Superior vena cava syndrome

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

• Provide accurate info on location obstruction

• Determine etiology of obstruction

• Info on the extent of collaterals

• Guide biopsy attempts

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