superior vena cava syndrome & pancoast syndrome
TRANSCRIPT
SUPERIOR VENA CAVA SYNDROME &PANCOAST SYNDROME
DR.RAMPRASAD GORAI2nd yr PGT DEPT OF PULMONARY MEDICINEMEDICAL COLLEGE , KOLKATA
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
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”
ANATOMY OF SVC
DRAINAGE AREA OF SVC
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
Clinical Features of SVC
SYMPTOMS Facial swelling Headache Short of BreathDiziness
Chest Pain TinitusCough Brusting sensation Dysphagia Nasal congestion
Horseness of voice MEN 1st SYMPTOM = collar size
Patients
Clinical Features of SVCS
SIGNSEdema & 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%
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
Patients
• 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
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
Malignancy
• Account for 90% of SVCS cases• Lung Cancer small cell M/C squamaus cell• Lymphoma• Others
– Metastatic– Thymoma– Germ cell tumour
Benign
• 1st case of SVCS described by William Hunter in 1757 Secondary to aortic aneurysm
Benign Neoplasms
• Substernal thyroid• Teratoma/Dermoid cysts• Benign Thymoma• Cystic hygroma
Iatrogenic(invasive procedures)
• Thrombus formation d/t venous catheters
• PM implantation• TPN lines• Swan-Ganz catheters• HD catheters
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%
• Normal chest X-ray does not exclude SVCO
Venography
• Can give precise level of obstruction• Less information on etiology of SVCS• Requires larger contrast dose
CT/MRI/MRV
• Provide accurate info on location obstruction
• Determine etiology of obstruction• Info on the extent of collaterals• Guide biopsy attempts
SVCS
Sagittal MRI
Tissue Diagnosis
ProcedureSputum cytologyBronchoscopyLN biopsyMediastinoscopy Thoracotomy
Which First---> Tx or Dx?
OLD CONCEPTSVCO =ONCOLOGICAL EMERGENCY can
cause cerebral venous thrombosis T.O.C= Very high dose RT 3000-4000
Rads for 4 days
NOW A DAYSTISSUE DIAGNOSIS is given importance
before starting T/t(Prev chemo/RT alter Histoplogy)
Treatment
• Conservative • Chemotherapy• Radiotherapy• Surgery• Interventional Procedures(Stent)
Treatment
CONSERVATIVE T/t• Salt restriction• Diuretics• Steroids(shrink lymphoma mass;no role
in bronchogenic ca)• Anticoagulant
T/t options
CHEMOTHERAPY• Small cell ca• Lymphoma• Germ cell ca
RADIOTHERAPY• Non Small cell ca• Metastatic solid tumour
surgery
• intra vascular STENTING with expandable venous stent (Gianturco or Pulmaz) for poor risk pts not responding to RT
Treatment in BENIGN Case
• Substernal goiter=Resection• Aneurism=cardiopulmonary bypass and
resection• Thrombophlebitis=antibiotics+anticoagu
lants+fibrinolytics(urokiase,streptokinase)
• Fibrosing mediastinitis=medial sternotomy& PTFE graft
Surgical Tx
BYPASS
PROGNOSIS
• Depends on ETIOLOGY,not on SEVEARITY
• SVCO recur in 10-30% pts,palliated with Intravascular self expanding STENT
PANCOAST SYNDROME
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
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.
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
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).
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
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).
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.
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
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.
Chest XRAY PA view
CXR PA
CT
Coronal CT
MRI pancoast
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).
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.
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.
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
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
THANK YOU