Superior vena cava syndrome

Opinion statement

Lung cancer is the most common cause of superior vena cava syndrome (SVCS) and requires timely recognition and management. The syndrome is rarely an oncologic emergency in the absence of tracheal compression and airway compromise. Treatment depends on the etiology of the obstructive process. Treatment should also be individualized and should not be undertaken until a diagnosis is obtained. Most patients with SVCS secondary to lung cancer can be treated with appropriately directed chemotherapy or radiotherapy. With the refinement of endovascular stents, percutaneous stenting of the SVC is being increasingly used as primary treatment modality. Thrombotic occlusion can be treated with appropriate lytic agents. In rare circumstances, surgical decompression can be performed; bypass or replacement of the SVC results in immediate improvement in the majority of cases and can be accomplished with low morbidity.

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References and Recommended Reading

  1. 1.

    Hunter W: History of aneurysm of the aorta with some remarks on aneurysms in general. Medical Observations and Inquiries (London) 1757, 1:323.

    Google Scholar 

  2. 2.

    Stokes W: A Treatise on the Diagnosis and Treatment of Diseases of the Chest. Dublin: Hodges and Smith; 1837:370.

    Google Scholar 

  3. 3.

    Rosenblatt MB: Lung cancer in the 19th century. Bull Hist Med 1964, 38:395.

    PubMed  CAS  Google Scholar 

  4. 4.

    Mcintyre FT, Sykes EM: Obstruction of the superior vena cava: a review of the literature and report of two personal cases. Ann Interny Med 1949:925.

  5. 5.

    Markman M: Diagnosis and management of superior vena cava syndrome. Cleve Clin J Med 1999, 66:59.

    PubMed  CAS  Google Scholar 

  6. 6.

    Fincher RE: Superior vena cava syndrome: experience in a teaching hospital. South Med J 1987, 80:1243.

    PubMed  Article  CAS  Google Scholar 

  7. 7.

    Parish JM, Marschke RF, Dines DE, et al.: Etiologic considerations in superior vena cava syndrome. Mayo Clin Proc 1981, 56:407.

    PubMed  CAS  Google Scholar 

  8. 8.

    Banker VP, Maddison FE: Superior vena cava syndrome secondary to aortic disease: report of two cases and review of the literature. Dis Chest 1967, 51:656.

    PubMed  CAS  Google Scholar 

  9. 9.

    Kamiya K, Nahata Y, Naiki K, et al.: Superior vena cava syndrome. Vasc Dis 1967, 4:59.

    PubMed  CAS  Google Scholar 

  10. 10.

    Ahmann FR: A reassessment of the clinical implications of the superior vena cava syndrome. J Clin Oncol 1984, 2:961.

    PubMed  CAS  Google Scholar 

  11. 11.

    Lockridge SK, Knibbe WP, Doty DB: Obstruction of the superior vena cava. Surgery 1979, 85:14.

    Google Scholar 

  12. 12.

    Chen JC, Bongard F, Klein SR: A contemporary perspective on superior vena cava syndrome. Am J Surg 1990, 160:207–211.

    PubMed  Article  CAS  Google Scholar 

  13. 13.

    Inoue H, Shohtse A, Koide S, et al.: Resection of the superior vena cava for primary lung cancer: 5 years’ survival. Ann Thorac Surg 1990, 50:661–662.

    PubMed  CAS  Article  Google Scholar 

  14. 14.

    Dartevelle PG, Chapelier AR, Pastorino U, et al.:Long-term follow-up after prosthetic replacement of the superior vena cava combined with resections of mediastinal-pulmonary malignant tumors. J Thorac Cardiovasc Surg 1991, 102:29–65.

    Google Scholar 

  15. 15.

    Perez CA, Presant CA, VanAmburg AL: Management of superior vena cava syndrome. Semin Oncol 1978, 5:123.

    PubMed  CAS  Google Scholar 

  16. 16.

