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Malignancy-related superior vena cava syndrome

INTRODUCTION

Superior vena cava (SVC) syndrome results from any condition that leads to obstruction of blood flow through the SVC. Obstruction can be caused by invasion or external compression of the SVC by adjacent pathologic processes involving the right lung, lymph nodes, and other mediastinal structures, or by thrombosis of blood within the SVC. In some cases, both external compression and thrombosis coexist [1].

In the preantibiotic era, syphilitic thoracic aortic aneurysms, fibrosing mediastinitis, and other complications of untreated infection were frequent causes of the SVC syndrome. In the postantibiotic era, malignancy became the most common cause, accounting for 90 percent of cases. More recently, the incidence of SVC syndrome due to thrombosis has risen, largely because of increased use of intravascular devices such as catheters and pacemaker wires. In the present era, benign causes now account for 20 to 40 percent of cases of SVC syndrome.

This review will focus on the etiology, clinical presentation, diagnosis, and treatment of SVC syndrome, emphasizing management in patients with malignancy. The management of SVC thrombosis in the setting of hemodialysis and other indwelling intravascular catheters in patients who do not have cancer is addressed elsewhere. (See "Thrombosis associated with chronic hemodialysis vascular access: Catheters" and "Central vein stenosis associated with dialysis access" and "Catheter-induced upper extremity venous thrombosis".)

PATHOPHYSIOLOGY

Obstruction of the SVC can be caused by invasion or external compression by adjacent pathologic processes involving the right lung, lymph nodes, or other mediastinal structures, or by thrombosis of blood within the SVC. As the flow of blood within the SVC becomes obstructed, venous collaterals form, establishing alternative pathways for the return of venous blood to the right atrium (figure 1). Collateral veins may arise from the azygos, internal mammary, lateral thoracic, paraspinous, and esophageal venous systems (image 1).

The venous collaterals dilate over several weeks. As a result, upper body venous pressure is markedly elevated initially but decreases over time. However, even when well-developed collateral drainage patterns are present, central venous pressures remain elevated, producing the characteristic signs and symptoms of SVC syndrome.

                                  

