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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. - PowerPoint PPT Presentation
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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. Thyroid gland
2. Trachea
3. Brachiocephalic artery
4. Common carotid artery
5. Internal jugular vein
6. 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. Group 1: small tumours, minimally attached. Surgical excision not difficult
2. Group 2: larger, moderate attachments. Can be resected, but many require temporary intra-luminal carotid shunt
3. Group 3: very large, encase carotid arteries. Often require arterial resection and grafting
Incidence
Sporadic More common 5% incidence of bilateral tumours
Familial 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
Neck or ear pain Local tenderness Hoarseness Dysphagia Tinnitus
Occasionally Cranial nerve dysfunction
Rarely 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 Documents the highly vascularised mass in the area of carotid bifurcation Tumour dimensions Co-existent carotid occlusive disease
Angiography Gold standard Identifies collaterals, concurrent atherosclerosis and multicentric disease
Dynamic or rapid sequencing CT 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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