Paraganglioma

1. Paraganglioma
DAPCIT
Current trends in the diagnosis nad management of head and neck
paragangliomas
Chetan S. Gujrathi and Paul J. Donald
Current opinion in Otolaryngology and Head and Neck Surgery 12:339-342. 2005

2. Definition
•Neuroendocrine neoplasm derived from paraganlia
composed of chief and sustentacullar cells arranged
in characteristic pattern (Zellballen).
•The correct terminology is based on location
–Carotid body tumour paraganglioma of the carotid body
–Glomus tympanicum/jugulare Jugulotympanic paraganglioma
–Glomus vagule Vagal paraganglioma

3. Aetiology
•Sporadic and familial
•Genetic:
–10-50% AD with maternal imprinting (no tumor when gene from
mother; paternal transmission result in tumor even father
unaffected)
–3 genes identified code for mitochodrial respiratory chain
protein, complex II, succinyl dehydrogenase
–SDHB; SDHC, SDHD. Located C/r 11
–Familial more likely to be multifocal (30% vs 4%)& less likely to
be malignant (2.5% vs 10%)
•? Chronic hypoxia

4. Epidimiology
•Rare; overall 1:30,000 of the H&N tumour
•Age 40-50, F>M
•Difference in geographic; increased incidence
in high attitude  chronic hypoxia?
•Median growth rate 0.8mm/yr
•Doubling time 7 years

5. Pathology
•Macro
–Firm, rubbery, well circumscribed mass with fibrous capsule
–Yellow/tan, pink read or brown appearance with areas of
fibrosis and haemorrhage
•Micro
–Zellballen
–Chief cell  catecholamine secreting cells
–Sustentacular cells supporting cells
–Surrounded by fibromuscular stroma/vessels
•Malignant and benign has same appearance!

6. Clinical
•Depends on the location of the tumour
–Cervical group
•Carotid body
•Glomus vagale
–Temporal bone
•Glomus jugulare
•Glomus tympanicum

7. Carotid body tumour
•D: Neuroendocrine tumour arising from
paraganglionic tissue adjacent to carotid
bifurcation
•E/A: rare, most common type of
paraganglioma; genetic
•P: zellballen (firm rubbery, yellow/tan, brown, red, pick
with fibross & haemorrhage)

8. Carotid body tumour
•Clinical
–Slowly enlarging painless mass deep to anterior
border of SCM below angel of mandible
–Fontaine’s sign
–Neural involvement  pain, dysphonia,
dysphagia, dysarthria, horner’s
–Secretion  headache, syncope
–Bruit/thrill
–(maybe bilateral or associated with other paraganglioma)

9. Carotid body tumour
•Investigation
–CT: homogenous,
hypervascular, well
defined strongly
enhancing mass at
acrotid bifurcation
with splaying of ICA
and EAC
–Lyre’s sign

10. Carotid body tumour
•MRI/MRA
–Well defined mass with
salf and pepper
appearance (esp >2cm)
–T1: low signal; T2 high
signal, contract
enhance

11. Carotid body tumour
•Angiography
–Used pre-op for embolisation/consider ballon occlusion
–Controversial
•Octreotide scintigraphy
–Detection of additional occult tumour
–Separate post-OP changes from residual to recurrent
disease
–Screening
•Urinary catecholamines

12. Carotid body tumour
•Treatment
–Surgery
•Preop emoblisation
•Control carotid above and below
–Radiotherapy
•If surgery in contraindicated
•Reduce size and slows growth
•Good response ? >90%
–No role in Chemo

13. Classification
•Shamblin
–I: non adherent
–II: adherent
–III : Encasting

14. Jugulotympanic
•D: neuroendocrine tumours arising from
paraganglia in vicinity of jugular bulb or on the
promontory of the middle ear
•E/A: most common middle ear neoplasm; 2
nd

most common neoplasm of temporal bone;
genetic/hypoxia
•P: zellballen (firm rubbery, yellow/tan, brown, red, pick with
fibross & haemorrhage)

15. Jugulotympanic
•Clinical:
–GT&GJ  otological sym (pulsatile tinnitis/hearing
loss/vertigo/bleeding
–GJ  Cn IX, X, XI
–Sytemic  if secretory or associated pheochromocytoma
–Brown’ sign
–Aquino sign
–CN deficit (compression or invasion) VII, VIII, XI, XII < IX & X
–SNHL  labyrithine invasion

16. Jugulotympanic
•Audiogram
•CT temporal bone/neck
–Air between tumour and
jugular bulb  glomus
tympanicum
–Erosion of
caroticojugular ridge 
glomus jugulare

17. Jugulotympanic
•MRI/MRA
•Angiography
•Urinary catecholamines (VMA,
metanephrines)
•Octreotide scintigraphy

18. Jugulotympanic
•Treatment
–Surgery
–Rtx
•Unfit for surgery
•Unacceptable functional deficit from surgery
–Extensive intracranial extension
–Carotid artery sacrifice
–Bilateral lower cranial nerve sacrifice
•Complication -> ORN, brain necrosis,
hypothalamic/pituitary dysfunction, 2ndary malignancy

19. Classification
•Fisch
•A: mesotympanum
•B: tympanomastoid without infralabrythine involvement
•C: carotid canal
–C1: limited involvement of vertical portion of caroitd canal
–C2: Invasion of vertical portion of carotid canal
–C3: Invasion of horizontal portion of caroitd canal not to foramen lacerum
–C4: extending to foramen lacerum +/- cavernous sinus
•D: intracranial
–De: extradural
•De1: displace posterior fossa dura <2cm
•De2: displace posterior fossa dura >2cm
–Di: intracrnial
•Di1: <2cm
•Di2>2cm

20. Classificaiton
•Glasscock-Jackson
•Tympanium
–I: Small mass limited to promontory
–II: completely filling midle ear
–Filling middle ear extending to mastoid process
–IV: filling middle ear extending into mastoid or EAC may extend to
anterior to ICA
•Jugulare
•I: Small, jugular bulb, middle ear and mastoid
•II: extending under IAC may have intracranial extension
•III: extending into petrous apex may have intracrnial extension
•IV: exteding beyond petrous apex into clivus or infratemporal fossa may
have intracranial extension

21. Fisch Approach
•A: Canalplasty
•B: transmastoid/ posterior tympanotomy
•C 1-2: Extended facial recess approach
•C 3-4: Combined transtemporal /
infratemporal approach
•D: combined transtemporal / neurosurgical
approach