Download SUPERIOR VENA CAVA SYNDROME

SUPERIOR VENA CAVA
SYNDROME& MALIGNANT
SPINAL CORD COMPRESSION
By: Eman Mahmoud Abd El-Ghaffar
Shoaib
M.B.B.CH.
Resident of Clinical Oncology & Nuclear medicine,
Faculty of Medicine, Mansoura University
objectives
Anatomy and physiology of SVC.
 Etiology.
 Clinical presentation.
 Investigations.
 Management of SVC syndrome.
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Anatomy and Physiology
The superior vena cava is formed by the union
of the right and left brachiocephalic veins.
 It is located in the middle mediastinum, to the
right of the aorta and anterior to the trachea.
 The superior vena cava carries blood from the
head, arms, and upper half of the body to the
heart; it carries approximately one third of the
venous return to the heart.
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When the superior vena cava is obstructed, blood flows
through a collateral vascular network to the lower body
and the inferior vena cava or the azygos vein.
The superior vena cava is vulnerable to
obstruction due to the following factors:
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its strategic location in the visceral compartment of the
mediastinum, surrounded by rigid structures (such as
the sternum, trachea, right mainstem bronchus, aorta
and right pulmonary artery).
its thin, easily compressed walls.
the transport of blood at low pressures; and
completely circumscribed by the mediastinal
(subcarinal, perihilar and paratracheal) lymph nodes.
Superior vena cava syndrome
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Superior vena cava syndrome (SVCS) results
from obstruction of SVC and is a common
occurrence in cancer patients and can lead to
life-threatening complications such as cerebral
or laryngeal edema
Etiology
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Malignant causes
-Primary intrathoracic malignancies
-Metastatic disease
 Nonmalignant causes
Malignant causes
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Primary intrathoracic malignancies
Cause of 87%-97%.
The most frequent malignancy associated with the syndrome is
lung cancer, followed by lymphomas and solid tumors that
metastasize to the mediastinum.
Lung cancer ….SVCS develops in approximately 3%-15% of
patients with bronchogenic carcinoma, and it is four times more
likely to occur in patients with right- vs left-sided lesions.
Metastatic disease
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Breast and testicular cancers are the most common
metastatic malignancies causing SVCS, accounting
for > 7% of cases.
Metastatic disease to the thorax is responsible for
SVCS in ~3%-20% of patients.
Nonmalignant causes
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Thrombosis The most common nonmalignant cause of
SVCS in cancer patients is thrombosis secondary to
venous access devices.
cystic hygroma.
substernal thyroid goiter.
benign teratoma, dermoid cyst.
thymoma, tuberculosis, actinomycosis.
Syphilis.
pyogenic infections.
silicosis, and sarcoidosis.
Symptoms
• Dyspnoea
• Neck and facial swelling
• Head fullness / headache
• Trunk and arm swelling
• Cough
• Dysphagia
Signs
• Thoracic vein distension 65%
• Neck vein distension 55%
• Tachypnoea.
• Plethora 15%
• Facial / conjunctival oedema 55%
• Central / peripheral cyanosis 15%
• Arm oedema 10%
• Vocal cord paresis &Horner’s syndrome 3%
Neck vein distension
Investigations
• Assess for hypoxia
• CXR …… bulky mediastinal shadow,
pleural/pericardial effusion
• CT chest …. assess level of obstruction differentiate
between thrombosis and tumour differentiate between
compression and infiltration
• Venous angiogram
• Blood tests ….blood gases FBC, U&E, LFT. Clotting
screen. Serum calcium. Uric acid.
•
tumour markers…… Beta HCG, AFP, LDH, CEA,
CA15-3 .
a chest x-ray of a patient with small cell lung
cancer and superior vena cava syndrome. (b)
Contrast-enhanced CT scan in the same
CT chest
Pathology
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it is necessary to have histological confirmation
before starting treatment.
