SVC‌‌Syndrome‌ 

My‌‌name‌‌is‌‌Lucy‌‌and‌‌this‌‌Kaviya,‌‌our‌‌presentation‌‌today‌‌is‌‌on‌‌superior‌‌vena‌‌cava‌‌syndrome‌  ‌
 ‌
Definition‌‌
   ‌
SVC‌‌syndrome‌‌is‌‌defined‌‌as‌‌partial‌‌or‌‌complete‌‌obstruction‌‌of‌‌SVC‌‌which‌‌causes‌‌a‌‌group‌‌of‌‌
 
clinical‌‌signs‌‌and‌‌symptoms‌‌from‌‌venous‌‌congestion.‌  ‌
 ‌
Etiology‌‌
   ‌
So‌‌there‌‌are‌ ‌two‌ ‌main‌‌types‌‌of‌‌aetiology,‌‌malignant‌‌and‌‌non-malignant.‌   ‌
 ‌
Malignant‌  ‌
Malignant‌‌obstruction,‌‌which‌‌is‌‌the‌‌most‌‌common‌‌cause,‌‌can‌‌be‌‌due‌‌to‌‌direct‌‌invasion‌‌of‌‌a ‌‌
tumor,‌‌or‌‌external‌‌compression‌‌by‌‌an‌‌adjacent‌‌pathology‌‌in‌‌the‌‌right‌‌lung,‌‌lymph‌‌nodes‌‌and‌‌
 
other‌‌mediastinal‌‌structures.‌‌All‌‌this‌‌can‌‌lead‌‌to‌‌thrombosis.‌‌In‌‌some‌‌cases,‌‌both‌‌external‌‌
 
compression‌‌and‌‌thrombosis‌‌coexist.‌‌
   ‌
 ‌
An‌‌intrathoracic‌‌malignancy‌‌is‌‌responsible‌‌for‌‌60‌‌to‌‌85‌‌percent‌‌of‌‌cases,‌‌and‌‌SVC‌‌
 
obstruction‌‌is‌‌the‌‌presenting‌‌symptom‌‌in‌‌up‌‌to‌‌60‌‌percent‌‌of‌‌these‌‌cases.‌‌Non-small‌‌cell‌‌
 
lung‌‌cancer‌‌is‌‌the‌‌most‌‌common‌‌malignant‌‌cause‌‌of‌‌SVC‌‌syndrome,‌‌accounting‌‌for‌‌
 
approximately‌‌50‌‌percent‌‌of‌‌all‌‌cases,‌‌followed‌‌by‌‌small‌‌cell‌‌lung‌‌cancer‌‌(approximately‌‌25‌‌
 
to‌‌35‌‌percent‌‌of‌‌all‌‌cases)‌‌and‌‌non-Hodgkin‌‌lymphoma‌‌(10‌‌to‌‌15‌‌percent‌‌of‌‌cases).‌‌
   ‌
 ‌
Nonmalignant‌  ‌
- Thrombosis‌‌associated‌‌with‌‌intravascular‌‌device‌‌(eg,‌‌dialysis‌‌catheter,‌‌pacemaker‌‌
 
wire).‌T
‌ he‌‌overall‌‌incidence‌‌of‌‌this‌‌device-related‌‌SVC‌‌syndrome‌‌has‌‌risen,‌‌largely‌‌
 
because‌‌of‌‌their‌‌increased‌‌use,‌‌now‌‌accounting‌‌for‌‌20‌‌to‌‌40‌‌percent‌‌of‌‌case.‌‌
   ‌
- Other‌c‌ auses‌‌include‌‌postradiation‌‌fibrosis‌‌and‌‌the‌‌rest‌‌are‌‌listed‌‌on‌‌the‌‌slide‌  ‌
 ‌
Clinical‌ ‌features‌‌
   ‌
 ‌
The‌‌clinical‌‌features‌‌of‌‌SVC‌‌syndrome‌‌consist‌‌of‌‌4‌‌broad‌‌categories.‌‌Generalised‌‌oedema‌‌
 
