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superior vena cava (SVC) syndrome

Obstruction of blood flow through the superior vena cava. Etiology: 1) lung cancer, especially small cell carcinoma (75%) 2) Hodgkin's lymphoma 3) mediastinal fibrosis 4) radiation fibrosis 5) other mediastinal tumors a) thymoma b) germ cell tumor c) non-Hodgkin's lymphoma 6) thrombosis [6] - central venous catheters - implanted device (ICD) 7) goiter compressing subclavian vein & jugular vein [9] Pathology: - 3 collateral venous pathways - azygous vein - internal thoracic vein - long thoracic vein - when venous pathways overwelmed, venous pressures become elevated Clinical manifestations: 1) insidious onset 2) dyspnea is the most common presenting symptom 3) dysphagia 4) cough 5) chest pain 6) prominent collateral veins (venous distension) over the chest 7) jugular venous distension 8) facial edema, Pemberton's Sign [9] 9) fullness of the head, pethora 10) headache 11) arm swelling 12) stridor (tracheal obstruction) Special laboratory: 1) bronchoscopy has a high yield & is generally safe - not safe [NEJM knowlege+] 2) mediastinoscopy may be associated with excessive hemorrhage due to obstructed venous return but may have a higher diagnostic yield - complications 5% [2] 3) thorcentesis if pleural effusion 4) biopsy of peripheral lymphadenopathy Radiology: - computed tomography (CT of chest) with IV contrast (diagnostic test of choice) a) mediastinal widening b) pleural effusion may be present Complications: - tracheal obstruction Management: 1) generally NOT regarded as a medical emergency 2) generally MORE IMPORTANT to establish tissue diagnosis a) biopsy tissue external to obstructing mass b) if no external tissue to biopsy, biopsy mediastinal mass c) if tissue biopsy unsuccessful at establishing diagnosis, bronchoscopy, mediastinoscopy, thoracotomy 3) treatment directed at specific etiology (type of cancer) a) radiation therapy (mainstay of treatment [2]) - palliation in 70% of patients with lung cancer & in 95% of patients with lymphoma - appropriate even with unstaged NSCLC [6] b) chemotherapy: small cell lung cancer, germ cell neoplasms, NSCLC c) continuous infusion of heparin for thrombosis [6] 4) endovascular stenting a) refractory disease, hempdynamic instability [10] b) rapid symptomatic improvement c) warfarin 1 mg daily with goal of INR < 1.6 ?? [3] 5) does not worsen prognosis of otherwise curable malignancies [2]

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superior vena cava (SVC) thoracic outlet syndrome

General

syndrome

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 721
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2021.
  3. UpToDate 14.1 http://www.utdol.com
  4. Wan JF, Bezjak A. Superior vena cava syndrome. Hematol Oncol Clin North Am. 2010 Jun;24(3):501-13 PMID: 20488350
  5. Kumar B and Hosn NA Superior Vena Cava Syndrome N Engl J Med 2014; 371:1142. September 18, 2014 PMID: 25229918 http://www.nejm.org/doi/full/10.1056/NEJMicm1311911
  6. Wilson LD et al Superior Vena Cava Syndrome with Malignant Causes. N Engl J Med 2007; 356:1862-1869. May 3, 2007 PMID: 17476012 http://www.nejm.org/doi/full/10.1056/NEJMcp067190
  7. NEJM Knowledge+/ Question of the Week. July 26, 2016 http://knowledgeplus.nejm.org/question-of-week/936/
  8. DeFilippis EM, Vaidya A, Braun D et al A Shocking Turn of Events N Engl J Med. 2018 May 24;378(21):e29. PMID: 29791827 https://www.nejm.org/doi/full/10.1056/NEJMimc1710576
  9. Abu-Shama Y, Cuny T. Pemberton's Sign in a Patient with a Goiter. N Engl J Med 2018; 378:e31. May 31, 2018 PMID: 29847764 https://www.nejm.org/doi/full/10.1056/NEJMicm1712263
  10. NEJM Knowledge+ Hematology
  11. Superior Vena Cava Syndrome (PDQ) http://www.cancer.gov/cancertopics/pdq/supportivecare/superior-vena-cava/HealthProfessional