Carotid Body Tumour
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Transcript Carotid Body Tumour
Carotid Body Tumour
Dr. Maha Al Marashi
KM. 34 Female
Elective admission for Right Carotid body
tumour excision
Had been referred initially to the vascular
service with bilateral carotid body tumours
Incidental finding with no local pressure
symptoms or systemic effects
KM. 34 Female
BGHx:
Left carotid body tumour embolizaion
Appendectomy as a child
Tonsillectomy as a child
KM. 34 Female
Medications:
Nil
Allergies:
Nil
KM. 34 Female
Family Hx:
Grandfather – Carotid body tumour
Brother – Carotid body tumour bilaterally
KM. 34 Female
Ultrasound scan neck
Evidence of bilateral
carotid body tumours of
the carotid bifurcation
consistent with carotid
body tumours.
Thyroid gland is normal.
No other abnormalities.
KM. 34 Female
Duplex scan of carotids
Bilateral masses in the
region of the carotid body at
the bifurcation of the
internal and external
carotids.
Right is smaller and more
vascular.
Left encases vasculature.
KM. 34 Female
Genetic screening:
KM. 34 Female
Right carotid body tumour excision
KM. 34 Female
Histology:
KM. 34 Female
Discharged home day 2 post op with no
complications
Simple analgesia and aspirin
For OPD follow up in 4 weeks.
Carotid body tumours
Anatomy
Bifurcation of the
common carotid artery
Right side coming of
the brachiocephalic
artery
Left side from arch of
aorta
Anatomy
1.
2.
3.
4.
5.
6.
Thyroid gland
Trachea
Brachiocephalic artery
Common carotid
artery
Internal jugular vein
Superior vena cava
Embryology
Derived from:
Mesodermal elements of the third branchial arch
Neural elements originating from the neural crest
ectoderm
Neural crests differentiate into forerunners of
paraganglionic cells
Paragangangliomas
Physiology
Chemoreceptors located in the
bifurcation of the common
carotid artery
Monitor changes in the oxygen
and CO2 content and pH of the
blood and rely that sensory
information to the hypothalmus
and brain stem to help them
control cardiovascular and
respiratory functions
Other cells in the carotid body
respond to blood temperature
and to certain chemicals, e.g.,
nicotine and cyanide.
Has extremely high blood flow
and oxygen consumption
Histology
Resemble the normal
architecture of the carotid
body
Highly vascular
Zellballen (cell nests)
“Sustentacular” cell
Epithelioid cell
Cytochemical techniques
have demonstrated:
Adrenaline
Noradrenaline
Serotonin
Classification
Chromaffin
Capable of producing catecholamines
Non-chromaffin
Initially, Carotid body tumours were thought to be non-chromaffin
paragangliomas
≤5% of carotid body tumours are endocrinologically active
May be part of the neurocristopathies e.g. MEN 1 & 2
Secondary tumours are common, including
phaeochromocytomas
Pathology
Only known pathology is neoplasia
Most common of the non-chromaffin
paragangliomas
Shamblin et al described the following anatomic
groups:
1.
2.
3.
Group 1: small tumours, minimally attached. Surgical
excision not difficult
Group 2: larger, moderate attachments. Can be resected,
but many require temporary intra-luminal carotid shunt
Group 3: very large, encase carotid arteries. Often require
arterial resection and grafting
Incidence
Sporadic
Familial
More common
5% incidence of bilateral tumours
Autosomal dominant
32% incidence of bilateral tumours
Men:Women = 1:1
Screening of family members recommended
Age
Range between 20-80
Most apparent in 5th decade
Biologic behaviour
Malignant potential
Cannot be predicted by histological markers
Made by presence of lymph nodes or metastases
Metastatic spread
In region of lymph nodes
Kidney, thyroid, pancreas, cerebellum, lungs, bones, brachial
plexus, abdomen and breast
Rate approximately 5%
Predictors
Severity of symptoms
Size at time of diagnosis
History
Painless swelling in neck at the angle of the mandible
Non-specific
Occasionally
Cranial nerve dysfunction
Rarely
Neck or ear pain
Local tenderness
Hoarseness
Dysphagia
Tinnitus
Lateralizing central neurological signs or symptoms
Neurosecretory
Dizziness
Flushing
Palpitations
Tachycardia and arrhythmias
Headache and photophobia
Diaphoresis
Examination
Neck mass below the angle of the mandible
Laterally mobile but vertically fixed
Non-tender, rubbery, firm and non-compressible
Often pulsatile
Bruit
Abnormalities caused by vagal or hypoglossal nerve
impingement
Horner’s syndrome (rare)
Palpate opposite side
Differential diagnosis
Lymphoma
Metastatic tumours
Carotid artery
aneurysm
Thyroid lesions
Submandibular salivary
gland tumours
Branchial cleft cysts
Investigations
Duplex scan with colour flow imaging
Angiography
Gold standard
Identifies collaterals, concurrent atherosclerosis and multicentric disease
Dynamic or rapid sequencing CT
Documents the highly vascularised mass in the area of carotid bifurcation
Tumour dimensions
Co-existent carotid occlusive disease
Differentiates between aneurysm and neoplasm
Size and extent
MRI
Demonstrates relationship of tumour to adjacent structures
Differentiate from other soft tissue lesions at base of skull
Size and extent
Management
Mainstay is complete surgical excision due to:
≥5% incidence of metastases
Unrelenting growth of unresected tumours
Early excision decreases incidence of cranial nerve and carotid
artery damage
Most are in Shamblin’s group 2 or 3 at time of clinical
presentation
Radiation for local control of residual or recurrent disease
Chemotherapy has no role
Pre-operative embolization
Pros: Decrease vascularity and improve safety
Cons: thrombosis of ICA or cerebral embolization
Prognosis
Carotid body tumours are slow growing and
exhibit benign characteristics
Can survive for long periods without surgical
intervention
Death due to asphyxia and intra-cranial
extension; Martin et al noticed death rate of
approximately 8% in untreated patients
Even after prolonged disease-free intervals,
local recurrence following surgical resection
described
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