affecting‌‌upper‌‌extremities‌‌and‌‌face‌‌which‌‌include‌‌prominent‌‌veins,‌‌JVP‌‌distention‌‌and‌‌
 
swelling.‌‌Consequences‌‌of‌‌neck‌‌congestion,‌‌consisting‌‌of‌‌airway‌‌compromise‌‌with‌‌
 
dyspnoea‌‌and‌‌vocal‌‌hoarseness‌‌and‌‌also‌‌raised‌‌intracranial‌‌pressure.‌‌Starting‌‌off‌‌with‌‌
 
headaches‌‌which‌‌can‌‌progress‌‌to‌‌visual‌‌impairment,‌‌confusion‌‌and‌‌mental‌‌obtundation.‌‌
 
These‌‌symptoms‌‌are‌‌all‌‌exacerbated‌‌by‌‌lying‌‌supine.‌  ‌
 ‌
Typically,‌‌the‌‌symptoms‌‌progress‌‌over‌‌a‌‌period‌‌of‌‌weeks‌‌but‌‌uncommonly‌‌can‌‌present‌‌with‌‌
 
rapid‌‌deterioration.‌  ‌
 ‌
The‌‌reason‌‌for‌‌why‌‌chronic‌‌onset‌‌produces‌‌a‌‌slower‌‌progression‌‌of‌‌symptoms‌‌is‌‌that‌‌
 
collateral‌‌veins‌‌are‌‌able‌‌to‌‌dilate‌‌and‌‌compensate‌‌for‌‌the‌‌occlusion.‌‌This‌‌relieves‌‌pressure‌‌
 
and‌‌delays‌‌symptom‌‌onset.‌‌It‌‌can‌‌take‌‌several‌‌weeks‌‌to‌‌accommodate‌‌the‌‌reduction‌‌in‌‌
 
blood‌‌flow‌‌through‌‌the‌‌SVC.‌‌There‌‌are‌‌4‌‌main‌‌venous‌‌systems‌‌involved:‌‌the‌‌azygos,‌‌internal‌‌
 
mammary,‌‌lateral‌‌thoracic‌‌and‌‌vertebral‌‌venous‌‌pathways.‌‌If‌‌the‌‌occlusion‌‌occurs‌‌too‌‌
 
quickly,‌‌there‌‌is‌‌not‌‌enough‌‌time‌‌for‌‌these‌‌collaterals‌‌to‌‌form‌‌resulting‌‌in‌‌more‌‌severe‌‌
 
symptoms.‌  ‌
 ‌

-  ‌
 ‌
 ‌
Diagnosis‌   ‌ ‌
 ‌
The‌‌investigations‌‌used‌‌for‌‌each‌‌patient‌‌is‌‌determined‌‌by‌‌whether‌‌they‌‌are‌‌stable‌‌or‌‌
 
unstable.‌‌Stable‌‌patients‌‌undergo‌‌CT‌‌with‌‌venography‌‌which‌‌can‌‌visualise‌‌the‌‌presence‌‌of‌‌
 
collaterals.‌‌An‌‌US‌‌can‌‌also‌‌be‌‌used‌‌to‌‌assess‌‌for‌‌thrombus‌‌in‌‌the‌‌jugular,‌‌subclavian‌‌and‌‌
 
axillary‌‌veins.‌‌Unstable‌‌patients‌‌should‌‌have‌‌a‌‌digital‌‌subtraction‌‌venography‌‌with‌‌or‌‌without‌‌
 
a‌‌stent.‌‌This‌‌is‌‌the‌‌gold‌‌standard‌‌for‌‌SVC‌‌obstruction.‌‌The‌‌main‌‌limitation‌‌to‌‌this‌‌procedure‌‌
 
is‌‌that‌‌extrinsic‌‌causes‌‌of‌‌compression‌‌cannot‌‌be‌‌evaluated.‌‌The‌‌alternative‌‌for‌‌contrast‌‌
 
allergy‌‌or‌‌not‌‌being‌‌able‌‌to‌‌gain‌‌venous‌‌access‌‌is‌‌MR‌‌venography‌‌which‌‌is‌‌equally‌‌sensitive‌‌
 
