Clinicopathological features of jugular foramen lesions.

1. Clinicopathologic Features
of Jugular Foramen Tumors
James T. Kryzanski, M.D., and Carl B. Heilman, M.D.
The jugular foramen (JF) is an anatomically complex region where important bony, neural,
and vascular structures converge. Tumors are the primary cause of JF pathology and often
present therapeutic challenges. In this article we discuss the clinical and pathologic
aspects of JF tumors. These aspects include the classic JF clinical syndromes, imaging
characteristics of specific JF tumors, and gross pathologic and histopathologic features.
The three most common and important JF tumors, glomus jugulare tumors, meningiomas,
and schwannomas, are discussed in depth. A wide variety of tumors can occur in the JF.
Nonetheless, our current clinical and radiographic knowledge usually allows a clinician to
ascertain the pathology of a JF tumor preoperatively with a high degree of certainty. Doing
so allows the clinician not only to formulate the most effective treatment plan but also to
provide accurate and thorough preoperative counseling to patients harboring these serious
lesions.
Oper Tech Neurosurg 8:6-12 © 2005 Elsevier Inc. All rights reserved.
KEYWORDS jugular foramen, meningioma, schwannoma, glomus jugulare
The jugular foramen (JF) region is a complex anatomical
area that is an important though relatively uncommon
location for skull base tumors. Pathology in the JF may man-ifest
with dysfunction of only the traversing cranial nerves
(CN IX, X, XI). More commonly, however, surrounding
structures such as the middle ear, cerebellopontine angle, or
high retropharyngeal space are also involved. In the surgical
literature the term, JF tumor, usually refers not only to tu-mors
that arise in the foramen itself (eg, glomus jugulare
tumors) but also to tumors that arise in the jugular fossa
adjacent to the foramen such as jugular fossa meningiomas.
The differential involvement of the lower cranial nerves by
disease processes in and near the JF led to the characteriza-tion
of a number of JF syndromes.1 These syndromes must be
understood in context because the most common JF tumor,
glomus jugulare, rarely ever leads to one of them. When seen,
JF syndromes usually result from less common lesions like
schwannomas or metastases. Though termed JF syndromes,
these clinical pictures may result from lesions from the
brainstem nuclei to the extracranial retropharyngeal space
(Table 1).
JF (Vernet’s) syndrome: Unilateral involvement of CNs IX,
X, XI leads to loss of taste in the posterior third of the tongue;
hemianesthesia of the palate, pharynx, and larynx; and weak-ness
or paralysis of the vocal cords, palate, trapezius, and
sternocleidomastoid muscle.
Posterior lacerocondylar (Collet-Sicard) syndrome: Involve-ment
of the hypoglossal (CN XII) nerve in addition to CNs IX,
X, and XI leads to tongue atrophy and weakness or paralysis.
Posterior retropharyngeal (Villaret’s) syndrome: This syn-drome
has the same clinical picture as the Collet-Sicard syn-drome
with the addition of Horner’s syndrome, usually re-lated
involvement of the sympathetic nerves near the high
cervical or petrous internal carotid artery.
Allied syndromes: Several other syndromes involving lower
cranial nerve dysfunction also have been described. They are
usually the result of brainstem ischemic injury and rarely
because of tumor. In Avellis’s syndrome, vocal cord/palate pa-ralysis
(CN X) is associated with contralateral dissociative
anesthesia (spinothalamic tract). Schmidt’s syndrome involves
ipsilateral anesthesia of the pharynx and larynx (CN X), pa-ralysis
of the soft palate and larynx (CN X), and weakness or
paralysis of the trapezius and sternocleidomastoid muscles
(spinal CN XI). Tapia’s syndrome involves paralysis of the
ipsilateral tongue, pharynx, and larynx because of a lesion
affecting the motor nuclei of CNs X and XII. Finally, Jackson’s
syndrome involves ischemic nuclear or radicular injury to
CNs X, XI, and XII.
Many etiologies other than tumor and brainstem ischemia
must be considered in patients presenting with one of the JF
syndromes or a single lower cranial nerve deficit. Histori-cally,
foremost among these are leptomeningeal processes
such as tuberculosis and syphilis, which used to account for
almost half of the cases of JF or related syndromes.1 Other
Tufts-New England Medical Center Department of Neurosurgery, Boston,
MA.
