SUPERIOR VENA CAVA SYNDROME &PANCOAST SYNDROME Dr Ramprasad Gorai

1. SUPERIOR VENA CAVA
SYNDROME
&
PANCOAST SYNDROME
DR.RAMPRASAD GORAI
2nd
yr PGT
DEPT OF PULMONARY MEDICINE
MEDICAL COLLEGE , KOLKATA

2. ANATOMY
 SVC formed by union of Right & Left
Brachiocephalic vein.
 Extent b/w 1st
to 3rd
costal cartilage
 receives the azygos vein immediately
before entering the pericardial sac
 Venous blood from head/neck/upper
extremities
 6 to 8 cm in length &1.5 to 2 cm wide

3. ANATOMY
• 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
“neighbours”

4. ANATOMY OF SVC

5. DRAINAGE AREA OF SVC

6. 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

7. 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

8. Clinical Features of SVC
SYMPTOMS
Facial swelling Headache
Short of Breath Diziness
Chest Pain Tinitus
Cough Brusting sensation
Dysphagia Nasal congestion
Horseness of voice
MEN 1st
SYMPTOM = collar size

9. Patients

10. Clinical Features of SVCS
SIGNS
Edema & Plethora of face, neck,
arm,ant chest wall.
Neck vein distension(non pulsatile)
Venous collateral over ant chest wall
cyanosis,conj congestion,Proptosis
CNS = headache ,visual
disturbance,papilledema
Downhill Esophageal varices
Pleural effusion 25%

11. Site of esophageal varices indicate
site of SVC obstruction
• Varices in upper 1/3
SVCO proximal to azygos vein
• Varices in entire esophagus
SVCO involve or distal to
azygos vein

12. Patients

13. • As obstruction develops, venous
collaterals form
• Alternate pathways for venous return to
the RA
• Severity of symp depends on the time
course of obstruction
• Mass b/w 1st
& 3rd
rib can cause SVCO
Applied antomy

14. Etiology of SVC
• Malignancy 90%
– Lung cancer
– Lymphoma
– Thymoma
– Metastatic
– Germ Cell
• “Benign” 10%
– Invasive technique
– Benign tumor
– Aortic aneurysm
– Goiter
– Thrombosis
– Fibrosing
mediastinitis
– Histoplasmosis
– Bechet syndrome

15. Malignancy
• Account for 90% of SVCS cases
• Lung Cancer
small cell M/C
squamaus cell
• Lymphoma
• Others
– Metastatic
– Thymoma
– Germ cell tumour

16. Benign
• 1st case of SVCS described by William
Hunter in 1757 Secondary to aortic
aneurysm

17. Benign Neoplasms
• Substernal thyroid
• Teratoma/Dermoid cysts
• Benign Thymoma
• Cystic hygroma

18. Iatrogenic(invasive procedures)
• Thrombus formation d/t venous
catheters
• PM implantation
• TPN lines
• Swan-Ganz catheters
• HD catheters

19. Diagnosis
• Chest radiograph
• Duplex ultrasound
• CT/MRI/MRV
• Venogram
• Radionuclide studies

20. Chest Radiograph
• CXR FINDINGS FREQUENCY
• Mediastinal Mass
• or Widening 59-84%
• Hilar LAD 19-50%
• Pleural Effusions 25%
• Normal chest X-ray does not exclude
SVCO

21. Venography
• Can give precise level of obstruction
• Less information on etiology of SVCS
• Requires larger contrast dose

22. CT/MRI/MRV
• Provide accurate info on location
obstruction
• Determine etiology of obstruction
• Info on the extent of collaterals
• Guide biopsy attempts

23. SVCS

24. Sagittal MRI

25. Tissue Diagnosis
Procedure
Sputum cytology
Bronchoscopy
LN biopsy
Mediastinoscopy
Thoracotomy

26. Which First---> Tx or Dx?
OLD CONCEPT
SVCO =ONCOLOGICAL EMERGENCY
can cause cerebral venous thrombosis
T.O.C= Very high dose RT 3000-4000
Rads for 4 days
NOW A DAYS
TISSUE DIAGNOSIS is given importance
before starting T/t(Prev chemo/RT alter
Histoplogy)

27. Treatment
• Conservative
• Chemotherapy
• Radiotherapy
• Surgery
• Interventional Procedures(Stent)

28. Treatment
CONSERVATIVE T/t
• Salt restriction
• Diuretics
• Steroids(shrink lymphoma mass;no role
in bronchogenic ca)
• Anticoagulant

29. T/t options
CHEMOTHERAPY
• Small cell ca
• Lymphoma
• Germ cell ca
RADIOTHERAPY
• Non Small cell ca
• Metastatic solid tumour