and‌‌specific.‌  ‌
 ‌
Grading‌‌of‌‌SVC‌‌syndrome‌‌
   ‌
 ‌
The‌‌grading‌‌system‌‌for‌‌the‌‌clinical‌‌features‌‌of‌‌SVC‌‌syndrome‌‌has‌‌been‌‌proposed‌‌by‌‌a‌‌study‌ 
by‌‌Yu‌‌et‌‌al.‌‌which‌‌is‌‌the‌‌one‌‌most‌‌commonly‌‌used‌‌in‌‌Australia‌‌to‌ ‌determine‌‌diagnostic‌‌
 
approach‌‌and‌‌management.‌‌It‌‌consists‌‌of‌‌grades‌‌0‌‌to‌‌5‌‌based‌ ‌on‌ ‌different‌‌severity‌ ‌of‌‌
 
symptoms.‌  ‌
 ‌
Another‌‌classification‌‌that‌‌is‌‌more‌‌popular‌‌in‌‌other‌‌parts‌‌of‌‌the‌‌world‌‌is‌‌the‌‌Stanford‌‌method‌‌
 
which‌‌classifies‌‌SVC‌‌obstruction‌‌using‌‌venography.‌  ‌
 ‌
Management‌  ‌
 ‌
We‌‌will‌‌focus‌‌on‌‌the‌‌management‌‌of‌‌malignant‌‌superior‌‌vena‌‌cava‌‌(SVC)‌‌syndrome‌‌(tumor‌‌
 
invasion,‌‌compression‌‌by‌‌tumor).‌  ‌
 ‌
The‌‌goals‌‌of‌‌management‌‌for‌‌malignant‌‌SVC‌‌syndrome‌‌are‌‌to‌‌alleviate‌‌symptoms‌‌and‌‌treat‌‌
 
the‌‌underlying‌‌disease.‌‌There‌‌are‌‌factors‌‌that‌‌affect‌‌the‌‌treatment‌‌choice‌‌and‌‌they‌‌include‌‌
 
the‌‌type‌‌of‌‌cancer,‌‌the‌‌extent‌‌of‌‌disease,‌‌and‌‌the‌‌overall‌‌prognosis,‌‌which‌‌is‌‌closely‌‌linked‌‌to‌‌
 
histology‌‌and‌‌whether‌‌or‌‌not‌‌prior‌‌therapy‌‌has‌‌been‌‌administered.‌‌Note‌‌that‌‌the‌‌avg‌‌life‌‌
 
expectancy‌‌is‌‌approx.‌‌6‌‌months‌‌but‌‌with‌‌a‌‌wide‌‌range.‌‌
   ‌

[the‌‌National‌‌Comprehensive‌‌Cancer‌‌Network‌‌(NCCN;‌‌for‌‌patients‌‌with‌‌advanced‌‌
 
malignancy‌‌and‌‌a‌‌life‌‌expectancy‌‌that‌‌is‌‌estimated‌‌in‌‌weeks‌‌to‌‌months)‌‌and‌‌the‌‌American‌‌
 
College‌‌of‌‌Chest‌‌Physicians‌‌(ACCP)‌‌for‌‌lung‌‌cancer.]‌  ‌

Initial‌‌management‌‌should‌‌be‌‌guided‌‌by‌‌the‌‌severity‌‌of‌‌symptoms‌‌assessed‌‌with‌‌the‌‌first‌‌
 
grading‌‌scale‌‌we‌‌talked‌‌about‌‌and‌‌the‌‌underlying‌‌malignant‌‌condition,‌‌as‌‌well‌‌as‌‌the‌‌
 
anticipated‌‌response‌‌to‌‌treatment.‌‌This‌‌slide‌‌and‌‌the‌‌print‌‌outs‌‌have‌‌an‌‌algorithmic‌‌approach‌‌
 
to‌‌diagnosis‌‌and‌‌management‌‌(found‌‌out‌‌Upto‌‌Date‌‌and‌‌modeled‌‌after‌‌one‌‌proposed‌‌by‌‌
 
clinicians‌‌at‌‌Yale‌‌University‌‌is‌‌provided).‌‌This‌‌is‌‌what‌‌we‌‌will‌‌be‌‌further‌‌discussing.‌‌
   ‌

