Paraganglioma

DAPCITCurrent trends in the diagnosis nad management of head and neck

paragangliomasChetan S. Gujrathi and Paul J. Donald

Current opinion in Otolaryngology and Head and Neck Surgery 12:339-342. 2005

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

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

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

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!

Clinical

• Depends on the location of the tumour– Cervical group • Carotid body• Glomus vagale

– Temporal bone• Glomus jugulare• Glomus tympanicum

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)

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)

Carotid body tumour

• Investigation– CT: homogenous,

hypervascular, well defined strongly enhancing mass at acrotid bifurcation with splaying of ICA and EAC

– Lyre’s sign

Carotid body tumour

• MRI/MRA– Well defined mass with

salf and pepper appearance (esp >2cm)

– T1: low signal; T2 high signal, contract enhance

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

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

Classification

• Shamblin– I: non adherent– II: adherent– III : Encasting

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; 2nd most common neoplasm of temporal bone; genetic/hypoxia

• P: zellballen (firm rubbery, yellow/tan, brown, red, pick with fibross & haemorrhage)

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

Jugulotympanic

• Audiogram• CT temporal bone/neck– Air between tumour and

jugular bulb glomus tympanicum

– Erosion of caroticojugular ridge glomus jugulare

Jugulotympanic

• MRI/MRA• Angiography• Urinary catecholamines (VMA,

metanephrines)• Octreotide scintigraphy

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

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

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

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