CT guided core biopsy: 90-100% positive histology
Mediastinal biopsy: 90-100% positive histology
Bronchoscopic biopsy: 60% positive histology
Sputum cytology: 40% positive histology
Management
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Management of the superior vena cava syndrome
associated with malignant conditions involves both
treatment of the cancer and relief of the symptoms of
obstruction
The median life expectancy among patients with
obstruction of the superior vena cava is
approximately 6 months; but estimates vary widely
according to the underlying malignant condition.
Management
Supportive Care and Medical Management
 Radiotherapy
 Chemotherapy
 Surgery
 Placement of an Intravascular Stent
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Supportive Care and Medical
Management
Dexamethasone 16 mg pO or 8mg b.d PO
• If unable to tolerate oral medication prescribe
Dexamethasone 16mg IV /24 hours.
 Analgesics as required but avoid
oversedation.
 If thrombosis is found, thrombolysis and
anticoagulation may be indicated
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Radiotherapy
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Its use requires a tissue diagnosis.
The majority of the tumor types causing the superior
vena cava syndrome are sensitive to radiotherapy.
complete relief of the symptoms occcur in 78% of
patients with small-cell lung cancer and 63% of those
with non–small-cell lung cancer at 2 weeks after ttt
with radiotherapy.
Improvement is often apparent within 72 hours.
Technique of RT
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3D Conformal RT
GTV…..defined on CT scan as mediastinal mass and
site of SVCO.
PTV….+1-2cm SM.
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2D RT
fied size 12x12cm &upper border is SSN
By anterior&posterior beams.
dose of radiotherapy
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2000 cGy /5ttt or 3000cGy/10ttt.
For chemosenstive tumers single fraction 4Gy
with chemotherapy give immediate palliation.
depend on…… the histological nature of the
tumor , whether the radiotherapy was or was not
combined with chemotherapy and whether the
therapeutic objective was palliative or curative.
Systemic Chemotherapy
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Complete relief of symptoms of vena caval
obstruction is achieved with chemotherapy in
approximately 80% of patients with nonHodgkin’s lymphoma or small-cell lung cancer
and in 40% of those with non–small-cell lung
cancer
Placement of an Intravascular Stent
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Percutaneous placement of an intravascular stent can
be done before a tissue diagnosis is available, it is a
useful procedure for patients with severe symptoms
such as respiratory distress that require urgent
intervention.
Stent placement strongly considered for patients with
mesothelioma, which tends not to respond well to
chemotherapy or radiation, and may also be
particularly useful when obstruction of the superior
vena cava is caused by a thrombus associated with an
indwelling catheter
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cyanosis is usually relieved within hours, and edema
resolves within 48 to 72 hours in most series (response
rate, 75 to 100%).
Complications …… infection, pulmonary embolus,
stent migration, hematoma at the insertion site,
bleeding, and, very rarely, perforation. Late
complications include bleeding and death
Surgery
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Surgical bypass grafting is infrequently used to treat
the superior vena cava syndrome.
The more common approach is sternotomy or
thoracotomy with extensive resection and
reconstruction of the superior vena cava
Thymomas …………….are relatively resistant to
chemotherapy and radiation than lymphomas, so
surgery is appropriate when the superior vena cava
syndrome is caused by thymoma.
Malignant spinal cord
compression (MSCC)
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Spine is the most common site of osseous metastases.
It is involved in up to 40% of all cancer .
The most common causes ….. breast cancer (29%)
lung cancer (17%), lymphoma, myeloma, prostate
cancer and sarcoma.
Thoracic spine is affected in more than 70% of
cases, followed by lumbosacral in 20% and cervical
in 10% of cases
Pathphysiology
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Almost all MSCC (98%) are caused by an epidural
compression by one of the following mechanisms
Vertebral bone metastasis grows into the epidural space and
compresses the spinal cord.
Para spinal mass grows through the neural foramina.
Metastasis in the vertebral body causes its collapse and bone
fragments are displaced in the epidural space.
venous plexus compression, which leads to oedema
of the spinal cord which cause increased pressure to the
small arterioles which results in diminished blood flow
causing ischemia of the white matter and, if this
continues long enough, cord damage.