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Literature review current through: Apr 2013. | This topic last updated: Mar 21, 2013.
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References
Top
  1. García Mónaco R, Bertoni H, Pallota G, et al. Use of self-expanding vascular endoprostheses in superior vena cava syndrome. Eur J Cardiothorac Surg 2003; 24:208.
  2. Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med 2007; 356:1862.
  3. SCHECHTER MM. The superior vena cava syndrome. Am J Med Sci 1954; 227:46.
  4. Yellin A, Rosen A, Reichert N, Lieberman Y. Superior vena cava syndrome. The myth--the facts. Am Rev Respir Dis 1990; 141:1114.
  5. Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore) 2006; 85:37.
  6. Chee CE, Bjarnason H, Prasad A. Superior vena cava syndrome: an increasingly frequent complication of cardiac procedures. Nat Clin Pract Cardiovasc Med 2007; 4:226.
  7. Schraufnagel DE, Hill R, Leech JA, Pare JA. Superior vena caval obstruction. Is it a medical emergency? Am J Med 1981; 70:1169.
  8. Markman M. Diagnosis and management of superior vena cava syndrome. Cleve Clin J Med 1999; 66:59.
  9. Armstrong BA, Perez CA, Simpson JR, Hederman MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys 1987; 13:531.
  10. Salsali M, Cliffton EE. Superior vena caval obstruction in carcinoma of lung. N Y State J Med 1969; 69:2875.
  11. Rowell NP, Gleeson FV. Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic review. Clin Oncol (R Coll Radiol) 2002; 14:338.
  12. Dombernowsky P, Hansen HH. Combination chemotherapy in the management of superior vena caval obstruction in small-cell anaplastic carcinoma of the lung. Acta Med Scand 1978; 204:513.
  13. Spiro SG, Shah S, Harper PG, et al. Treatment of obstruction of the superior vena cava by combination chemotherapy with and without irradiation in small-cell carcinoma of the bronchus. Thorax 1983; 38:501.
  14. Sculier JP, Evans WK, Feld R, et al. Superior vena caval obstruction syndrome in small cell lung cancer. Cancer 1986; 57:847.
  15. Urban T, Lebeau B, Chastang C, et al. Superior vena cava syndrome in small-cell lung cancer. Arch Intern Med 1993; 153:384.
  16. Würschmidt F, Bünemann H, Heilmann HP. Small cell lung cancer with and without superior vena cava syndrome: a multivariate analysis of prognostic factors in 408 cases. Int J Radiat Oncol Biol Phys 1995; 33:77.
  17. Perez-Soler R, McLaughlin P, Velasquez WS, et al. Clinical features and results of management of superior vena cava syndrome secondary to lymphoma. J Clin Oncol 1984; 2:260.
  18. Presswala RG, Hiranandani NL. Pleural effusion and superior vena cava canal syndrome in Hodgkin's disease. J Indian Med Assoc 1965; 45:502.
  19. Lazzarino M, Orlandi E, Paulli M, et al. Primary mediastinal B-cell lymphoma with sclerosis: an aggressive tumor with distinctive clinical and pathologic features. J Clin Oncol 1993; 11:2306.
  20. Savarese DM, Zavarin M, Smyczynski MS, et al. Superior vena cava syndrome secondary to an angiotropic large cell lymphoma. Cancer 2000; 89:2515.
  21. Airan B, Sharma R, Iyer KS, et al. Malignant thymoma presenting as intracardiac tumor and superior vena caval obstruction. Ann Thorac Surg 1990; 50:989.
  22. Dib HR, Friedman B, Khouli HI, et al. Malignant thymoma. A complicated triad of SVC syndrome, cardiac tamponade, and DIC. Chest 1994; 105:941.
  23. Holbert BL, Libshitz HI. Superior vena caval syndrome in primary mediastinal germ cell tumors. Can Assoc Radiol J 1986; 37:182.
  24. Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg 1990; 160:207.
  25. Parish JM, Marschke RF Jr, Dines DE, Lee RE. Etiologic considerations in superior vena cava syndrome. Mayo Clin Proc 1981; 56:407.
  26. Rozmus G, Daubert JP, Huang DT, et al. Venous thrombosis and stenosis after implantation of pacemakers and defibrillators. J Interv Card Electrophysiol 2005; 13:9.
  27. Otten TR, Stein PD, Patel KC, et al. Thromboembolic disease involving the superior vena cava and brachiocephalic veins. Chest 2003; 123:809.
  28. Sivaram CA, Craven P, Chandrasekaran K. Transesophageal echocardiography during removal of central venous catheter associated with thrombus in superior vena cava. Am J Card Imaging 1996; 10:266.
  29. Van Putten JW, Schlosser NJ, Vujaskovic Z, et al. Superior vena cava obstruction caused by radiation induced venous fibrosis. Thorax 2000; 55:245.
  30. Bell DR, Woods RL, Levi JA. Superior vena caval obstruction: a 10-year experience. Med J Aust 1986; 145:566.
  31. Bechtold RE, Wolfman NT, Karstaedt N, Choplin RH. Superior vena caval obstruction: detection using CT. Radiology 1985; 157:485.
  32. Kim HJ, Kim HS, Chung SH. CT diagnosis of superior vena cava syndrome: importance of collateral vessels. AJR Am J Roentgenol 1993; 161:539.
  33. Eren S, Karaman A, Okur A. The superior vena cava syndrome caused by malignant disease. Imaging with multi-detector row CT. Eur J Radiol 2006; 59:93.