Address reprint requests to James T. Kryzanski, M.D., Tufts-New England
Medical Center Department of Neurosurgery, 750 Washington Street,
Box 178, Boston, MA 02111. E-mail: jkryzanski@tufts-nemc.org
6 1092-440X/05/$-see front matter © 2005 Elsevier Inc. All rights reserved.
doi:10.1053/j.otns.2005.07.004

2. Clinicopathologic features of JF tumors 7
leptomeningeal processes include neurosarcoidosis and car-cinomatous
meningitis. Basilar skull fractures, retropharyn-geal
abscesses, jugular vein thrombosis, and aneurysms are a
few of the other rare causes of JF syndromes.
Imaging of JF Lesions
Advances in neuroimaging have greatly simplified the diag-nosis
of pathology involving the JF. With respect to tumors,
assessment with magnetic resonance imaging (MRI), com-puted
tomography (CT), and cerebral angiography can usu-ally
suggest the pathologic entity with a high degree of cer-tainty.
This has obvious advantages for preoperative decision
making, including the choice of approach, surgical goals,
need for preoperative embolization, and patient counseling.
The most common tumors involving the JF are glomus
tumors, meningiomas, and schwannomas.2 Less commonly
found are metastatic lesions, primary bone tumors, chordo-mas,
chondrosarcomas, and epidermoid tumors. Among the
three most common tumors of the JF, glomus tumors are by
far the most numerous. A comprehensive review of JF lesions
revealed 509 glomus jugulare tumors in comparison to 104
schwannomas and 34 meningiomas.3 Because glomus tu-mors,
meningiomas, and nerve sheath tumors compose most
JF lesions, the surgeon must have an intimate knowledge of
their imaging characteristics.4
This will be reviewed in the next article, but the salient
features for these tumors are listed in Table 2.
Clinicopathologic
Aspects of Individual Tumors
Meningiomas, schwannomas, and glomus jugulare tumors
compose more than 80% of tumors involving the JF in one
series2 and probably compose a higher proportion of tumors
whose primary involvement is the JF. Considering them in-dividually
is useful.
JF Meningiomas
Primary meningiomas of the jugular fossa and foramen are
rare tumors; fewer than 50 cases have been reported. Their
rarity is attested to by their absence from the authoritative
meningioma treatises by both Cushing and Eisenhardt5 and
Castellano and Ruggiero.6 The largest published series have
contained only eight patients,7,8 and in a series of 161 poste-
Table 1 JF and Allied Syndromes
Syndrome Neurologic involvement
JF (Vernet’s) syndrome CN IX, X, XI
Posterior lacerocondylar (Collet-Sicard) syndrome CN IX, X, XI, XII
Posterior retropharyngeal (Villaret’s) syndrome CN IX, X, XI, XII, sympathetic chain
Avellis’s syndrome CN X, spinothalamic tract
Schmidt’s syndrome CN X, spinal XI
Tapia’s syndrome CN X, XII
Jackson’s syndrome CN X, XI, XII
Table 2 Imaging Characteristics of Common JF Area Tumors
Tumor MRI CT Angiography
Meningioma Isointense to cortex on
T1- and T2-weighted
images, homogeneous
enhancement with
gadolinium, enhancing
dural tail, absence of
vascular flow voids
Jugular foramen is not
enlarged, occasional
calcifications, hyperostosis
Faint tumor blush
Glomus jugulare Salt and pepper
appearance, intense
enhancement with
gadolinium
Erosive enlargement of JF with
irregular bony margins
Extreme vascularity with obvious
tumor blush, AV shunting, JF
filling defect, occasionally
tumor extends intraluminally
up the sigmoid sinus or down
the jugular vein
Nerve sheath tumor:
schwannoma or
neurofibroma
Irregular enhancement
with gadolinium, high
signal intensity on T2-
weighted images
Smooth, scalloped
enlargement of the JF, low
signal density
Minimal if any tumor blush
Chondrosarcoma Slight enhancement with
gadolinium, high signal
intensity on T2-
weighted images
Erosive enlargement of JF with
irregular bony margins
Avascular mass without tumor
blush

3. 8 J.T. Kryzanski and C.B. Heilman
rior fossa meningiomas only seven lesions were classified as
JF meningiomas.9
The most important fact concerning the presentation of JF
meningiomas is that they often mimic the presentation of the
much more common glomus jugulare tumors. The most
common presenting signs and symptoms are hearing loss,
tinnitus, the presence of a middle ear mass on otoscopy, and
lower cranial nerve deficits.7,8,10-12 Variation in the presenting
signs and symptoms in the published series of JF meningio-mas
may reflect referral bias. The two largest series in the
otolaryngologic literature reported preoperative hearing loss
in 11 of 14 patients, tinnitus in 8 of 14, and a middle ear mass
in 11 of 14. Notably, lower CN (IX–XII) deficits were only
present in 4 of 14 patients and involved a single lower cranial
nerve; no patients had multiple lower cranial nerve syn-dromes.