Overview‌‌of‌‌definitive‌‌management‌‌plan‌‌
   ‌
 ‌
Patients‌ ‌with‌‌life‌‌threatening‌‌symptoms‌‌
   ‌
 ‌
In‌‌patients‌‌who‌‌present‌‌with‌‌life-threatening‌‌symptoms‌‌(central‌‌airway‌‌obstruction,‌‌severe‌‌
 
laryngeal‌‌edema,‌‌coma‌‌from‌‌cerebral‌‌edema,‌‌which‌‌all‌‌represents‌‌a‌‌true‌‌medical‌‌
 
emergency,‌ ‌the‌‌first‌‌priority‌‌is‌‌initial‌‌stabilization,‌‌secure‌‌airway,‌‌support‌‌breathing‌‌and‌‌
 
circulation).‌ ‌After‌‌that,‌‌these‌‌patients‌‌require‌‌immediate‌‌intervention‌‌using‌‌endovenous‌‌
 
recanalization‌‌with‌‌SVC‌‌stent‌‌placement‌‌as‌‌needed,‌‌to‌‌decrease‌‌the‌‌risk‌‌of‌‌sudden‌‌
 
respiratory‌‌failure‌‌and‌‌death.‌‌
   ‌

In‌‌the‌‌past,‌‌it‌‌was‌‌thought‌‌that‌‌immediate‌‌RT‌‌was‌‌the‌‌quickest‌‌way‌‌to‌‌relieve‌‌obstruction‌‌in‌‌
 
potentially‌‌life-threatening‌‌malignant‌‌SVC‌‌syndrome.‌‌However,‌‌immediate‌‌RT‌‌is‌‌no‌‌longer‌‌
 
considered‌‌the‌‌best‌‌option‌‌for‌‌most‌‌patients‌‌because‌‌a‌‌few‌‌reasons:‌  ‌

- Endovascular‌‌recanalization‌‌with‌‌or‌‌without‌‌stenting‌‌is‌‌a‌‌faster‌‌way‌‌to‌‌relieve‌‌
 
symptoms‌‌compared‌‌with‌‌RT,‌‌particularly‌‌for‌‌patients‌‌with‌‌life-threatening‌‌symptoms.‌‌
   ‌
- R
‌ T‌‌given‌‌prior‌‌to‌‌biopsy‌‌may‌‌obscure‌‌the‌‌histologic‌‌diagnosis‌‌particularly‌‌if‌‌this‌‌is‌‌
 
their‌‌first‌ ‌presentation‌‌and‌‌if‌‌the‌‌diagnosis‌‌is‌‌not‌‌certain.‌‌As‌‌an‌‌example,‌‌in‌‌one‌‌
 
study‌‌of‌‌19‌‌patients‌‌with‌‌symptomatic‌‌mediastinal‌‌masses‌‌who‌‌received‌‌emergency‌‌
 
RT,‌‌a‌‌histologic‌‌diagnosis‌‌could‌‌not‌‌be‌‌established‌‌in‌‌eight‌‌(42‌‌percent)‌‌from‌‌a ‌‌
biopsy‌‌obtained‌‌after‌‌such‌‌treatment.‌  ‌
- If‌‌RT‌‌is‌‌needed,‌‌it‌‌can‌‌be‌‌deferred‌‌until‌‌after‌‌severe‌‌symptoms‌‌have‌‌been‌‌relieved‌‌
 
through‌‌endovascular‌‌techniques,‌‌and‌‌a‌‌biopsy‌‌is‌‌secured.‌‌As‌‌we‌‌described‌‌
 
