carotid body tumor ,glomus jugulare ,glomus tympanicum,glomus vagale ,laryngeal paragangliomas

1. Dr . Mamoon Ameen

2. Definitions
• Paragangliomas are neoplasms arising from
paraganglionic tissue located throughout the
body
• Paraganglia are aggregates of neuroendocrine
cells situated throughout the body, frequently
near nerves and blood vessels

3. NOMENCLATURE
• Glomus tumor
• Chemodectoma
• Nonchromaffin paragangliomas
• Paraganglioma preceded by the anatomic site (carotid
body paraganglioma (CBP), jugular paraganglioma,
tympanic paraganglioma, and vagal paraganglioma)

4. • Head and neck paragangliomas (HNPs) comprise only 3% of all paragangliomas
• 0.6% of all head and neck tumors.
• Typically occurs 4th and 5th decade.
• The most common locations for HNPs are the carotid body > jugulotympanic > vagal

5. Etiology
• Familial 10%
• Sporadic
• Genetics
• Mutations of the succinate dehydrogenase(SDH) gene
• HNPs are also associated with multiple endocrine
neoplasia (MEN) type 2A and 2B (which is related to
the RETgene), von HippelLindau disease (related to
theVHLgene)
• Chronic Hypoxia
• Present earlier(mean age 30
years)
• prone to the development of
multiple paragangliomas at
different sites
• More likely to develop
malignant tumor and
pheochromocytoma
• Male predominance

6. HISTOPATHOLOGY OF
PARAGANGLIOMAS
GROSSLY
• well encapsulated,
• Reddish brown in color
• Firm, rubbery consistency
• Highly vascular
• Locally invasive

7. HISTOPATHOLOGY OF
PARAGANGLIOMAS
Microscopic :
Type 1 (chief cells)
• Eosinophilic and polygonal to oval shaped
• Arranged in distinctive cell clusters called
(Zellballen) separated by a fibrovascular
stroma
Type 2 (sustentacular)
• scattered at the periphery with basophilic
affinity

8. Immunohistochemistry
Chief cells are positive for
• Chromogranin
• Synaptophysin,
• Neuron specific enolase
• Serotonin
The sustentacular cells are
• S-100 positive and
• Focally positive for glial fibrillary acidic protein
 Malignancy is not reliably detected on histopathological grounds
Malignancy only detected once tumor metastasizes to regional nodes or distantly

9. Functional Paraganglioma
• Type I cells (chief cells) within paragangliomas store and secrete catecholamine
• Incidence in HNPS 1%
• Higher with abdominal and pheochromytoma
• HNPs mainly store and secrete norepinephrine
• Can lead to classical symptoms of catecholamine excess(palpitations, diaphoresis and
headaches) associated with hypertension
• Patients suspected of having a secreting tumor should be screened with a 24hour urine
evaluation for metanephrine, normetanephrine, and vanillymandelic acid.

10. Natural course of paragangliomas
• The majority of paragangliomas are slow-growing, benign, but locally invasive and
destructive lesions
• Spread is centrifugal, following paths of least resistance, and can lead to extensive bony
destruction at the skull base
• Growth is slow , with a median increase in dimension of 0.83 mm/year
• 6–10% of HNPs are malignant
• Malignant paragangliomas have been reported in up to 24% of sinonasal paragangliomas

11. Carotid paraganglioma
• Most common HNPS
• Most common bilateral tumor
• Arise from carotid body which is small ovoid structure
located within the adventitia at the posterior aspect of the
carotid bifurcation.
• Typically present with a slowly enlarging, pulsatile, painless,
soft mass at the angle of the mandible
• The mass is expansile, mobile only laterally but not
vertically

12. Carotid paraganlioma
• Bruit may be heard over the mass and this can be
silenced with pressure
• Occasionally the mass may project into the lateral
oropharynx
• The Xth nerve, Horner’s syndrome
• Splays the bifurcation of the CCA
• Shamblin classification is commonly used to stage
carotid body tumors

13. Shamblin classification

14. Tympanic paragangliomas
• Arising from paraganglion cells of the tympanic plexus
in the middle ear
• distinguished from glomus jugulare by a lack of bony
erosion around the jugular bulb.
• Present typically with pulsatile tinnitus 80%
• Conductive hearing loss but can be sensorineural if the
labyrinth is involved by tumor
• Bleeding from the ear