    Salsali M, Clifton EE: Superior vena caval obstruction with carcinoma of the lung. Surg Gynecol Obstet 1965, 121:783.

    PubMed  CAS  Google Scholar 

  17. 17.

    Escalante CP: Causes and management of superior vena cava syndrome. Oncology 1993, 7:61.

    PubMed  CAS  Google Scholar 

  18. 18.

    Nogeire C, Mincer F, Botstein C: Long survival in patients with bronchogenic carcinoma complicated by superior vena cava obstruction. Chest 1979, 75:325.

    PubMed  CAS  Google Scholar 

  19. 19.

    Nesbitt JC: Surgical management of superior vena cava syndrome. In Lung Cancer: Principles and Practice. Philadelphia: Lippincott Williams & Wilkins; 1996:671–681.

    Google Scholar 

  20. 20.

    Yellin A, Rosen A, Reichert N, et al.: Superior vena cava syndrome: the myth—the facts. Am Rev Respir Dis 1990, 141:1114.

    PubMed  CAS  Google Scholar 

  21. 21.

    Perez CA, Simpson JR,: Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys 1987, 3:531.

    Google Scholar 

  22. 22.

    Netter FH: Superior vena cava syndrome. The CIBA Collection of Medical Illustrations: Respiratory System. West Caldwell, NJ: CIBA Pharmaceutical Company; 1980:164.

    Google Scholar 

  23. 23.

    Baker GL, Barnes JH: Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care 1992, 1:54.

    PubMed  CAS  Google Scholar 

  24. 24.

    Adelstein DJ: Managing three common oncologic emergencies. Cleve Clin J Med 1991, 586:457.

    Google Scholar 

  25. 25.

    Helms SR, Carlson MD: Cardiovascular emergencies. Semin Oncol 1989, 16:463.

    PubMed  CAS  Google Scholar 

  26. 26.

    Stanford W, Doty DB: The role of venography and surgery in the management of patients with superior vena cava obstruction. Ann Thorac Surg 1986, 41:158.

    PubMed  CAS  Article  Google Scholar 

  27. 27.

    Hussey JJ, Katz S, Yayer WM: The superior vena cava syndrome: report of thirty-five cases. Am Heart J 1946, 31:1.

    Article  Google Scholar 

  28. 28.

    Gooding GA, Hightower DR, Moore EH, et al.: Obstruction of the superior vena cava or subclavian veins: sonographic diagnosis. Radiology 1986, 159:663–665.

    PubMed  CAS  Google Scholar 

  29. 29.

    Mancuso L, Pitrolo F, Bondi F, et al.: Echocardiographic recognition of mediastinal masses. Chest 1988, 93:144–148.

    PubMed  CAS  Google Scholar 

  30. 30.

    Sharma RP, Keller CE, Shetty PC, et al.: Superior vena cava obstruction: evaluation with digital subtraction angiography. Radiology 1986, 160:845.

    PubMed  CAS  Google Scholar 

  31. 31.

    Abner A: Approach to the patient who presents with superior vena cava obstruction. Chest 1993, 103:394s-397s.

    PubMed  CAS  Google Scholar 

  32. 32.

    Schraufnagel DE, Hill R, Leech JA, et al.: Superior vena cava obstruction. Am J Med 1981, 70:1169.

    PubMed  Article  CAS  Google Scholar 

  33. 33.

    Davenport D, Ferree C, Blake D, et al.: Response of superior vena cava syndrome to radiation therapy. Cancer 1976, 38:1577.

    PubMed  Article  CAS  Google Scholar 

  34. 34.

    Kirschner PA: Mediastinoscopy in superior vena cava obstruction. In Mediastinoscopy: Proceedings of an International Symposium. Edited by Jepssen O, Ruhbek-Soreneon H. Odense, Denmark: Odense University Press; 1971:40.

    Google Scholar 

  35. 35.