  34. Ahmann FR. A reassessment of the clinical implications of the superior vena caval syndrome. J Clin Oncol 1984; 2:961.
  35. Uberoi R. Quality assurance guidelines for superior vena cava stenting in malignant disease. Cardiovasc Intervent Radiol 2006; 29:319.
  36. Engel IA, Auh YH, Rubenstein WA, et al. CT diagnosis of mediastinal and thoracic inlet venous obstruction. AJR Am J Roentgenol 1983; 141:521.
  37. Podoloff DA, Kim EE. Evaluation of sensitivity and specificity of upper extremity radionuclide venography in cancer patients with indwelling central venous catheters. Clin Nucl Med 1992; 17:457.
  38. Conte FA, Orzel JA. Superior vena cava syndrome and bilateral subclavian vein thrombosis. CT and radionuclide venography correlation. Clin Nucl Med 1986; 11:698.
  39. Qanadli SD, El Hajjam M, Bruckert F, et al. Helical CT phlebography of the superior vena cava: diagnosis and evaluation of venous obstruction. AJR Am J Roentgenol 1999; 172:1327.
  40. Stanford W, Jolles H, Ell S, Chiu LC. Superior vena cava obstruction: a venographic classification. AJR Am J Roentgenol 1987; 148:259.
  41. Lin J, Zhou KR, Chen ZW, et al. Vena cava 3D contrast-enhanced MR venography: a pictorial review. Cardiovasc Intervent Radiol 2005; 28:795.
  42. Thornton MJ, Ryan R, Varghese JC, et al. A three-dimensional gadolinium-enhanced MR venography technique for imaging central veins. AJR Am J Roentgenol 1999; 173:999.
  43. Dosios T, Theakos N, Chatziantoniou C. Cervical mediastinoscopy and anterior mediastinotomy in superior vena cava obstruction. Chest 2005; 128:1551.
  44. Ferrari LR, Bedford RF. General anesthesia prior to treatment of anterior mediastinal masses in pediatric cancer patients. Anesthesiology 1990; 72:991.
  45. Mineo TC, Ambrogi V, Nofroni I, Pistolese C. Mediastinoscopy in superior vena cava obstruction: analysis of 80 consecutive patients. Ann Thorac Surg 1999; 68:223.
  46. Porte H, Metois D, Finzi L, et al. Superior vena cava syndrome of malignant origin. Which surgical procedure for which diagnosis? Eur J Cardiothorac Surg 2000; 17:384.
  47. Pullerits J, Holzman R. Anaesthesia for patients with mediastinal masses. Can J Anaesth 1989; 36:681.
  48. Cosmo L, Haponik EF, Darlak JJ, Summer WR. Neoplastic superior vena caval obstruction: diagnosis with percutaneous needle aspiration. Am J Med Sci 1987; 293:99.
  49. Greillier L, Barlési F, Doddoli C, et al. Vascular stenting for palliation of superior vena cava obstruction in non-small-cell lung cancer patients: a future 'standard' procedure? Respiration 2004; 71:178.
  50. Marcy PY, Magné N, Bentolila F, et al. Superior vena cava obstruction: is stenting necessary? Support Care Cancer 2001; 9:103.
  51. Nagata T, Makutani S, Uchida H, et al. Follow-up results of 71 patients undergoing metallic stent placement for the treatment of a malignant obstruction of the superior vena cava. Cardiovasc Intervent Radiol 2007; 30:959.
  52. Kee ST, Kinoshita L, Razavi MK, et al. Superior vena cava syndrome: treatment with catheter-directed thrombolysis and endovascular stent placement. Radiology 1998; 206:187.
  53. Kvale PA, Selecky PA, Prakash UB, American College of Chest Physicians. Palliative care in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest 2007; 132:368S.
  54. National Comprehensive Cancer Network (NCCN) guidelines. Available at: www.nccn.org (Accessed on May 15, 2012).
  55. Yu JB, Wilson LD, Detterbeck FC. Superior vena cava syndrome--a proposed classification system and algorithm for management. J Thorac Oncol 2008; 3:811.
  56. Loeffler JS, Leopold KA, Recht A, et al. Emergency prebiopsy radiation for mediastinal masses: impact on subsequent pathologic diagnosis and outcome. J Clin Oncol 1986; 4:716.
  57. Warde P, Payne D. Does thoracic irradiation improve survival and local control in limited-stage small-cell carcinoma of the lung? A meta-analysis. J Clin Oncol 1992; 10:890.
  58. Martins SJ, Pereira JR. Clinical factors and prognosis in non-small cell lung cancer. Am J Clin Oncol 1999; 22:453.
  59. Citron ML, Fossieck BE Jr, Krasnow SH, et al. Superior vena cava syndrome due to non--small-cell lung cancer. Resolution with chemotherapy alone. JAMA 1983; 250:71.
  60. Roberts JR, Bueno R, Sugarbaker DJ. Multimodality treatment of malignant superior vena caval syndrome. Chest 1999; 116:835.
  61. Levitt SH, Jones TK Jr, Kilpatrick SJ Jr, Bogardus CR Jr. Treatment of malignant superior vena caval obstruction. A randomized study. Cancer 1969; 24:447.
  62. Mose S, Stabik C, Eberlein K, et al. Retrospective analysis of the superior vena cava syndrome in irradiated cancer patients. Anticancer Res 2006; 26:4933.
  63. Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care 1992; 1:54.
