Paragangliomas are slow-growing, benign tumors that arise from paraganglion cells located along parasympathetic nerves in the head and neck. There are several types classified by their location, with carotid body and glomus jugulare tumors being the most common. Diagnosis involves imaging like CT/MRI/angiography to identify the vascular tumor. While surgery is the primary treatment, the tumors are challenging to remove due to their proximity to critical nerves and vessels. Radiation may be used for incompletely resected or recurrent tumors.

2. ParagangliomasParagangliomas
ByBy
Mohammed HusseinMohammed Hussein

3. IntroductionIntroduction
Glomus Tumors (Paragangliomas, Chemodectomas)
 Glomus Tumor: slow growing, benign tumor although locally
destructive (expansive, bone eroding) of the chemoreceptive cells
(paraganglion cells, neural crest in origin) distributed along
parasympathetic nerves in the base of skull, neck, and chest
 most common benign tumor of the temporal bone of adults
(rare in pediatrics)
 10% multiple
 <5% malignant degeneration
 1–3% associated with a paraneoplastic syndrome (paroxysmal
hypertension, headache, and palpitations) from secretion of
vasoactive catecholamines and other neuropeptides

4. HistologicallyHistologically
 Type I cells (chief) cellsType I cells (chief) cells are APUD type cells withare APUD type cells with
copious cytoplasm and large round or oval nuclei. Theircopious cytoplasm and large round or oval nuclei. Their
cytoplasm contains dense core granules that store andcytoplasm contains dense core granules that store and
release catecholamines.release catecholamines.
 Type II (sustentacular) cellsType II (sustentacular) cells are elongated cells thatare elongated cells that
closely resemble Schwann cells although their functionclosely resemble Schwann cells although their function
is not entirely clear.is not entirely clear.
 The two cell types are arranged into clusters with a coreThe two cell types are arranged into clusters with a core
of chief cells surrounded by sustentacular cellsof chief cells surrounded by sustentacular cells
embedded in a fibrous stroma.embedded in a fibrous stroma.
 These clusters make up the fundamental histologicThese clusters make up the fundamental histologic
structure (termed "structure (termed "ZellballenZellballen")")

5. Paraganglioma . Intracranial extension of a glomus jugulare tumour. Typical
cellular groups ("Zellballen") surrounded by a capillary network.

6. TypesTypes
Types:
1. Carotid Body: arise from carotid body, most common type,
typically does not involve the temporal bone
2. Glomus Tympanicum: arise from the promontory along the
course of Jacobson’s nerve, tympanic branch of CN IX, confined
to the middle ear space
3. Glomus Jugulare: arise from the region of the jugular foramen
4. Glomus Vagale: arise from paraganglia around the vagus nerve
at the base of skull
5. Laryngeal paragangliomas: arise from the superior laryngeal
paraganglia above the anterior part of the vocal folds near the
aryepiglottic fold.

7. Carotid ParagangliomasCarotid Paragangliomas
 Carotid paragangliomas are the most commonCarotid paragangliomas are the most common
paragangliomas of the head and neck (60%).paragangliomas of the head and neck (60%).
 Mean age at diagnosis is 45-50 years.Mean age at diagnosis is 45-50 years.
 A slightly higher female predominance persists .A slightly higher female predominance persists .
 Multicentric 10% of the time with bilateral carotid bodyMulticentric 10% of the time with bilateral carotid body
lesions being the most common combination inlesions being the most common combination in
multicentric lesions.multicentric lesions.
 Familial in 20% of cases in an autosomal dominantFamilial in 20% of cases in an autosomal dominant
fashion .fashion .
 Malignancy occurs in approximately 10%.Malignancy occurs in approximately 10%.

8. DIAGNOSISDIAGNOSIS
 a painless mass that is slow growing along the anteriora painless mass that is slow growing along the anterior
border of the sternocleidomastoid muscle.border of the sternocleidomastoid muscle.
 Very large lesions may present with vocal cord paralysisVery large lesions may present with vocal cord paralysis
or dysphagiaor dysphagia
 On examinationOn examination, the masses are freely mobile laterally, the masses are freely mobile laterally
however they are immobile in a cephalad -caudadhowever they are immobile in a cephalad -caudad
direction.direction.
 The mass may be pulsatile and a bruit may beThe mass may be pulsatile and a bruit may be
auscultated.auscultated.

