This lecture is about the presentation,evaluation, investigation and management of patients presenting with Superior venacava syndrome

1. Superior Vena Cava Syndrome
(SCS)
Dr. Subhash Thakur
Clinical Oncologist, CMC, Bharatpur, Nepal
MD (PGIMER, Chandigarh)

2. Contents
 Introduction
 Physiology of SVC Obstruction
(SVCO)
 Aetiology
 Clinical Evaluation
 Investigations
 Treatment
Stenting in SVCO
Radiotherapy
Chemotherapy
Surgery
Thrombolytic and
Anticoagulation
 Summary

3. Introduction
 A range of signs and symptoms from compression of SVC or
associated greater veins (External Compression or Internal
Obstruction)
 Secondary to malignancies: 73-97%

4.  Rarely an emergency in absence of tracheal compression
 However, SVCO has an impact on prognosis
Median survival who receive treatment: 46 weeks
Median survival without treatment: 6 weeks

5. Physiology of Superior Vena Cava Obstruction
 Superior Venacava
Thin walled, compliant and easily
compressible vein
1/3rd of total venous return to heart
Head, neck and upper extremities

6.  SVC compression or Obstruction can
result in compromise of cardiac
output in acute setting, but within
few hours, collaterals develop
 Collateral vessels achieve steady state
blood return to the azygous vein or
inferior Venacava

7. Severity of Symptoms depend on:
 Degree of narrowing of SVC and
 Speed of Onset

8. Acute Setting

9. Elevated Venous pressure
Interstitial edema
Laryngeal and
Cerebral Edema

11. Aetiology
 Compression, Invasion or Thrombosis of
SVC
 Result of Inflammatory, Benign or
neoplastic processes
 Lung Cancer is the most frequent
malignant cause

12. Principle Causes of SVCO
 Lung Cancer (52 – 81%)
 Small cell cancer
 Non small cell cancer
 Diffuse large cell cancer
 Lymphoma (2 -20%)
 Lymphoblastic
 Metastatic Disease to Mediastinum
 Breast Cancer
 Germ Cell Cancer
 Gastrointestinal Cancer
 Others

13.  Primary Mediastinal Tumors
 Thymoma
 Sarcoma
 Melanoma
 Thymic Carcinoma
 Non-Malignant Causes
 Infectious diseases: syphilis, Tb, Histoplasmacytosis
 Central Thrombus and other iatrogenic causes
 Idiopathic fibrosing mediastinitis
 Congenital Heart Failure
 Goiter

14. Clinical Evaluation
 All patients with suspected SVCO should have thorough clinical history to assess
duration and speed of symptom onset
 History should also involve previous invasive procedures and malignancies
 Detail examination can rule common differentials like CHF and Cushing's
syndrome
 Careful examination of neurological system: subtle but life threatening due to
cerebral Edema

15. Clinical Evaluation
 Symptoms of SVCO
 Dyspnea
 Cough
 Facial edema
 Headache
 Nasal stuffiness
 Tongue swelling
 Hoarseness
 Stridor
 Signs of SVCO
 Jugular vein distension
 Upper extremity swelling
 Facial and upper body plethora
 Chemosis
 Mental status changes
 Lethargy, Stupor and coma
 Syncope
 Cyanosis
 Papilledema

16. Radiological Evaluation
 Chest X-ray
 Often abnormal
 Can identify superior
mediastinal masses or
mediastinal widening
 Hilar masses and pleural
effusion

17.  Contrast CT or MRI
 Gold standard
 Localize the level of SVCO and underlying pathology
 Tumor mass/size
 SVC diameter
 Length of stenosis/obstruction
 Evidence of SVCO thrombus
 Formation of collateral vessels

18. Tissue Biopsy
 Treatment is determined by underlying pathology
 Sub-acute setting, malignancy is suspected, tissue biopsy should be obtained
 Can be done via
 Bronchoscopy
 Endobronchial ultrasound
 Mediastinoscopy
 FNAC or excision biopsy
 CT guided biopsy