    Callejas MA, Rami R, Catalan M, et al.: Mediastinoscopy as an emergency diagnostic procedure in superior vena cava syndrome. Scand J Thorac Cardiovasc Surg 1991, 25:137.

    PubMed  CAS  Google Scholar 

  36. 36.

    Little AG, Golomb HM, Ferguson MK, et al.: Malignant superior vena cava obstruction reconsidered: the role of diagnostic surgical intervention. Ann Thorac Surg 1985, 40:285.

    PubMed  CAS  Article  Google Scholar 

  37. 37.

    Jahangiri M, Taggart DP, Goldstraw P: Role of media-stinoscopy in superior vena cava obstruction. Cancer 1993, 71:3006.

    PubMed  Article  CAS  Google Scholar 

  38. 38.

    Sculier JP, Evans WK, Feld R, et al.: Superior vena caval obstruction syndrome in small cell lung cancer. Cancer 1986, 57:847–851.

    PubMed  Article  CAS  Google Scholar 

  39. 39.

    Greenlee RT, Murray T, Bolden S, Wingo PA: Cancer statistics. CA Cancer J Clin 2000, 50:7.

    PubMed  CAS  Google Scholar 

  40. 40.

    Choi NC, Doucette JA: Improved survival of patients with unresectable non-small-cell bronchogenic carcinoma by an innovated high-dose en-bloc radiotherapeutic approach. Cancer 1981, 48:101.

    PubMed  Article  CAS  Google Scholar 

  41. 41.

    Parker S, Tong T, Bolden S, et al.: Cancer statistics. CA Cancer J Clin 1996, 65:5–27.

    Google Scholar 

  42. 42.

    Sherry S: Clinical management of the thrombosed vessel: an overview. Angiology 1982, 33:6–10.

    PubMed  CAS  Google Scholar 

  43. 43.

    Marder VJ, Bele WR: Fibrinolytic Therapy. Philadelphia: JB Lippincott; 1987:1393–1437.

    Google Scholar 

  44. 44.

    Sculier JP, Feld R: Superior vena cava obstruction syndrome: recommendations for management. Cancer Treat Rev 1985, 12:209–218.

    PubMed  Article  CAS  Google Scholar 

  45. 45.

    Smith NL, Ravo B, Soroff HS, Khan SA: Successful fibrinolytic therapy for superior vena cava thrombosis secondary to long-tern total parenteral nutrition. J Parenteral Enteral Nutr 1985, 9:55–57.

    CAS  Google Scholar 

  46. 46.

    Matthews JA, Blake HA, Hall DJ: Iatrogenic superior vena cava syndrome treated with streptokinase. Gynecol Oncol 1987, 26:119–122.

    PubMed  Article  CAS  Google Scholar 

  47. 47.

    Meister FL, McLaughlin TF, Tenney RD, Sholkoff SD:Urokinase: a cost-effective alternative treatment of superior vena cava thrombosis and obstruction. Arch Intern Med 1989, 149:1209–1210.

    PubMed  Article  CAS  Google Scholar 

  48. 48.

    Gray B, Olin JW, Graor RA, Young JR, et al.: Safety and efficacy of thrombolytic therapy for superior vena cava syndrome. J Am Coll Cardiol 1990, 15:193A.

    Article  Google Scholar 

  49. 49.

    Ruckley CV, Boulton FE, Redhead D: The treatment of venous thrombosis of the upper and lower limbs with ‘APSAC’ (p-anisoylated streptokinase-plasminogen complex). Eur J Vasc Surg 1987, 1:107–112.

    PubMed  Article  CAS  Google Scholar 

  50. 50.

    Greenberg S, Kosinski R, Daniels J: Treatment of superior vena cava thrombosis with recombinant tissue type plasminogen activator. Chest 1991, 99:1298–1301.

    PubMed  CAS  Google Scholar 

  51. 51.