9. RadiologyRadiology
 CTCT oror MRIMRI which shows a mass arising from thewhich shows a mass arising from the
carotid bifurcation and displaces the internal andcarotid bifurcation and displaces the internal and
external carotid arteries.external carotid arteries.
 The diagnosis is confirmed withThe diagnosis is confirmed with arteriographyarteriography
by revealing a characteristic tumor blush at theby revealing a characteristic tumor blush at the
carotid bifurcation called the lyre sign.carotid bifurcation called the lyre sign.
 Color duplex ultra soundColor duplex ultra sound can clearlycan clearly
demonstrates the rich vascularity of the tumordemonstrates the rich vascularity of the tumor

10. Doppler ultra sound of extensive paraganglioma that hasDoppler ultra sound of extensive paraganglioma that has
grown to involve the IJVgrown to involve the IJV

11. TREATMENTTREATMENT
 SurgerySurgery is the mainstay of treatment for carotid paragangliomas.is the mainstay of treatment for carotid paragangliomas.
 The recurrence rate is approximately 10% with the mortality rateThe recurrence rate is approximately 10% with the mortality rate
intraoperatively of up to 8%.intraoperatively of up to 8%.
 Because of their close approximation to important vessels andBecause of their close approximation to important vessels and
nerves, there is a real risk of morbidity (usually CN X-XII andnerves, there is a real risk of morbidity (usually CN X-XII and
vascular injuries).vascular injuries).
 Perioperative alpha and beta adrenergic blockers should bePerioperative alpha and beta adrenergic blockers should be
available for all catecholamine producing tumors.available for all catecholamine producing tumors.
 RadiationRadiation is usually reserved for incompletely excised tumorsis usually reserved for incompletely excised tumors
(with intracranial extension), recurrent tumors, or poor surgical(with intracranial extension), recurrent tumors, or poor surgical
candidates.candidates.

13. VagalVagal ParagangliomasParagangliomas
 arise most commonly at the level of the nodosearise most commonly at the level of the nodose
ganglion but may occur anywhere along theganglion but may occur anywhere along the
course of the vagus nerve in the neck.course of the vagus nerve in the neck.
 Mean age at presentation is 50 years and there isMean age at presentation is 50 years and there is
a slight female predominance.a slight female predominance.
 Relatively rare 3% of paragangliomas.Relatively rare 3% of paragangliomas.
 Multicentric in 25% of cases.Multicentric in 25% of cases.

14. VagalVagal ParagangliomasParagangliomas
 The cardinal symptom is vagus nerve palsy withThe cardinal symptom is vagus nerve palsy with
hoarseness and aspirationhoarseness and aspiration
 The patient may complain of tongue weaknessThe patient may complain of tongue weakness
or have a Horner’s syndrome.or have a Horner’s syndrome.
 a painless slow growing mass located behind thea painless slow growing mass located behind the
angle of the mandible that has been present forangle of the mandible that has been present for
many years.many years.

15. VagalVagal ParagangliomasParagangliomas
 CT or MRICT or MRI may delineate the tumor frommay delineate the tumor from
surrounding structures withsurrounding structures with angiographyangiography
classically demonstrating a tumor blush thatclassically demonstrating a tumor blush that
displaces the carotid artery anteriorly anddisplaces the carotid artery anteriorly and
medially.medially.
 Some argue that surgical treatment is theSome argue that surgical treatment is the
mainstay however cranial nerve deficits can bemainstay however cranial nerve deficits can be
expected with their removal. Radiation can beexpected with their removal. Radiation can be
used but responses are often suboptimal.used but responses are often suboptimal.

16. Laryngeal ParagangliomasLaryngeal Paragangliomas
 Paragangliomas of the larynx usually arise from theParagangliomas of the larynx usually arise from the
superior laryngeal paraganglia above the anterior part ofsuperior laryngeal paraganglia above the anterior part of
the vocal folds near the aryepiglottic fold.the vocal folds near the aryepiglottic fold.
 Hoarseness and dysphagia are the most commonHoarseness and dysphagia are the most common
complaintscomplaints
 are associated with high rates of malignancy.are associated with high rates of malignancy.
 Laryngeal lesion usually require wide local excision orLaryngeal lesion usually require wide local excision or
partial laryngectomy. Radiation has not been effectivepartial laryngectomy. Radiation has not been effective
in controlling these rare entities.in controlling these rare entities.

17. Temporal bone paragangliomasTemporal bone paragangliomas

18. Temporal bone paragangliomaTemporal bone paraganglioma
 These tumors are highly vascular and they deriveThese tumors are highly vascular and they derive
the blood supply mainly fromthe blood supply mainly from the ascendingthe ascending
pharyngeal artery.pharyngeal artery.
 It is claimed that they have a hereditaryIt is claimed that they have a hereditary
transmission as autosomal dominant traits withtransmission as autosomal dominant traits with
penetrance that increases with age.penetrance that increases with age.