  64. Tanigawa N, Sawada S, Mishima K, et al. Clinical outcome of stenting in superior vena cava syndrome associated with malignant tumors. Comparison with conventional treatment. Acta Radiol 1998; 39:669.
  65. Urruticoechea A, Mesía R, Domínguez J, et al. Treatment of malignant superior vena cava syndrome by endovascular stent insertion. Experience on 52 patients with lung cancer. Lung Cancer 2004; 43:209.
  66. Oudkerk M, Kuijpers TJ, Schmitz PI, et al. Self-expanding metal stents for palliative treatment of superior vena caval syndrome. Cardiovasc Intervent Radiol 1996; 19:146.
  67. Nicholson AA, Ettles DF, Arnold A, et al. Treatment of malignant superior vena cava obstruction: metal stents or radiation therapy. J Vasc Interv Radiol 1997; 8:781.
  68. Baltayiannis N, Magoulas D, Anagnostopoulos D, et al. Percutaneous stent placement in malignant cases of superior vena cava syndrome. J BUON 2005; 10:377.
  69. Crowe MT, Davies CH, Gaines PA. Percutaneous management of superior vena cava occlusions. Cardiovasc Intervent Radiol 1995; 18:367.
  70. Courtheoux P, Alkofer B, Al Refaï M, et al. Stent placement in superior vena cava syndrome. Ann Thorac Surg 2003; 75:158.
  71. Schindler N, Vogelzang RL. Superior vena cava syndrome. Experience with endovascular stents and surgical therapy. Surg Clin North Am 1999; 79:683.
  72. Smayra T, Otal P, Chabbert V, et al. Long-term results of endovascular stent placement in the superior caval venous system. Cardiovasc Intervent Radiol 2001; 24:388.
  73. Lanciego C, Chacón JL, Julián A, et al. Stenting as first option for endovascular treatment of malignant superior vena cava syndrome. AJR Am J Roentgenol 2001; 177:585.
  74. Barshes NR, Annambhotla S, El Sayed HF, et al. Percutaneous stenting of superior vena cava syndrome: treatment outcome in patients with benign and malignant etiology. Vascular 2007; 15:314.
  75. Thierry B, Merhi Y, Bilodeau L, et al. Nitinol versus stainless steel stents: acute thrombogenicity study in an ex vivo porcine model. Biomaterials 2002; 23:2997.
  76. Gwon DI, Ko GY, Kim JH, et al. Malignant superior vena cava syndrome: a comparative cohort study of treatment with covered stents versus uncovered stents. Radiology 2013; 266:979.
  77. Wilson P, Bezjak A, Asch M, et al. The difficulties of a randomized study in superior vena caval obstruction. J Thorac Oncol 2007; 2:514.
  78. Stock KW, Jacob AL, Proske M, et al. Treatment of malignant obstruction of the superior vena cava with the self-expanding Wallstent. Thorax 1995; 50:1151.
  79. Chatziioannou A, Alexopoulos T, Mourikis D, et al. Stent therapy for malignant superior vena cava syndrome: should be first line therapy or simple adjunct to radiotherapy. Eur J Radiol 2003; 47:247.
  80. Hennequin LM, Fade O, Fays JG, et al. Superior vena cava stent placement: results with the Wallstent endoprosthesis. Radiology 1995; 196:353.
  81. Dinkel HP, Mettke B, Schmid F, et al. Endovascular treatment of malignant superior vena cava syndrome: is bilateral wallstent placement superior to unilateral placement? J Endovasc Ther 2003; 10:788.
  82. Gross CM, Krämer J, Waigand J, et al. Stent implantation in patients with superior vena cava syndrome. AJR Am J Roentgenol 1997; 169:429.
  83. Boraks P, Seale J, Price J, et al. Prevention of central venous catheter associated thrombosis using minidose warfarin in patients with haematological malignancies. Br J Haematol 1998; 101:483.
  84. Martin M, Baumgartner I, Kolb M, et al. Fatal pericardial tamponade after Wallstent implantation for malignant superior vena cava syndrome. J Endovasc Ther 2002; 9:680.
  85. Smith SL, Manhire AR, Clark DM. Delayed spontaneous superior vena cava perforation associated with a SVC wallstent. Cardiovasc Intervent Radiol 2001; 24:286.
  86. Doty JR, Flores JH, Doty DB. Superior vena cava obstruction: bypass using spiral vein graft. Ann Thorac Surg 1999; 67:1111.
  87. Dhaliwal RS, Das D, Luthra S, et al. Management of superior vena cava syndrome by internal jugular to femoral vein bypass. Ann Thorac Surg 2006; 82:310.
  88. Bacha EA, Chapelier AR, Macchiarini P, et al. Surgery for invasive primary mediastinal tumors. Ann Thorac Surg 1998; 66:234.
  89. Chen KN, Xu SF, Gu ZD, et al. Surgical treatment of complex malignant anterior mediastinal tumors invading the superior vena cava. World J Surg 2006; 30:162.
  90. Inoue M, Minami M, Shiono H, et al. Efficient clinical application of percutaneous cardiopulmonary support for perioperative management of a huge anterior mediastinal tumor. J Thorac Cardiovasc Surg 2006; 131:755.
  91. Shimizu N, Moriyama S, Aoe M, et al. The surgical treatment of invasive thymoma. Resection with vascular reconstruction. J Thorac Cardiovasc Surg 1992; 103:414.
  92. Charokopos N, Antonitsis P, Klimatsidas M, et al. Secondary endovascular repair of a reconstructed superior vena cava in a patient with a malignant thymic epithelial neoplasm. Thorac Cardiovasc Surg 2007; 55:267.