysm of the aorta, thromboses after implanted intravascular catheters or fibrosing mediastinitis.
The management of svc syndrome rests on the severity of symptoms, the cause of the obstruction, and the histological type of the tumor. The resulting increased venous pressure in the upper body may cause edema of the head, neck, and upper. 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. Superior vena cava syndrome is a form of vessel obstruction that occurs as a result of mechanical compression or due to thrombosis. 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. With the increasing use of intravascular devices, the incidence of the svc syndrome arising from benign etiologies is increasing. 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.
Although rarely fatal, may sometimes present as lifethreatening upper airway o. It is a medical emergency and most often manifests in patients with a malignant disease process within the thorax. 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. 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. 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. Radiation oncologypalliationsvc syndrome wikibooks, open. Malignant obstruction can be caused by direct invasion of tumor into the svc, or by external compression of the svc by an adjacent pathologic. Diagnosis superior vena cava syndrome due to thrombotic obstructiona complication of a cardiac procedure. What are the malignant causes of superior vena cava syndrome. Superior vena cava syndromea proposed classification. Ct of the chest axial image demonstrating moderate to severe narrowing of the pulmonary artery trunk due to compression from mediastinal adenopathy.
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. The superior vena cava is one of the primary veins within our bodies. Multimodality treatment of malignant superior vena caval. Svcs is a medical emergency if associated with laryngeal or cerebral edema. 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. Malignancy is the most common cause of the superior vena cava svc syndrome. Detterbeck, joachim yahalom a 58yearold man presents with a. Mid and longterm followup experience in patients with. 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 etiology bmj best practice. Malignant causes represents the majority of all cases of svcs. Thrombectomy, thrombosis, superior vena cava syndrome, superior vena cava obstruction doi. 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.
Mar 21, 2020 adelstein dj, hines jd, carter sg, et al. Hypercalcemia of malignancy, which is associated with poor outcomes. Lung cancer, particularly masses located in the upper lobe of the right lung, can impede blood flow. The syndrome was originally described as being secondary to an infection, such as tuberculosis, or a syphilitic aortic aneurysm 24. The majority of cases of superior vena cava syndrome arises with malignancies in the mediastinum. Superior vena cava syndrome svcs is obstruction of blood flow through the superior vena cava svc. 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 most common malignant cause of svc syndrome is bronchogenic carcinoma lung cancer and the most common type of small cell carcinoma. 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. Clinical condition that occurs due to obstruction of the superior vena cava.
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. There can be many causes, including lung cancer, metastatic breast cancer, and nonhodgkins lymphoma. In the case of a malignancy causing svc obstruction, management issues. The primary reason behind this compression or impingement is believed to be malignant tumors especially of the lungs. 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. An individualized clinical approach is important to pursue the accurate diagnosis of the. Superior vena cava syndrome svcs results from compression of the superior vena cava. In the case of a malignancy causing svc obstruction, management issues include treatment of the. Request pdf superior vena cava syndrome with malignant causes. Superior vena cava syndrome american journal of medicine.
Superior vena cava syndromea proposed classification system. Stent implantation for superior vena cava syndrome of. 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. 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. 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. Feb 29, 2016 superior vena cava syndrome svcs is a relatively common sequela of mediastinal malignancies and may cause significant patient distress. Non malignant svcs is typically caused by infectious etiologies or by. A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other conditions. It carries blood from the head, neck, upper chest, and arms to the heart. The other major malignant cause, although much less common, is nonhodgkins lymphoma. Superior vena cava syndromecausessymptomstreatmentprognosis. A contemporary perspective on superior vena cava syndrome.
Thromboembolic events in patients with malignant superior vena cava syndrome and the role of anticoagulation. Superior vena cava svc syndrome results from the obstruction of blood flow through the svc, having distinct pathophysiological underlying mechanisms. Superior vena cava syndrome epidemiology bmj best practice. Although the exact incidence of superior vena cava syndrome remains unknown, the condition affects about 15 000 patients in the united states each year. Clinical practice from the new england journal of medicine superior vena cava syndrome with malignant causes. 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. In superior vena cava syndrome svcs, this vein has.
The diagnosis can be made clinically, but imaging studies are recommended for confirmation. The superior vena cava is a major vein in a persons body. A restriction of the blood flow occlusion through this vein can cause superior vena cava syndrome svcs. Early case series reported about equal distribution of cases due to aortic aneurysms and thoracic neoplasms 9,14.
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 drawing below shows where the superior vena cava is located in the body. The superior vena cava svc obstruction by malignant diseases is either by direct invasion and compression or by tumour thrombus of the svc. Although rarely fatal, may sometimes present as lifethreatening upper airway ob. The severity of the signs and symptoms presented depends on the. 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. 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. In these instances, svcs results due to the obstruction of the superior vena cava by a tumor or thrombosis. It transports blood from our arms, chest, neck, and head to our hearts. A 58yearold man presents with a 2week history of progressive dyspnea on exertion, neck swelling, decreased appetite, and fatigue. It is considered an ominous complication of the malignancy.
Pdf 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. The most common cause of superior vena cava syndrome is cancer. Obstruction of the svc in the pediatric age group is rare and has a different etiologic spectrum. Most data regarding management of the superior vena cava syndrome are from case series. 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. Stabilization is focused on vigorous rehydration, maintaining urine output, and lowering uric acid levels. Malignant causes of svc syndrome include advanced lung cancer 67%, mediastinal tumors 20%, and metastatic solid malignancy 5%. 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. The difficulties of a randomized study in superior vena caval obstruction.
Superior vena cava syndrome comprises various symptoms and signs resulting from the obstruction of the superior vena cava and. Review of evolving etiologies, implications and treatment. Superior vena cava obstruction evaluation with mdct. Superior vena cava syndrome with malignant causes request pdf. The etiologies and management of svcs have evolved over time. 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.
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