Superior vena cava syndrome



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SUPERIOR VENA CAVA SYNDROME

  • Elesyia D. Outlaw

  • March 9, 2004


SVC Syndrome

  • Constellation of signs and symptoms caused by obstruction of blood flow in the superior vena cava.

  • Secondary to external compression, invasion, constriction or thrombosis of the SVC

  • Can be partial or complete obstruction



SCVS (cont)

  • Leads to increased venous pressure and results in edema of the head, neck, arms, and upper chest

  • Dilated veins on the chest wall

  • Pleural/pericardial effusions

  • Cerebral edema/Increased IC pressure



Patients



Patients



Clinical Features of SVC

  • SYMPTOMS FREQUENCY

  • Short of Breath 50%

  • Chest Pain 20%

  • Cough 20%

  • Dysphagia 20%

  • Markman, M. Cleveland Clinic Journal of Medicine, 1999



Clinical Features of SVCS

  • SIGNS FREQUENCY

  • Thorax Vein Distention 70%

  • Neck Vein Distention 60%

  • Facial Swelling 45%

  • UE/Trunk Swelling 40%

  • Cyanosis 15%

  • Markman, M. Cleveland Clinic Journal of Medicine, 1999



A/P #1



A/P #2

  • Formed by merger of left/right brachiocephalic veins + azygous

  • Venous blood from head/neck/upper extremities

  • 6 to 8 cm in length

  • 1.5 to 2 cm wide

  • Abner, A. Chest, 1993



A/P #3

  • SVC surrounded by rigid structures (ie mediastinum, sternum, right mainstem bronchus and LN)

  • Thin walled and easily compressible secondary to low pressure

  • Prone to obstruction relative to its “neighbors”



A/P #4

  • As obstruction develops, venous collaterals form

  • Alternate pathways for venous return to the RA

  • Severity of sx depends on the time course of obstruction



SVCS



Etiology of SVC

  • Malignancy

    • Lung cancer
    • Lymphoma
    • Thymoma
    • Metastatic
    • Germ Cell


Malignancy

  • Account for 80-97% of SVCS cases

  • Lung Cancer 75-80%

  • Lymphoma 10-15%

  • Others 5%

    • Metastatic
    • Thymoma
    • Germ cell tumor
  • Markman, M. Cleveland Clin JOM, 1999.

  • Ostler, P. Clin Onc, 1997.



Lung Cancer

  • 5-10% Lung cancer pts develop SVCS

  • SCLC pts account for 50% SVCS in this group--yet only 25% of lung cancers

  • Tend to arise in central/perihilar

  • Right>>>>Left

  • Markman, M. Cleveland Clin JOM, 1999.

  • Ostler, P. Clin Onc, 1997.



Lymphoma

  • MD Anderson experience

  • 915 pts treated for NHL

  • 36 pts (3.9%) presented with SVCS

  • 23 Diffuse LCL

  • 12 Lymphoblastic

  • 1 Follicular LCL

  • Perez-Soler, R. J Clin Onc, 1984.



Benign

  • 1st case of SVCS described by William Hunter in 1757

  • Secondary to aortic aneurysm 2/2 syphilis

  • Pre-abx era---->approx 50% SVCS cases

  • Current----->3-5% SVCS cases



Mediastinitis

  • Histoplasmosis 50%

    • Fibrosing mediastinitis
  • Others 50%

    • TB
    • Actinomycosis
    • Syphilis
    • Post XRT
    • Majahan, V. Chest, 1975


Benign Neoplasms

  • Substernal thyroid

  • Teratoma/Dermoid cysts

  • Benign Thymoma

  • Cystic hygroma



Iatrogenic

  • Thrombus formation 2/2 venous catheters

  • PM implantation

  • TPN lines

  • Swan-Ganz catheters

  • HD catheters

  • Mahajan, V. Chest, 1975.

  • Bertrand, M. Cancer, 1984.