Other presenting signs and symptoms were facial
weakness in 2 of 14 and dizziness in 5 of 14 patients.7,10
In the neurosurgical literature the preoperative lower CN
dysfunction was the most severe. The two largest neurosur-gical
series reported lower cranial nerve dysfunction in 10 of
15 patients, including two patients with Vernet’s syndrome
and one with Collet-Sicard syndrome.8,9 All published series
show that these tumors have a strong female predominance,
and the typical age for occurrence is in the fourth or fifth
decade of life.7,8,9,11
Anatomically, meningiomas historically termed JF may
arise in the foramen itself or more commonly in the jugular
fossa directly adjacent. There is no generally accepted ana-tomic
classification. One group8 divides meningiomas of the
JF area into three groups depending on their predominant
location relative to the jugular bulb: anterior, posterior, or
occlusive. These categories indicate a suprajugular, retro-jugular,
or transjugular surgical approach, respectively. Me-ningiomas
arising in the jugular fossa resemble the typical
sessile, well-circumscribed, solid lesions seen throughout the
posterior fossa. Meningiomas that arise in the JF itself have a
more invasive growth pattern with intraosseous growth into
the skull base.7,10 The hypotympanum and middle ear7 are
often invaded. The posterior fossa component varies from a
small nodule to a large tumor mass causing symptomatic
brainstem compression.
Extracranially, tumor extension is usually limited to the
carotid space where carotid artery encasement and jugular
vein occlusion are common.10 CN involvement depends on
the exact origin of the tumor and the growth pattern and
tends to be significant. In one series, the incidence was 50%
of the patients.7 This involvement is likely the cause of high
rates of new postoperative lower cranial nerve deficits seen in
operative series, ranging from 58% to 68% for each lower
cranial nerve (CN IX-XI) in one review.3
Though meningiomas have been histologically divided
into numerous subtypes, the World Health Organization
(WHO) has adopted a three-grade system with respect to
aggressive behavior and likelihood of recurrence.13 Review of
the published series’ and reports reveals the meningothelial
(WHO grade I) variant to be the most common. In 30 re-ported
cases where histopathologic subtype was included,
there were 27 WHO grade I tumors (17 meningothelial me-ningiomas,
five transitional meningiomas, five psammoma-tous
meningiomas, one WHO grade II (atypical meningi-oma),
and two grade III tumors (one anaplastic meningioma
Figure 1 Setting sun sign as seen on otoscopy of the external ear
canal. When a glomus jugulare tumor (asterisk) extends to the mid-dle
ear, a vascular tumor can sometimes be evident behind the
posterior inferior aspect of the tympanic membrane. S, superior; A,
Anterior; I, Inferior.
and one malignant meningioma).7,8,11 Although most JF me-ningiomas
are low grade, their tendency to invade bone and
surrounding neural structures makes their resection a chal-lenge.
Among the three tumors that commonly arise in the JF,
meningiomas have the worst prognosis for postoperative cra-nial
nerve function and recurrence.