Symptomatic‌‌obstruction‌‌is‌‌often‌‌a‌‌prolonged‌‌process,‌‌developing‌‌over‌‌a‌‌period‌‌of‌‌
 
weeks‌‌or‌‌longer.‌‌Deferring‌‌therapy‌‌until‌‌a‌‌full‌‌diagnostic‌‌work-up‌‌has‌‌been‌‌completed‌‌
 
does‌‌not‌‌pose‌‌a‌‌hazard‌‌for‌‌most‌‌patients,‌‌provided‌‌the‌‌evaluation‌‌is‌‌efficient‌‌and‌‌the‌‌
 
patient‌‌is‌‌clinically‌‌stable.‌‌
   ‌

 ‌
Patients‌‌without‌‌life‌‌threatening‌‌symptoms‌  ‌
 ‌
So‌‌there‌ ‌are‌‌a‌‌few‌‌options‌‌for‌‌management.‌   ‌ ‌

Stent:‌‌‌The‌‌placement‌‌of‌‌an‌‌endovenous‌‌stent‌‌is‌‌useful‌‌even‌‌in‌‌the‌‌absence‌‌of‌‌
 
life-threatening‌‌symptoms.‌‌An‌‌endovenous‌‌stent‌‌is‌‌particularly‌‌appropriate‌‌for‌‌rapid‌‌
 
symptom‌‌palliation‌‌in‌‌patients‌‌with‌‌tumors‌‌for‌‌which‌‌response‌‌to‌‌chemotherapy‌‌and/or‌‌RT‌‌is‌‌
 
intermediate‌‌or‌‌poor‌‌(ie,‌‌Non‌‌small‌‌cell‌‌lung‌‌cancer‌‌and‌‌pleural‌‌mesothelioma),‌‌and‌‌for‌‌those‌‌
 
with‌‌recurrent‌‌SVC‌‌syndrome‌‌who‌‌have‌‌previously‌‌received‌‌systemic‌‌therapy‌‌or‌‌RT.‌‌F
‌ or‌ 
patients‌‌with‌‌thrombus,‌‌endovenous‌‌thrombolysis‌‌is‌‌first‌‌done‌‌to‌‌uncover‌‌the‌‌location‌‌and‌‌
 
extent‌‌of‌‌any‌‌venous‌‌stenosis,‌‌and‌‌the‌‌patient‌‌is‌‌anticoagulated.‌‌In‌‌general,‌E
‌​ ndovenous‌‌
 
treatment‌‌successfully‌‌relieves‌‌symptoms‌‌in‌‌90-95%‌‌of‌‌patients.‌‌
   ‌

Radiotherapy‌‌‌is‌‌widely‌‌advocated‌‌for‌‌SVC‌‌syndrome‌‌caused‌‌by‌‌radiosensitive‌‌tumors‌‌in‌‌
 
patients‌‌with‌‌other‌‌less-chemotherapy-sensitive‌‌malignancies‌‌who‌‌have‌‌not‌‌been‌‌previously‌‌
 
irradiated.‌‌Most‌‌of‌‌the‌‌malignancies‌‌causing‌‌SVC‌‌syndrome‌‌are‌‌radiation‌‌sensitive,‌‌and‌‌at‌‌
 
least‌‌in‌‌lung‌‌cancer,‌‌symptomatic‌‌improvement‌‌is‌‌usually‌‌apparent‌‌within‌‌72‌‌hours.‌‌In‌‌a ‌‌
systematic‌‌review,‌‌RT‌‌was‌‌associated‌‌with‌‌complete‌‌relief‌‌of‌‌symptoms‌‌within‌‌two‌‌weeks‌‌in‌‌
 
78‌‌of‌‌patients‌‌with‌‌SCLC‌‌and‌‌63‌‌percent‌‌of‌‌patients‌‌with‌‌NSCLC.‌   ‌

Despite‌‌these‌‌data,‌‌for‌‌most‌‌patients,‌‌stent‌‌placement‌‌is‌‌preferred‌‌over‌‌urgent‌‌RT‌‌for‌‌the‌‌
 
following‌‌reasons:‌  ‌

- Objective‌‌measurement‌‌of‌‌the‌‌change‌‌in‌‌vena‌‌caval‌‌obstruction‌‌may‌‌not‌‌parallel‌‌
 
measures‌‌of‌‌symptomatic‌‌improvement.‌‌In‌‌a‌‌study‌‌of‌‌autopsy‌‌patients,‌‌complete‌‌and‌‌
 