15. Tympanic paragangliomas
On otomicroscopic
• Rising sun signs (reddish mass behind the TM)
• Aquino's sign (blanching of TM on gentle pressure on
ipsilateral carotid artery )
• Browns sign (blanching on shegalization )

16. Jugular paragangliomas
• Arise from paraganglia present on the jugular bulb
• It may extend to the middle ear and cause similar symptoms to tympanic paraganglioma
• 43 % of the patients had at least one cranial nerve deficit at presentation
• The most commonly affected nerves are the IXth and Xth
• 13 % had paralysis of all four lower cranial nerves(Jugular foramen syndrome)
• Horner's syndrome
• Intracranial extension (to the posterior cranial fossa via the medial wall of the jugular bulb)
is common in jugular paragangliomas

18. Fisch classification of jugulotympanic
paraganglioma

19. Vagal paragangliomas
• Account for 5% of all head and neck paragangliomas
• Originate in the vagus nerve either from superior , middle, and inferior vagal ganglia
• Female preponderance of 3:1.
• Most commonly nodose ganglion
• Tumon originating superiorly will often present with a dumbbell appearance with an intracranial
component in the posterior cranial fossa.
• Tumors originating inferiorly will extend into the poststyloid compartment of the parapharyngeal
space.
• Most commonly as a mass in the neck,
• lesions at the jugular foramen risk involvement of all four lowest cranial nerve
• while lesions originating at the nodose ganglion may only impair vagal function,

20. Vagal paragangliomas
• Rare
• 5% all H&N paragangliomas
• Most commonly nodose ganglion
• limited to cervical region
• Attached to skull base, or intracranial

21. Vagal paragangliomas
Presentation
• Neck mass, pulsatile tinnitus, pharyngeal mass, Hoarseness
• cranial nerve deficits at presentation X-28%, XII-17%, XI-11%, IX-
11%, VII-6%
• Displaces the ICA anteriorly

22. Laryngeal Paragangliomas
• Supraglottic or infraglottic
• Hoarseness, SOB, odynophagia
• Submucosal lesion
• May require tracheotomy

23. Laryngeal Paragangliomas
• Image all submucous laryngeal
lesions preoperatively

24. Evaluation of head and neck paragangliomas
• Detail history
• Complete head and neck examination
• Investigations

25. INVESTIGATIONS
Radiological investigations
• Plays a critical role in the diagnosis, management, and
surveillance of these tumors.

26. INVESTIGATIONS
HRCT scan and with I/V Contrast
• The particular strength of CT scanning is in the evaluation of paragangliomas of the
temporal bone, allowing accurate estimation of tumour size ,bony destruction and
detailed assessment of surgical landmark
• Paragangliomas classically appear as homogeneous masses with intense enhancement
following intravenous contrast administration

27. INVESTIGATIONS
MRI :
• Is complementary to CT scans in evaluating HNPs
• More valuable in assessing intracranial extension and soft tissues (for example in
detecting encroachment and encasement of the ICA
• Can distinguish tumor from surrounding inflammatory tissue or fluid (for example,
in the mastoid cavity)
• More effective at identifying small (5 mm) synchronous paragangliomas

28. INVESTIGATIONS
Angiography :
• Angiography plays an important role in evaluating the important feeder vessels
preoperatively prior to tumor embolization
• Carotid balloon occlusion test
• DSA can be beneficial for cases in which the diagnosis of a HNP remains in
question after CT and MRI,

29. INVESTIGATIONS
Ultrasonography
• Ultrasound has a role in the diagnosis and assessment of neck masses
• Cost-effective follow up of carotid and low vagal paragangliomas that are being
managed conservatively
• colour Doppler flow imaging will demonstrate the hypervascularity of paragangliomas

30. INVESTIGATIOS
Octreotide Scintigraphy
• paragangliomas express somatostatin receptors
• Octreotide is a somatostatin analogue that can be couple to a radioisotope such as In
Pentetreotide
• Octreotide scintigraphy has a sensitivity of 94–97% and a specificity of 75–82% for HNPs
• Benefits of octreotide scintigraphy are the ability to detect multicentric tumors and distant
metastases
• Used to follow up the after surgery or radiotherapy
• Screening technique for patients at risk of familial paraganglioma syndromes

31. INVESTIGATIOS
• Metaiodobenzylguanidine (MIBG) scanning
• F-DOPA-PET 18 (fluorinedihydroxyyphenylalanine positron
emission tomography) is arelatively new whole-body technique
that identifies neuroendocrine cells due to their uptake of
dopamine