20. Treatment
 Treatment
 Stenting in SVCO
 Radiotherapy
 Chemotherapy
 Surgery
 Thrombolytic and Anticoagulation
 Head elevation and supplementary Oxygen while
obtaining investigations
 Steroids and diuretics are often used but their evidences
are not well studied
 Anxiolytic and morphine: initial supportive management

21. Stenting in SVCO
 Safe and effective with rapid resolution of SVCO symptoms
 Endovascular stenting relieves symptoms in 95% of patients with lung cancer
 Can be accompanied even if there is complete SVCO or thrombosis
 Are percutaneously delivered into Venacava under fluoroscopic guidance
 Available in two fundamental designs:
 Self expanding or
 expandable

22.  Immediate Endovascular Stenting
 Life threatening symptoms such as hemodynamic compromise, laryngeal edema or
cerebral edema
 Strongly recommended for patients with limited treatment approaches like
mesothelioma
 Questionable in chemo sensitive tumors like SCLC, Lymphoma and germ cell
tumors

23. Complications of stenting
 3 – 7%
 Infection
 Pulmonary emboli
 Pericardial tamponade
 Stent migration
 Perforation
 Bleeding
 Stent failure due to extrinsic
tumor compression, infiltration
of tumor through the stent or
thrombus

24. Radiotherapy
 An effective treatment modality for certain tumor types as an
 Initial intervention or
 Adjuvant treatment after stenting
 Subjective improvement is seen within 72 hours of initiation of therapy
 75% of malignancies associated SCS notice symptomatic improvement within 3 –
5 days, 90% in 1 week
 Objective response requires 1 – 3 weeks

25.  Dose: 30 Gray in 10 # or 50 Gray in 25 #
 For lymphomas, daily dose of 1.8 to 2 Gy is recommended and for lung cancers 2
to 3 Gy daily dose
 All locoregional diseases including hilar and supraclavicular region should be
treated with sufficient margin

26. Side effects of Radiotherapy
 Initial worsening of symptoms secondary to
 edema
 tumor necrosis with fever
 myelosuppression
 alopecia
 nausea, vomiting
 stomatitis
 esophagitis and
 infection

27. Failure of radiation therapy: Reasons
 Obstructive Thrombosis
 Tumor recurrence
 Radiation fibrosis
 Failure of development of collaterals secondary to fibrosis
 The mean post treatment survival is 6 to 7 months

28. Chemotherapy
 Treatment of Choice for
 Non Hodgkin lymphoma
 Germ Cell Tumors
 SCLC
 These tumors are exquisitely chemo sensitive
 Relief of symptoms: 80% of NHL, 77% in SCLC and 40% NSCL patients
 Symptoms usually improve within 1 – 2 weeks of treatment initiation

29. Targeted Therapy
 No data available till date

30. Surgery
 To bypass or resect tumors to decompress the venous system are effective in
selected patients
 However, invasive procedures in this predominantly palliative patients has very
limited role
 In patients with malignant Thymoma and Thymic carcinoma, surgery should be
evaluated as part of multimodal treatment strategy

31. Thrombolytics and Anticoagulation
 Benefit is unclear
 30 – 50 % of patients with SVCO have thrombosis at post mortem
 Experts recommendation: anticoagulation after thrombolysis to
 Prevent recurrence of thrombus and
 Reduce the incidence of pulmonary emboli
 Aspirin is often recommended after stent placement in absence of thrombus

32. Summary
 SCS is often clinically striking but rarely requires emergency intervention
 Treatment planning should be multidisciplinary
 Tissue biopsy is warranted to guide diagnosis and optimize treatment
 Life threatening symptoms or signs: intravascular stenting provide rapid relief
 In patients with malignancy, after stenting, radiotherapy/chemotherapy is
advised
 Chemo sensitive malignancies: Chemotherapy should be initiated