19. Fish ClassificationFish Classification
Classification of the glomus tumors according to fischClassification of the glomus tumors according to fisch
Class AClass A: Glomus tympanicum: Glomus tympanicum
Class BClass B: Tympanomastoid: Tympanomastoid
Class CClass C: Glomus jugulare: Glomus jugulare
CICI: Carotid foramen: Carotid foramen
C2C2: Vertical ICA until genu: Vertical ICA until genu
C3C3: Horizontal ICA: Horizontal ICA
C4C4: ICA + FL: ICA + FL
Class DClass D: Intracranial extension: Intracranial extension
De (1-2):De (1-2): Intracranial extraduralIntracranial extradural
Di (1-2):Di (1-2): Intracranial intraduralIntracranial intradural
ICA = internal carotid artery; FL = anterior foramen lacerumICA = internal carotid artery; FL = anterior foramen lacerum

20. The class A tumor originates from glomus formations along the course of
Jacobson's nerve. They are localized to the middle ear

21. Glomus tympanicum class A tumor. The small red mass behind the
anteroinferior quadrant is localized on the promontory and does not extend
towards the hypotympanum

22. CT scan of the case presented in previous Figure. The lesion is limited to
the region of the promontory. There are no visible signs of bone erosion

23. Class A glomus tumor. The tumor is again limited to the promontory

24. CT scan of the Previous case

25. The class B tumor originates at the level of the promontory and invades the
hypotympanum without affecting the jugular bulb. The tumor also can extend into
the mastoid and the retrofacial air cells

26. Left ear. Class B glomus tumor or hypotympanic tumor. The reddish mass
is visible through the inferior quadrants of the tympanic membrane.

27. CT of type B glomus. Tumor extension towards the hypotympanum is
observed. There is no erosion of the bony plate covering the jugular bulb.

28. Right ear. Class B glomus tumor. The highly vascular red tumor mass pushes the
tympanic membrane laterally. A middle ear effusion is present.

29. Left ear. Type B glomus tumor. The tumor causes bulging of the posterior quadrants of the
tympanic membrane

30. CT of previous fig An axial section demonstrates the presence of effusion in
the mastoid due to retention. The tumor extends to the hypotympanum but
does not erode the bone overlying the dome of the jugular bulb.

31. The class C tumor originates in the dome of the jugular bulb and destroys
the infralabyrinthine compartment. The C1 tumor erodes the carotid foramen
without involvement of the carotid artery.

32. Class C1 glomus tumor. CT scan, coronal view showing enlargement of the jugular
foramen with extension of the tumor into the middle ear.

33. The class C2 tumor erodes the vertical carotid canal up to the carotid
genu.

34. Class C2 De2 glomus jugulare tumor .The marked erosion of the jugular foramen
and the vertical portion of the carotid canal can be appreciated.

35. The class C3 tumor involves the horizontal segment of the carotid

36. Right ear. Class C3 Di2 glomus jugulare tumor. MRI, sagittal view
demonstrating intradural extension of the tumor.

37. The class C4 tumor grows to the anterior foramen lacerum and extends
to the cavernous sinus. Class D indicates intracranial extension of the
tumor. This might be extradural (De) or intradural (Di).

38. DIAGNOSISDIAGNOSIS
 Pulsatile tinnitus (most common symptom), hearing
loss, cranial nerve palsies, aural discharge, otalgia,
dizziness (invasion of the labyrinth)
 reddish middle ear mass
 These tumors may blanch with positive pressure on
pneumotoscopy (Brown’s sign) or have cessation of
tumor pulsation with ipsilateral carotid artery
compression (Aquino’s sign).
 patients with glomus jugulare tumors may present with
dysfunction of jugular foramen contents (Vernet’s
syndrome, involving cranial nerves IX, X, and XI).

39. RadiologyRadiology
 CTCT with contrast andwith contrast and MRIMRI with gadoliniumwith gadolinium
allow exact definition of the tumor extension.allow exact definition of the tumor extension.
 Carotid and vertebralCarotid and vertebral angiographyangiography allowsallows
identification of the arteries supplying theidentification of the arteries supplying the
tumor; and they should be embolized beforetumor; and they should be embolized before
surgery to avoid excessive intraoperativesurgery to avoid excessive intraoperative
bleeding.bleeding.

40. The ascending pharyngeal artery (large arrow) is the dominant feeding vessel.
Tumor fills the jugular vein (triple arrow) and extends into the posterior fossa
(small single arrow).

41. which classwhich class????????????????

43. Left ear. A polypoidal pulsating red mass is seen in the external auditory canal. This
example has been included to emphasize the fact that biopsy of external auditory canal
polypi should never be taken without radiological investigations.

44. Paraganglioma of CPAParaganglioma of CPA

45. TREATMENTTREATMENT
 Surgical removal is the treatment of choice.
Preoperative embolization can decrease
operative morbidity.
 Radiation is usually reserved for patients with
concurrent medical problems or the elderly, who
may be at higher risk for surgical complications.

46. A, Angiogram blush before embolization. B, Successful embolization of this same tumor show.

47. Surgical approachSurgical approach
 Glomus Tympanicum:Glomus Tympanicum:
 Transcanal approach with drilling theTranscanal approach with drilling the
hypotympanumhypotympanum
 Posterior tympanotomy or extended facialPosterior tympanotomy or extended facial
recess approachrecess approach
 Radical mastiodectomyRadical mastiodectomy

48. INFRATEMPORAL FOSSA APPROACH.
 For access to the infratemporal fossa and the
jugular foramen, the infratemporal fossa
approach is available. Glomus jugulare and
vagale tumors
 A large curvilinear incision is made to allow
access to the lateral skull and the upper cervical
areas