    Goldhaber SZ, Kessler CM, Heit J, et al.: Randomized controlled trial of tissue plasminogen activator versus urokinase in the treatment of acute pulmonary embolism. Lancet 1988, 2:293–298.

    PubMed  Article  CAS  Google Scholar 

  52. 52.

    Magnani B, for the PAIMS Investigators: Plasminogen Activator Italian Multicenter Study (PAIMS). Comparison of intravenous recombinant single-chain human tissue-type plasminogen activator (rt-PA) with intra-venous streptokinase in acute myocardial infarction. J Am Coll Cardiol 1989, 13:19–26.

    PubMed  CAS  Article  Google Scholar 

  53. 53.

    Collen D, Stassen JM, Verstraete M: Thrombolysis with human extrinsic (tissue-type) plasminogen activator in rabbits with experimental jugular vein thrombosis: effect of molecular form and dose of activator, age of thrombosis, and route of administration. J Clin Invest 1983, 71:368–376.

    PubMed  CAS  Google Scholar 

  54. 54.

    Fraschini G, Jadeja J, Lawson M, et al.: Infusion of urokinase for the lysis of thrombosis associated with permanent central venous catheters in cancer patients. J Clin Oncol 1987, 5:672–678.

    PubMed  CAS  Google Scholar 

  55. 55.

    Armstrong BA, Perez CA, Simpson JR, Hederman MA:Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol 1987, 13:531–539.

    CAS  Article  Google Scholar 

  56. 56.

    Rodrigues CI, Njo KH, Karim AB: Hypofractionated radiation therapy in the treatment of superior vena cava syndrome. Lung Cancer 1993, 10:221–228.

    PubMed  Article  CAS  Google Scholar 

  57. 57.

    Rosch J, Bedell JE, Putnam J, et al.: Gianturco expand-able wire stents in the treatment of superior vena cava syndrome recurring after maximum-tolerance radiation. Cancer 1987, 60:1243–1246.

    PubMed  Article  CAS  Google Scholar 

  58. 58.

    Charnsangavej C, Carrasco CH, Wallace S, et al.:Stenosis of the vena cava: preliminary assessment of treatment with expandable metallic stents. Radiology 1986, 161:295–298.

    PubMed  CAS  Google Scholar 

  59. 59.

    Rocchini AP, Meliones JN, Beckman RH, et al.: Use of balloon-expandable stents to treat experimental peripheral pulmonary artery and superior vena caval stenosis: preliminary experience. Pediatr Cardiol 1992, 13:92–96.

    PubMed  Article  CAS  Google Scholar 

  60. 60.

    Wright KC, Wallace S, Charnsangavej C, et al.:Percutaneous endovascular stents: an experimental evaluation. Radiology 1985, 156:69–72.

    PubMed  CAS  Google Scholar 

  61. 61.

    Mullins CE, O’Laughlin MP, Vick GW, et al.: Implanta-tion of balloon-expandable intravascular grafts by catheterization in pulmonary arteries and systemic veins. Circulation 1988, 77:188–199.

    PubMed  CAS  Google Scholar 

  62. 62.

    Sherry CS: Successful treatment of SVC syndrome by venous angioplasty. Am J Roentgenol 1986, 147:834–835.

    CAS  Google Scholar 

  63. 63.

    Hochrein J, Bashore TM, O’Laughlin MP, Harrison JK:Percutaneous stenting of superior vena cava syndrome: a case report and review of the literature. Am J Med 1998, 104:78–84. A case presentation of a patient with complete occlusion of the SVC who underwent successful vena caval revascularization with placement of balloon expandable metallic stents. It also provides a review of published reports on the use of stents for SVCS, demonstrating that stents can be used with excellent results.

    PubMed  Article  CAS  Google Scholar 

  64. 64.

    Furui S, Sawada S, Kuramoto K, et al.: Gianturco stent placement in malignant caval obstruction: analysis of factors for predicting the outcome. Radiology 1995, 195:147–152.

