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