Symptoms and signs
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pain …..
initial symptom in 96% of patients .
present weeks or even months before the development
of the true MSCC.
Pain is located at the level of compression and can be
present with or without the radicular component.
Backache worsened by movement, vertebral
compression, valsalva manoveure (the percussion of
vertebral bodies).
pain from MSCC can not be relieved by rest; actually
with lying down it worsens.
Symptoms and signs
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Neurologic impairment…
develops in 80% of patients. It usually involves the
lower limbs (thoracic spine involvement) and causes
motor weakness. Weakness can progress to paresis or
to paraplegia. Impairment
Sensory disturbances are present in 50% of the
patients.
Sympathetic involvement with loss of bowel and
bladder function (incontinence, impotence and or
retence) appears very late in the course of the disease
with the exception of conus medullaris involvement
Diagnosis
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MRI with or with out contrast …is the best
diagnostic modality is magnetic resonance. Provide
information on the three dimensional extension of
the tumour and is an essential tool for planning the
treatment .
plain radiographs and bone scans have some
importance in diagnosis of MSCC.
CT done in patients who have contraindications for
MRI .
MRI
Treatment
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Medical ttt.
Surgery.
Radiotherpy.
Others… Combined surgery and radiotherapy
Stereotactic Radiotherapy
Treatment
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Medical ttt…. Unless contraindicated (including a significant
suspicion of lymphoma) offer all patients with MSCC a loading
dose of at least 16 mg of dexamethasone as soon as possible
after assessment, followed by a short course of 16 mg
dexamethasone daily while treatment is being planned.
Continue dexamethasone 16 mg daily in patients awaiting
surgery or radiotherapy for MSCC. After surgery or the start of
radiotherapy the dose should be reduced gradually over 5–7
days and stopped. If neurological function deteriorates at any
time the dose should be increased temporarily.
Patients on steroids should be monitored carefully for
hyperglycemia, hypertension and electrolyte disorders.
All patients should receive H2 blockers for gastric protection.
Steroids must be tapered gradually.
Surgery(decompression)
The only method for immediate relief of MSCC.
 Indications:
- Bony fragment causing SCC, spinal instability, single
level disese with good bone stock above and below, when
need tissue diagnosis, progression during or after RT, prior
RT.
 Vascular tumors—including renal cell carcinoma, thyroid
carcinoma and hepatocellular carcinoma—that are
approached surgically may be considered for
preoperative embolization to diminish intraoperative
blood loss .
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Radiotherapy
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Indications:
-multiple levels of bony mets, poor performance
status,
contraindications of surgery, radiosestive tumers, limited life
expectancy<3month.
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Target:
GTV…vertebral or soft tissue mass seen on CT or MRI.
CTV…spinal canal +width of the vertebra +
One vertebra above and one below if planning based on MRI or,two
above and two below if based on CT planning.
 Depth….5cm in cervical,7 cm on thoracic .7-8cm on lumbar
vertebrae.
 Field arrangement…direct field on dorsal,two paralel
opposing fiels on CX and lumbar vertebrae.
Dose of Radiotherapy
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2000 cGy /5ttt or 3000cGy/10ttt .
Single fraction(8 Gy).
Studies showed no difference between single fraction
RT(8GY/1ttt) and usual fractionation(2000cGy or
3000cGy) in…..
releif of back pain, restoring ambulatiom , bladder
function and incidence of field recurrence SCC).
Recommendation…to use single fraction RT in patients
with MSSC with short life expectancy(less than 3
month)or in management of pain in patients with
established paraplegia for more than 24 hours.
others
Combined surgery and radiotherapy…..
RT start 2-3 ws after surgery till healing of the tissue.
 Stereotactic Radiotherapy…….
In patients who received prior radiotherapy .
In patients received 800cGy or 2000cGy, dose can
be continued to 2000cGY/8ttt with out harm the
spinal cord.
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