Transcript Glomus jugular tumor.ppt

Glomus jugular tumor
Chunfu Dai M.D & Ph.D
Background
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Originate from chemoreceptor jugular vein
glomus jugulare tumor is arisen in the
adventitia of the dome of the jugular bulb
This tumor is part of the neuroendocrine
system, these tumors are highly
vascularized.
Glomus tumors represent 0.6% of
neoplasms of the head and neck
Pathology
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All paraganglia are composed of chief cells
(type I cells, ie, chemoreceptive cells) and
sustentacular cells (type II cells, ie,
supporting cells). Developmentally, both types
of cells are of neuroectodermal origin;
specifically, they arise from neural crest cells
The vast majority of glomus tumors are
benign and slow to grow with bone erosion.
Pathology
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Glomus jugulare tumors are typically located just
under the skull base, at the bulb of the internal
jugular vein. The tumors may spread superiorly
into the jugular foramen, causing CN IX, CN X,
and CN XI deficits.
The primary blood supply to jugulare tumors is
via the ascending pharyngeal artery. In addition,
the occipital and posterior auricular arteries can
contribute to vascularization.
Intraoperatively ligation of external carotid artery
can significantly reduce the hemorrhage
Symptoms
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Pulsatile tinnitus,
Conductive hearing loss, aural
bleeding, and aural discharge.
Sensorineural hearing loss,
vertigo, aural pain and
Cranial neuropathy. Such as it
can compress CN IX, X, and XI.
Symptoms
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Functioning tumors, which are rare, can
increase risk of mortality. These active
tumors secrete catecholamines, which can
lead to clinical manifestations of
hypertension, headaches, palpitations,
and tachycardia
Signs
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Positive Brown’s sign.
Aural polyp
Cranial nerves
involvement
Lab tests
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Routine laboratory studies are not helpful
In the rare patient with functioning lesions,
preoperative and postoperative
catecholamine measures may help
confirm successful resection of the lesion
Image study
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A combination of contrast-enhanced CT,
MRI, and angiography is ideal for proper
diagnosis and localization of the tumors.
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MRI
Contrast-enhanced CT
Angiography remains of paramount
importance if the diagnosis is obscure or if
embolization is planed.
CT
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Glomus jugulare tumors are
enhancing soft-tissue masses
at the skull base, but skullbase artifact can mask their
presence.
These tumors are seen within
the jugular foramen.
The demonstration of bone
erosion of the jugular foramen
and petrous apex is often a
key finding in the diagnosis.
Careful review of bone
windows is necessary.
MRI
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It can show intense tumor enhancement,
and is a key finding in the diagnosis.
A salt-and-pepper fine vascular pattern
can be seen in the tumors; this finding is
suggestive of intrinsic tumor
neovascularity, particularly on T2-weighted
images.
MRI
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Images show that enhancing soft-tissue
masses protrude both intracranially and
extracranially at the skull base.
Coronal imaging can show tumoral
relationships to the brainstem and skull
base, and deep cervical soft-tissue
structures
AG
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Demonstrating the feeding artery
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Jugulare tumors involve higher external carotid
branch vessels; the ascending pharyngeal, tympanic,
and occipital arteries dominate the arterial blood
supply.
Arteriovenous fistulae may be present.
Rarely, the internal carotid and vertebral arteries may
contribute feeders to the neoplasms.
Typically, these tumors are evaluated, with attention
paid to all potential feeding arteries.
Contraindication
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Biopsy is prohibitive for patient with
glomus jugular tumor
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Due potential hemorrhage
Surgical Intervention
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Surgery is the preferred method of treatment for
glomus tumors.
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Most paragangliomas are slow-growing and benign,
radiotherapy alone or no treatment at all is preferred
in elderly patients in whom the risks of surgery are
relatively high and the tumor is unlikely to cause
serious morbidity or mortality.
If the patient is young, surgery is the best option that
allows total cure.
Surgical Intervention
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Current techniques are highly successful
with relatively low morbidity rates: Blood
loss and CN neuropathies are the major
complications.
Depending on the specific type and
location of the tumor, different surgical
approaches are required.
Surgical Intervention
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Extensive facial recess approach
Surgical Intervention
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Mastoid –neck approach:
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tumors are confined to jugular foramen and
may extend to the middle ear and mastoid.
They do not involve the carotid artery or the
intracranial compartment.
Infratemporal fossa approach:
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tumors involve in carotid artery and transdural
tumor are far more extensive.
Radiotherapy
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Gamma-knife irradiation is used in patients who
are poor candidates for surgical excision or
embolization due to their age or disease state or
because of unacceptable morbidity.
This procedure is expensive, and clear
remission is not reported.
It can control the tumor and prevent it from
growing larger.
Embolization
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Embolization is a common technique used as
the lone treatment option or as a precursor to
surgical excision.
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Starving the lesion of its blood supply and inducing
necrosis..
embolization is often used to reduce blood loss, and it
has been proven to be highly effective.
Care must be taken to avoid inadvertent extracranialintracranial embolization and the subsequent the risk
of stroke