    PubMed  CAS  Google Scholar 

  65. 65.

    Crowe MTI, Davies CH, Gaines PA: Percutaneous management of superior vena cava occlusions. Cardiovasc Intervent Rad 1995, 18:367–372.

    Article  CAS  Google Scholar 

  66. 66.

    Dondelinger RF, Goffette P, Kurdziel J, Roche A: Expandable metal stents for stenosis of the venae cavae and large veins. Semin Intervent Radiol 1991, 8:252–263.

    Google Scholar 

  67. 67.

    Watkinson AF, Hansell DM: Expandable Wallstent for the treatment of obstruction of the superior vena cava. Thorax 1993, 48:915–920.

    PubMed  CAS  Google Scholar 

  68. 68.

    Dyet JF, Nicholson AA, Cook AM: The use of the Wallstent endovascular prosthesis in the treatment of malignant obstruction of the superior vena cava. Clin Radiol 1993, 48:381–385.

    PubMed  Article  CAS  Google Scholar 

  69. 69.

    Rosemblum J, Leef J, Messersmith R, et al.: Intravascular stents in the management of acute superior vena cava obstruction of benign etiology. J Parenteral Enteral Nutr 1994, 18:362–366.

    Google Scholar 

  70. 70.

    Antonucci F, Salomonowitz E, Stuckman G, et al.:Placement of venous stents: clinical experience with a self-expanding prosthesis. Radiology 1992, 183:394–397.

    Google Scholar 

  71. 71.

    Zollikofer CL, Antonucci F, Stuckman G, et al.: Use of the Wallstent in the venous system including hemodialysis-related stenoses. Cardiovasc Intervent Radiol 1992, 15:334–341.

    PubMed  CAS  Google Scholar 

  72. 72.

    Solomon N, Wholey MH, Jarmolowski CR: Intravascular stents in the management of superior vena cava syndrome. Cathet Cardiovasc Diagn 1991, 23:245–252.

    PubMed  Article  CAS  Google Scholar 

  73. 73.

    Elson JD, Becker GJ, Wholey MH, Ehrman KO: Vena caval and central venous stenoses: management with Palmaz balloon-expandable intraluminal stents. J Vasc Interv Radiol 1991, 2:215–223.

    PubMed  CAS  Article  Google Scholar 

  74. 74.

    Palmaz JC: Balloon-expandable intravascular stent. AJR 1988, 150:1263–1269.

    PubMed  CAS  Google Scholar 

  75. 75.

    Rosch J, Uchida BT, Hall LD, et al.: Gianturco-Rosch expandable Z-stents in the treatment of superior vena cava syndrome. Cardiovasc Interv Radiol 1992, 15:319–327.

    CAS  Google Scholar 

  76. 76.

    Oudkerk M, Heystraten FMJ, Stoter G: Stenting in malignant vena caval obstruction. Cancer 1993, 71:142–146.

    PubMed  Article  CAS  Google Scholar 

  77. 77.

    Swada S, Fujiwara Y, Koyama T, et al.: Application of expandable metallic stents to the venous system. Acta Radiol 1992, 33:156–159.

    Article  Google Scholar 

  78. 78.

    Irving JD, Dondelinger RF, Reidy JF, et al.: Gianturco self-expanding stents: clinical experience in the vena cava and large veins. Cardiovasc Interv Radiol 1992, 15:328–333.

    CAS  Google Scholar 

  79. 79.

    Kishi K, Sonomura T, Mitsuzane K, et al.: Self-expand-able metallic stent therapy for superior vena cava syndrome: clinical observations. Radiology 1993, 189:531–535.

    PubMed  CAS  Google Scholar 

  80. 80.

