Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome

SpringerPlus, Mar 2016

Superior vena cava syndrome (SVCS) is a relatively common sequela of mediastinal malignancies and may cause significant patient distress. SVCS is a medical emergency if associated with laryngeal or cerebral edema. The etiologies and management of SVCS have evolved over time. Non-malignant SVCS is typically caused by infectious etiologies or by thrombus in the superior vena cava and can be managed with antibiotics or anti-coagulation therapy, respectively. Radiation therapy (RT) has long been a mainstay of treatment of malignant SVCS. Chemotherapy has also been used to manage SVCS. In the past 20 years, percutaneous stenting of the superior vena cava has emerged as a viable option for SVCS symptom palliation. RT and chemotherapy are still the only modalities that can provide curative treatment for underlying malignant etiologies of SVCS. The first experiences with treating SVCS with RT were reported in the 1970’s, and several advances in RT delivery have subsequently occurred. Hypo-fractionated RT has the potential to be a more convenient therapy for patients and may provide equal or superior control of underlying malignancies. RT may be combined with stenting and/or chemotherapy to provide both immediate symptom palliation and long-term disease control. Clinicians should tailor therapy on a case-by-case basis. Multi-disciplinary care will maximize treatment expediency and efficacy.

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Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome

Straka et al. SpringerPlus Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome Christopher Straka 2 James Ying 2 Feng‑Ming Kong 1 Christopher D. Willey 4 Joseph Kaminski 3 D. W. Nathan Kim 0 0 Department of Radiation Oncology , Texas Oncology, 1700 W. Highway 6, Waco, TX 76712 , USA 1 Department of Radiation Oncology, GRU Cancer Center and Medical College of Georgia , Augusta, GA , USA 2 Department of Radiation Oncology, University of Texas Southwestern Medical Center , 5801 Forest Park Rd, Dallas, TX 75390 , USA 3 Dattoli Cancer Center , 2803 Fruitville Rd, Sarasota, FL 34237 , USA 4 Department of Radiation Oncology, The University of Alabama Birmingham , Birmingham, AL , USA Superior vena cava syndrome (SVCS) is a relatively common sequela of mediastinal malignancies and may cause significant patient distress. SVCS is a medical emergency if associated with laryngeal or cerebral edema. The etiologies and management of SVCS have evolved over time. Non‑ malignant SVCS is typically caused by infectious etiologies or by thrombus in the superior vena cava and can be managed with antibiotics or anti‑ coagulation therapy, respectively. Radiation therapy (RT) has long been a mainstay of treatment of malignant SVCS. Chemotherapy has also been used to manage SVCS. In the past 20 years, percutaneous stenting of the superior vena cava has emerged as a viable option for SVCS symptom palliation. RT and chemotherapy are still the only modalities that can provide curative treatment for underlying malignant etiologies of SVCS. The first experiences with treating SVCS with RT were reported in the 1970's, and several advances in RT delivery have subsequently occurred. Hypo‑ fractionated RT has the potential to be a more convenient therapy for patients and may provide equal or superior control of underlying malignancies. RT may be combined with stenting and/or chemotherapy to provide both immediate symptom palliation and long‑ term disease control. Clinicians should tailor therapy on a case‑ by‑ case basis. Multi‑ disciplinary care will maximize treatment expediency and efficacy. Superior vena cava syndrome (SVC syndrome; SVCS); Stenting; Thoracic malignancies; Hypo‑ fractionation; Multi‑ modality therapy Background Superior vena cava syndrome (SVCS), a clinical manifestation arising from compression of the thin-walled superior vena cava (SVC), was first described by William Hunter in 1757 and can be caused by a variety malignancies (Hunter and Johnston 1757) . SVCS is a significant disorder affecting up to 10  % of small cell lung cancer (SCLC) patients and 2–4  % of all lung cancer patients (Wan and Bezjak 2010) . The prognosis of SVCS caused by malignancies is primarily determined by the tumor type (Yu et al. 2008) . Traditionally, malignant SVCS has been considered to be an indication for emergent intervention, typically with radiation therapy (RT). However, accumulating evidence has suggested that accurate diagnosis and biopsy should precede emergent therapeutic intervention in most cases (Yu et  al. 2008) . Timely and effective intervention aimed at treating the malignant cause of this syndrome can relieve significant suffering and improve SVCS patients’ quality of life. Anatomy and physiology The SVC carries approximately one-third of the venous return to the heart. Situated slightly to the right of midline and coursing through the superior and middle mediastinum, the SVC is the major drainage outlet for venous return from the head, arms, and upper torso. Despite its high flow volume, the SVC is quite distensible and can be compressed by an adjacent mass originating in the middle or anterior mediastinum, the right paratracheal or © 2016 Straka et al. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. precarinal lymph node stations, or the right lobe bronchus (Wan and Bezjak 2010; Wilson et  al. 2007) . Rightsided masses are more likely to cause SVCS, presumably due to the anatomic location of the SVC (Sculier et  al. 1986) . Rarely, a thrombus can occlude the SVC even in the absence of an external mass. The superior and middle mediastinum is an anatomically confined space populated by a large number of lymph nodes. The SVC is thin walled and is opened by relatively low venous pressure, making it particularly susceptible to compression by adjacent masses (Koetters 2012) . In the event of SVC obstruction, venous pressure in collateral vessels increases and, over time, a collateral blood-flow network develops (Lacout et  al. 2012) . Many different vessels may enlarge in response to the increased blood pressure (...truncated)


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Christopher Straka, James Ying, Feng-Ming Kong, Christopher D. Willey, Joseph Kaminski, D. W. Nathan Kim. Review of evolving etiologies, implications and treatment strategies for the superior vena cava syndrome, SpringerPlus, 2016, pp. 229, Volume 5, Issue 1, DOI: 10.1186/s40064-016-1900-7