Post on 26-May-2015
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