    Nicholson A: Treatment of malignant superior vena cava obstruction: metal stents or radiation therapy. J Vasc Interv Radiol 1997, 8:781–788. A prospective study looking at the results of treating malignant superior vena cava obstruction by means of percutaneous self-expanding stent insertion or by means of radiation therapy. This study suggests that percutaneous stent insertion in malignant superior vena cava obstruction fulfills the requirements of a palliative procedure better than radiation therapy.

    PubMed  CAS  Article  Google Scholar 

  81. 81.

    Edwards RD, Jackson JE: Case report: superior vena caval obstruction treated by thrombolysis, mechanical thrombectomy, and metallic stents. Clin Radiol 1993, 48:215–217.

    PubMed  Article  CAS  Google Scholar 

  82. 82.

    Effler DB, Groves LK: Superior vena cava obstruction. J Thorac Cardiovasc Surg 1962, 43:574.

    Google Scholar 

  83. 83.

    Ahmann FR: A reassessment of the clinical implications of the superior vena cava syndrome. J Clin Oncol 1984, 2:961–969.

    PubMed  CAS  Google Scholar 

  84. 84.

    Doty DB: Bypass of superior vena cava: six years experience with spiral vein graft for obstruction of the superior vena cava due to benign and malignant disease. J Thorac Cardiovasc Surg 1982, 83:326.

    PubMed  CAS  Google Scholar 

  85. 85.

    Doty DB, Baker WH: Bypass of the superior vena cava with spiral vein graft. Ann Thorac Surg 1976, 22:490.

    PubMed  CAS  Article  Google Scholar 

  86. 86.

    Doty DB, Doty JR, Jones KW: Bypass of superior vena cava. Fifteen years’ experience with spiral vein graft for obstruction of superior vena cava caused by benign disease. J Thorac Cardiovasc Surg 1990, 99:889.

    PubMed  CAS  Google Scholar 

  87. 87.

    Masuda H, Ogata T, Kikuchi K, et al.: Longevity of expanded polytetrafluoroethylene grafts for superior vena cava. Ann Thorac Surg 1989, 48:376.

    PubMed  CAS  Article  Google Scholar 

  88. 88.

    Moghissi K, Dhumale R, Sench M: Innominate bypass graft for malignant superior vena caval obstruction. Paper presented to the Society of Thoracic and Cardio-vascular Surgeons of Great Britain and Ireland; London:1983.

  89. 89.

    Dartevelle PG, Chapelier AR, Pastorino U, et al.:Long-term follow-up after prosthetic replacement of the superior vena cava combined with resection of mediastinal-pulmonary malignant tumors. J Thorac Cardiovasc Surg 1991, 102:259.

    PubMed  CAS  Google Scholar 

  90. 90.

    Nakahara K, Ohno K, Mastumura A, et al.: Extended operation for lung cancer invading the aortic arch and superior vena cava. J Thorac Cardiovasc Surg 1989, 97:428.

    PubMed  CAS  Google Scholar 

  91. 91.

    Lai WW, Wu MH, Chou NS, Lin MY: Surgery for malignant involvement of the superior vena cava. J Formos Med Assoc 1992, 91:991.

    PubMed  CAS  Google Scholar 

  92. 92.

    Larsson S, Lepore V: Technical options in reconstruction of large mediastinal veins. Surgery 1992, 111:311.

    PubMed  CAS  Google Scholar 

  93. 93.

    Adelstein DJ, Hines JD, Carter SG, et al.: Thromboembolic events in patients with malignant superior vena cava syndrome and the role of anticoagulation. Cancer 1988, 62:2258–2262.

    PubMed  Article  CAS  Google Scholar 

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Wudel, L.J., Nesbitt, J.C. Superior vena cava syndrome. Curr. Treat. Options in Oncol. 2, 77–91 (2001). https://doi.org/10.1007/s11864-001-0019-3

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Keywords

  • Stent Placement
  • Streptokinase
  • Superior Vena
  • Superior Vena Cava
  • Main Drug Interaction