Diagnosis

  • Chest radiograph

  • Duplex ultrasound

  • CT/MRI/MRV

  • Venogram

  • Radionuclide studies



Chest Radiograph

  • CXR FINDINGS FREQUENCY

  • Mediastinal Mass

  • or Widening 59-84%

  • Hilar LAD 19-50%

  • Pleural Effusions 25%

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987

  • Markman, M. Cleveland Clinic JOM, 1999

  • Parish, JM. Mayo Clin Proc, 1981



CT/MRI/MRV



Venography

  • Can give precise level of obstruction

  • Less information on etiology of SVCS

  • Requires larger contrast dose

  • Usually done during IR mgmt



Tissue Diagnosis

  • Procedure Yield

  • Sputum cytology 33-40%

  • Bronchoscopy 33-60%

  • LN biopsy 46-80%

  • Mediastinoscopy 100%

  • Thoracotomy 100%

  • Ostler, J. Clin Onc, 1997

  • Schindler, N. Surg Clin N Am, 1999



Which First---> Tx or Dx?

  • Ahman

  • Literature search 1934-1984

  • 1986 cases SVC reviewed

  • Only 1 clearly documented death 2/2 SVCS

  • Ahman, F. J Clin Onc, 1984.



1st--->Tx or Dx?

  • 843 inv dx proced Comps

  • 119 Thoractomies 2

  • 53 Mediastinoscopies 3

  • 217 Bronchoscopies 2

  • 120 LN biopsies 1

  • 197 Venograms 1



Treatment

  • Tailored to etiology

  • Historically standard tx----->XRT

  • Emergent tx before tissue dx 2/2 presumed risk of bleeding

  • Current standard----> tissue dx prior to initiating tx



Treatment

  • Goal

    • treat symptoms
    • treat underlying cause
  • Tx should be tailored to histologic diagnosis---->determine if curative vs palliative



Treatment

  • Chemotherapy

  • XRT

  • Surgery

  • Interventional Procedures

  • Spiro, S. Thorax, 1983

  • Perez-Soler, P. J Clin Onc, 1984



Treatment

  • Chemo vs XRT=equally effective

  • Combination of chemo/xrt did not improve response rate, symptoms or LT survival

  • Decreased LR in lymphoma but no change in OS

  • Armstrong, B. Intl J RO Biol Phys, 1984.

  • Perez-Stoler, P. J Clin Onc, 1984.



Surgical Tx



IR Treatment



IR Tx #2



IR Tx #3



IR Tx #4



Prognosis

  • Varies depending on the etiology

  • SVCS in its own right is rarely fatal

  • 10-20% survive at least 2 years

  • Ahman,F. J Clin Onc, 1984

  • Ostler, PJ. Clin Onc, 1997

  • Perez & Brady, 2004.



Prognosis

  • Reviewed 5052 patients tx at MIR 1/1965-12/1984

  • 125 patients tx SVCS 2/2 malignancy

  • Lung Cancer 79%, Lymphoma 18%, Other 6%

  • XRT+/- chemotherapy

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987



Prognosis Overall

  • Median Survial=5.5 months

  • 1 year survival=24%

  • 5 year survival= 9%

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987



Prognosis-SCLC

  • 1 year survival=24%

  • 5 year survival= 5%

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987



Prognosis-Lymphoma

  • 1 year survival=41%

  • 5 year survival=41%

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987



Prognosis-NSLC

  • 1 year survival=17%

  • 2 year survival= 2%

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987



Prognosis

  • No statistical difference in survival rates between patients treated with chemoradiation vs either tx alone

  • Pts who responding clinically within 30days of treatment had better 1 year survival (27% vs 7%)

  • Armstrong, B. Int J Radiot Onc Biol Phys, 1987



Prognosis-BSVCS

  • Depends on collateral circulation

  • 20-50 years

  • GreenbergA. Ann Thorac Surg, 1985

  • Mahajan, V. Chest, 1975

  • Murdock, W. Scott Med J, 1960




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