SUPERIOR VENA CAVA SYNDROME Elesyia D. Outlaw March 9, 2004

SVC Syndrome 4 Constellation of signs and symptoms caused by obstruction of blood flow in the superior vena cava. 4 Secondary to external compression, invasion, constriction or thrombosis of the SVC 4 Can be partial or complete obstruction

SCVS (cont) 4 Leads to increased venous pressure and results in edema of the head, neck, arms, and upper chest 4 Dilated veins on the chest wall 4 Pleural/pericardial effusions 4 Cerebral edema/Increased IC pressure

Patients

Clinical Features of SVC SYMPTOMSFREQUENCY Short of Breath50% Chest Pain20% Cough20% Dysphagia20% Markman, M. Cleveland Clinic Journal of Medicine, 1999

Clinical Features of SVCS SIGNSFREQUENCY Thorax Vein Distention70% Neck Vein Distention60% Facial Swelling45% UE/Trunk Swelling40% Cyanosis15% Markman, M. Cleveland Clinic Journal of Medicine, 1999

A/P #1

A/P #2 4 Formed by merger of left/right brachiocephalic veins + azygous 4 Venous blood from head/neck/upper extremities 4 6 to 8 cm in length to 2 cm wide Abner, A. Chest, 1993

A/P #3 4 SVC surrounded by rigid structures (ie mediastinum, sternum, right mainstem bronchus and LN) 4 Thin walled and easily compressible secondary to low pressure 4 Prone to obstruction relative to its “neighbors”

A/P #4 4 As obstruction develops, venous collaterals form 4 Alternate pathways for venous return to the RA 4 Severity of sx depends on the time course of obstruction

SVCS

Etiology of SVC 4 Malignancy –Lung cancer –Lymphoma –Thymoma –Metastatic –Germ Cell 4 “Benign” –Infection/Inflammation –Benign Neoplasms –Iatrogenic –Trauma

Malignancy 4 Account for 80-97% of SVCS cases 4 Lung Cancer75-80% 4 Lymphoma10-15% 4 Others 5% –Metastatic –Thymoma –Germ cell tumor Markman, M. Cleveland Clin JOM, Ostler, P. Clin Onc, 1997.

Lung Cancer % Lung cancer pts develop SVCS 4 SCLC pts account for 50% SVCS in this group--yet only 25% of lung cancers 4 Tend to arise in central/perihilar 4 Right>>>>Left Markman, M. Cleveland Clin JOM, Ostler, P. Clin Onc, 1997.

Lymphoma 4 MD Anderson experience pts treated for NHL 4 36 pts (3.9%) presented with SVCS 4 23 Diffuse LCL 4 12 Lymphoblastic 4 1Follicular LCL Perez-Soler, R. J Clin Onc, 1984.

Benign 4 1st case of SVCS described by William Hunter in Secondary to aortic aneurysm 2/2 syphilis 4 Pre-abx era---->approx 50% SVCS cases 4 Current----->3-5% SVCS cases

Mediastinitis 4 Histoplasmosis50% –Fibrosing mediastinitis 4 Others50% –TB –Actinomycosis –Syphilis –Post XRT Majahan, V. Chest, 1975

Benign Neoplasms 4 Substernal thyroid 4 Teratoma/Dermoid cysts 4 Benign Thymoma 4 Cystic hygroma

Iatrogenic 4 Thrombus formation 2/2 venous catheters 4 PM implantation 4 TPN lines 4 Swan-Ganz catheters 4 HD catheters Mahajan, V. Chest, Bertrand, M. Cancer, 1984.

Diagnosis 4 Chest radiograph 4 Duplex ultrasound 4 CT/MRI/MRV 4 Venogram 4 Radionuclide studies

Chest Radiograph CXR FINDINGSFREQUENCY Mediastinal Mass or Widening59-84% Hilar LAD19-50% 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 4 Provide accurate info on location obstruction 4 Determine etiology of obstruction 4 Info on the extent of collaterals 4 Guide biopsy attempts

Venography 4 Can give precise level of obstruction 4 Less information on etiology of SVCS 4 Requires larger contrast dose 4 Usually done during IR mgmt

Tissue Diagnosis ProcedureYield Sputum cytology33-40% Bronchoscopy33-60% LN biopsy46-80% Mediastinoscopy 100% Thoracotomy 100% Ostler, J. Clin Onc, 1997 Schindler, N. Surg Clin N Am, 1999

Which First---> Tx or Dx? 4 Ahman 4 Literature search cases SVC reviewed 4 Only 1 clearly documented death 2/2 SVCS Ahman, F. J Clin Onc, 1984.

1st--->Tx or Dx? 843 inv dx procedComps 119 Thoractomies2 53Mediastinoscopies3 217Bronchoscopies2 120LN biopsies1 197Venograms1

Treatment 4 Tailored to etiology 4 Historically standard tx----->XRT 4 Emergent tx before tissue dx 2/2 presumed risk of bleeding 4 Current standard----> tissue dx prior to initiating tx

Treatment 4 Goal –treat symptoms –treat underlying cause 4 Tx should be tailored to histologic diagnosis---->determine if curative vs palliative

Treatment 4 Chemotherapy 4 XRT 4 Surgery 4 Interventional Procedures Spiro, S. Thorax, 1983 Perez-Soler, P. J Clin Onc, 1984

Treatment 4 Chemo vs XRT=equally effective 4 Combination of chemo/xrt did not improve response rate, symptoms or LT survival 4 Decreased LR in lymphoma but no change in OS Armstrong, B. Intl J RO Biol Phys, Perez-Stoler, P. J Clin Onc, 1984.

Surgical Tx

IR Treatment

IR Tx #2

IR Tx #3

IR Tx #4

Prognosis 4 Varies depending on the etiology 4 SVCS in its own right is rarely fatal % survive at least 2 years Ahman,F. J Clin Onc, 1984 Ostler, PJ. Clin Onc, 1997 Perez & Brady, 2004.

Prognosis 4 Reviewed 5052 patients tx at MIR 1/ / patients tx SVCS 2/2 malignancy 4 Lung Cancer 79%, Lymphoma 18%, Other 6% 4 XRT+/- chemotherapy Armstrong, B. Int J Radiot Onc Biol Phys, 1987

Prognosis Overall 4 Median Survial=5.5 months 4 1 year survival=24% 4 5 year survival= 9% Armstrong, B. Int J Radiot Onc Biol Phys, 1987

Prognosis-SCLC 4 1 year survival=24% 4 5 year survival= 5% Armstrong, B. Int J Radiot Onc Biol Phys, 1987

Prognosis-Lymphoma 4 1 year survival=41% 4 5 year survival=41% Armstrong, B. Int J Radiot Onc Biol Phys, 1987

Prognosis-NSLC 4 1 year survival=17% 4 2 year survival= 2% Armstrong, B. Int J Radiot Onc Biol Phys, 1987

Prognosis 4 No statistical difference in survival rates between patients treated with chemoradiation vs either tx alone 4 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 4 Depends on collateral circulation years GreenbergA. Ann Thorac Surg, 1985 Mahajan, V. Chest, 1975 Murdock, W. Scott Med J, 1960