30. surgery
• intra vascular STENTING with
expandable venous stent (Gianturco or
Pulmaz) for poor risk pts not
responding to RT

31. Treatment in BENIGN Case
• Substernal goiter=Resection
• Aneurism=cardiopulmonary bypass
and resection
• Thrombophlebitis=antibiotics+anticoag
ulants+fibrinolytics(urokiase,streptokina
se)
• Fibrosing mediastinitis=medial
sternotomy& PTFE graft

32. Surgical Tx
BYPASS

33. PROGNOSIS
• Depends on ETIOLOGY,not on
SEVEARITY
• SVCO recur in 10-30% pts,palliated
with Intravascular self expanding
STENT

34. PANCOAST
SYNDROME

35. HISTORY
• First description of
superior sulcus
tumours was given
by
Edward Selleck Hare
in 1838.
• Henry Khunrath Pancoast
, a radiologist in
Philadelphia, first
described the
syndrome in 1932

36. Pancoast tumours arise in lung
apex, and may invade
• Parietal pleura.
• Endothoracic fascia.
• First, second and third ribs.
• C8, T1 & T2 nerve roots (as they pass
over first rib) of brachial plexus
(brachial plexopathy).
• Intercostal nerves.

37. Cont….
• Stellate (inferior cervical) ganglion and
paravertebral cervical sympathetic
chain (at or above T1 level).
• Subclavian vessels.
• Bodies and transverse processes of
adjacent vertebras.
• Endothoracic lymphatics

38. Staging
• Staging of NSCLC Pancoast tumor: At
least T3 disease
• Stage IIB (T3N0) or
• Stage IIIA (T3N1-2, or T4N0-1), or
• Stage IIIB (T4N2).

39. Pancoast syndrome is
characterized by
pain in shoulder, upper anterior chest
wall, interscapular region, or neck
Horner’s syndrome,
Wasting and weakness of the ipsilateral
intrinsic hand musculatures

40. PAIN
• pain in shoulder, upper anterior chest
wall, interscapular region, or neck and
• the pain characteristically radiates to
along ulnar aspect of the upper limb,
extending upto fourth and fifth digits
(C8 – T2 segmental distribution).

41. Horner’s syndrome
Horner’s syndrome, comprised of
• ipsilateral partial ptosis,
• meiosis,
• enophthalmos,
• anhydrosis, and
• loss of ciliospinal reflex, results from
invasion of the stellate ganglion and
paravertebral cervical sympathetic
chain.

42. Wasting and weakness hand
musculature
• Wasting and weakness of the ipsilateral
intrinsic hand musculature, and medial
forearm wrist and finger flexors,
sensory loss and parasthesias (tingling
& numbness) along medial border of
arm, forearm, fourth and fifth digits are
resulted from invasion of C8, T1 and T2
nerve roots of the brachial plexus

43. X – ray findings
• Unilateral apical cap of more than 5
mm.
• Asymmetry of bilateral apical caps of
more than 5 mm.
• Apical mass.
• Rib erosion.

44. Chest XRAY PA view

45. CXR PA

46. CT

47. Coronal CT

48. MRI pancoast

49. causes
• Non small cell lung carcinomas
(Squamous cell carcinoma) are most
common cause of Pancoast syndrome,
but only 5% of non small cell lung
carcinomas present as superior sulcus
tumors. D/d of Pancoast tumor:
• TB,
• Apical pleural plaque or thickening
(Pleural cap).

50. Causes of Pancoast
syndrome
• Bronchogenic carcinoma: M/C cause,
Squamous cell carcinoma
• Hematologic malignancies like
lymphoma.
• Solid tumour metastases.
• Infection – induced destructive
sclerosing fibrosis.
• Cervical rib.

51. TREATMENT
• The best form of treatment remains the
subject of controversy
• curative treatment possible .
• Patients with superior sulcus
pulmonary carcinoma should be
considered for surgery after appropriate
diagnostic evaluation.
• The perfect candidate has a carcinoma
restricted to the chest with T3N0M0
staging.

52. SURGERY
• Surgery is indicated in patients who
have very localized early disease
• Contraindications to surgery include
the following:
1. Extensive invasion of the neck,
brachial plexus, or vertebrae
2.Perinodal mediastinal extension
3.Peripheral metastases

53. Modern approach…
• Neo adjuvant
chemoradiotherapy(cisplatin &
etoposide –based) followed by
• surgical resection after 2-4 wks.
• Ultrasound-guided cervical nerve roots
ablation can be considered for patients
with intractable neuropathic pain
secondary to Pancoast tumor

54. THANK YOU