partial‌‌SVC‌‌patency‌‌was‌‌found‌‌in‌‌only‌‌14‌‌and‌‌10‌‌percent‌‌of‌‌patients‌‌after‌‌RT,‌‌despite‌‌
 
reported‌‌relief‌‌of‌‌symptoms‌‌in‌‌85‌‌percent.‌‌These‌‌data‌‌have‌‌led‌‌some‌‌to‌‌suggest‌‌that‌‌
 
the‌‌development‌‌of‌‌collateralization‌‌may‌‌have‌‌contributed‌‌more‌‌to‌‌symptomatic‌‌
 
improvement‌‌than‌‌the‌‌effect‌‌of‌‌RT,‌‌and‌‌to‌‌question‌‌the‌‌value‌‌of‌‌urgent‌‌RT‌‌in‌‌patients‌‌
 
with‌‌SVC‌‌syndrome‌‌from‌‌chemotherapy-sensitive‌‌malignancies.‌  ‌
- With‌‌RT,‌‌relief‌‌of‌‌symptoms‌‌may‌‌not‌‌be‌‌achieved‌‌for‌‌up‌‌to‌‌four‌‌weeks,‌‌and‌‌
 
approximately‌‌20‌‌percent‌‌of‌‌patients‌‌do‌‌not‌‌obtain‌‌symptomatic‌‌relief.‌  ‌
- Furthermore,‌‌the‌‌benefits‌‌of‌‌RT‌‌are‌‌often‌‌temporary,‌‌with‌‌many‌‌patients‌‌developing‌‌
 
recurrent‌‌symptoms‌‌before‌‌dying‌‌of‌‌the‌‌underlying‌‌disease.‌   ‌ ‌

Third‌‌option‌‌is‌c
‌ hemotherapy‌.  
‌‌ ‌

For‌‌patients‌‌with‌c
‌ hemotherapy-sensitive‌‌‌malignancies‌‌such‌‌as‌‌small‌‌cell‌‌lung‌‌cancer‌‌
 
(SCLC),‌‌non-Hodgkin‌‌lymphoma‌‌(NHL),‌‌or‌‌germ‌‌cell‌‌cancer‌‌and‌‌possibly‌‌breast‌‌cancer,‌‌
 
initial‌‌chemotherapy‌‌is‌‌the‌‌treatment‌‌of‌‌choice‌‌for‌‌patients‌‌with‌‌symptomatic‌‌SVC‌‌syndrome.‌‌
 
The‌‌clinical‌‌response‌‌to‌‌chemotherapy‌‌alone‌‌is‌‌usually‌‌rapid,‌‌and‌‌these‌‌patients‌‌can‌‌often‌‌
 
achieve‌‌long-term‌‌remission‌‌and‌‌durable‌‌palliation‌‌with‌‌standard‌‌treatment‌‌regimens.‌   ‌ ‌

Although‌‌‌surgical‌‌resection‌o
‌ f‌‌mediastinal‌‌tumor‌‌combined‌‌with‌‌SVC‌‌reconstruction‌‌is‌‌
 
rarely‌‌considered‌‌for‌‌treatment‌‌of‌‌SVC‌‌syndrome‌‌in‌‌view‌‌of‌‌its‌‌morbidity‌‌and‌‌mortality,‌‌and‌‌
 
the‌‌limited‌‌life‌‌expectancy‌‌of‌‌most‌‌patients‌‌who‌‌present‌‌with‌‌this‌‌complication,‌‌it‌‌could‌‌be‌‌
 
considered‌‌in‌‌selected‌‌cases‌‌of‌‌thymoma‌‌and‌‌thymic‌‌carcinoma‌‌as‌‌a‌‌component‌‌of‌‌a ‌‌
multimodality‌‌approach‌‌to‌‌treatment,‌‌and‌‌for‌‌patients‌‌with‌‌residual‌‌masses‌‌after‌‌treatment‌‌of‌‌
 
a‌‌germ‌‌cell‌‌tumor.‌‌
   ‌

 ‌