32. INVESTIGATIONS
Other investigations
• 24-hour urinary collection of metanephrine, norepinephrine, epinephrine
,dopamine and vanillylmandlic acid .
• Audiogram
• FNAC

38. MANAGEMENT
Head and neck paragangliomas can be managed with
• Surgery
• Primary radiotherapy
• Observation
• Careful and open discussions with the patient regarding the risks and benefits of
surgery, radiation, and observation should occur
• Large, skull base HNPs require a multidisciplinary surgical team consisting of
otolaryngologists, neurosurgeons, and vascular surgeons, interventional radiologiST

39. Radiotherapy
• Radiotherapy does not usually lead to a reduction in tumor volume, but can halt tumor
progression
• Indicated for :
 Elderly patients
 Those with severe medical conditions,
 Multiple tumors,
 Extensive skull base, or intracranial involvement,
 cases in which resection would result in significant cranial nerve and/or
vascular injury

40. Radiotherapy
• Intensity modulated radiotherapy is now the mainstay fractionated radiation treatment
• Standard dosing is 45 Gy over 25 fractions
• Malignant HNPs receive higher doses of 64–74 Gy
• Hinerman et al. and Pemberton et al. showed 10year survival rates of 95% and 96%,
respectively
• Stereotactic radiosurgery recommended for small tympanic or jugular paragangliomas

41. Surgery
• Surgery for HNPs should be considered when the tumor can be excised without causing
additional significant neurovascular morbidity
• Surgery can also be considered in patients with jugular or vagal paragangliomas who
present with cranial nerve deficits and in whom surgery would not result in additional
significant deficits
• surgery should also be considered in young patients
• Policy of tumor removal at all costs is often inappropriate

42. Surgery
• Subtotal resection with cranial nerve preservation is being increasingly adopted as an
acceptable treatment plan
• Functioning tumors should have been identified preoperatively and appropriately
controlled prior to and during surgery.
• Preoperative embolization on the day prior to surgery has become a standard procedure in
larger paraganglioma

43. Carotid paraganglioma
– Transcervical approach with or with out mandibulotomy
– The incision should follow a skin crease 2 cm below the mandible
– Proximal and distal control with vessel loops
– Identification and preservation of neural structures if possible
– Dissection in Peri-adventitial, white line (plane of Gordon)
• Preparation for vascular reconstruction if necessary
• Routine shunting not recommended
-Use only in failed balloon occlusion

45. TYMPANIC PARAGANGLIOMAS

46. TYMPANIC PARAGANGLIOMAS
• Small tumor Fisch
• Anterior tympanotomy

47. TYMPANIC PARAGANGLIOMAS
• For a Class II - IV Glomus Tympanicum- a transmastoid approach
/Extended facial recess is elected.

48. JUGULAR PARAGANGLIOMA
• Infratemporal fossa approach to the jugular foramen
• Fisch C __Skull base approaches utilizing upper cervical
dissection,transmastoid approach ,infratemporal fossa approach
• Fisch D __Skull base approaches with posterior fossa craniotomy

51. VAGAL PARAGANGLIOMAS
• Lower lesions may be approached through the neck
• While higher lesions that extend to the skull base will require
an infratemporal approach

52. LARYNGEAL PARAGANGLIOMA
• The surgical treatment of laryngeal paragangliomas is conservative
• For tumor located in the false cord can be excised by a lateral pharyngotomy
approach
• In cases of larger tumours, supraglottic laryngectomy may be necessary
• Subglottic paragangliomas should be excised by a laryngofissure approach

53. Complications
• Hearing loss
• Facial nerve palsy
• Jugular foramen syndrome
• Hypoglossal nerve paralysis
• Horner syndrome
• CNS complications

54. Post op management & rehab
• Admitted to a well equipped HDU
• Cardiovascular stability should be monitored
• Early detection of cranial nerve palsies
• Eye protection is the first step in 7th nerve palsy
• Lower CN deficits resulting in speech & swallowing problems need a multidisciplinary approach

55. Observation
 Observation is an acceptable alternative to surgery in :
• NO lymphadenopathy suggestive of malignancy,
• Absence of rapid tumor growth,
• Resection would result in significant morbidity,
• Multiple tumors
• Patients who are poor surgical or radiation candidates
 Follow up imaging after 6 months and 12 months thereafter.

56. THANK YOU