Glomus Jugulare Tumors
Most tumors arising primarily in the JF are glomus jugulare
tumors. Glomus tumors in the head and neck (jugulare, tym-panicum,
and vagale) share a common cell of origin, patho-logic
appearance, and the small possibility of symptomatic
catecholamine secretion. The clinical presentations of head
and neck glomus tumors vary according to their location.
Glomus jugulare tumors arise in the paraganglionic tissue
lining the jugular bulb and are usually clinically silent when
small and readily diagnosed when large. They grow insidi-ously
and seldom become symptomatic until they involve the
middle ear.
In several large clinical series (50 patients), middle ear
findings have been the most common; hearing loss has
ranged from 63% to 91% and pulsatile tinnitus from 52% to
73%.14-17 In one series all 231 patients had at least one of
those two findings.15 Hearing loss is usually conductive as a
result of tumor in the middle ear, but it can also be sensori-neural
reflecting invasion of the inner ear. Other signs and
symptoms of ear involvement are common and include aural
pain, discharge, bleeding, vertigo and a vascular mass visible
behind the tympanic membrane (Fig. 1).
Next to hearing loss, cranial neuropathies are the most
common neurologic manifestations of glomus juglare tu-mors.
The facial nerve is most commonly affected, ranging
from 21% to 33% of patients.14,16,17 Lower cranial neuropa-thies,
found in 10% to 30% of patients,14,16 usually occur in
extensive tumors and are often well compensated for because
of a slow onset. Extensive tumors occasionally cause Horner’s
syndrome through carotid involvement or CN V and CN VI
palsy from intradural or extradural growth.18 Glomus jugu-

4. Clinicopathologic features of JF tumors 9
lare tumors may also cause venous hypertension and elevated
intracranial pressure from extensive unilateral or bilateral
involvement of the jugular bulb.19 Finally, catecholamine se-cretion
can be demonstrated in about 4% of glomus jugulare
tumors through 24-hour urinary collection of vanillylman-delic
acid, metanephrines, and catecholamines.16,20,21 Al-though
rarely symptomatic preoperatively, this deficit can
lead to perioperative hemodynamic instability.
Anatomically, glomus tumors in the temporal bone are
usually divided into glomus jugulare and glomus tympani-cum
tumors. This distinction is likely because of clinical dif-ferences
between the tumors more than to the actual distri-bution
of glomus bodies in the temporal bone. Rockley and
Hawke22 studied the anatomic distribution of glomus bodies
in the temporal bone. They suggested that tumors arising
from glomus bodies adjacent to the jugular bulb or proximal
to Jacobsen’s nerve in the inferior tympanic canaliculus
would enlarge into the jugular bulb and present as glomus
jugulare tumors. In contrast, tumors arising from Jacobsen’s
nerve or the promontory would present as glomus tympani-cum
tumors.
Glomus jugulare tumors usually arise in the lateral com-partment
of the jugular bulb and displace the cranial nerves
medially.3 They tend to spread first to the temporal bone and
middle ear. From there they may spread to involve adjacent
structures through preformed pathways: Down the eusta-chian
tube into the nasopharynx, along the carotid artery or
through the tegmen tympani to the middle fossa, along the
jugular vein or hypoglossal canal to the posterior fossa, or
through the round window and internal auditory canal to the
cerebellopontine angle.17 Several anatomic classifications of
glomus jugulare tumors categorize low-grade tumors as those
limited to the jugular bulb, middle ear, and mastoid whereas
high-grade tumors display intracranial or intradural inva-sion.
16,23
Glomus jugulare tumors display a strong female:male pre-dominance—
between 3:1 and 6:1 in large series—and most
often manifest in the fourth or fifth decade.14-16 In general,
head and neck paragangliomas will be multiple in 3% of all
patients and in 26% of patients with familial tumors.24 There-fore,
a radiological evaluation of the side contralateral to a
known glomus jugulare tumor must be performed as a cru-cial
part of the diagnostic work-up.
Familial occurrence of paragangliomas was noted as early
as 1933.25 Current evidence supports an autosomal domi-nant
pattern of inheritance consistent with genomic imprint-ing
where the gene does not result in the development of
tumors when maternally inherited.26 Screening after the age
of 16 is therefore recommended among family members of
paraganglioma patients.
Glomus jugulare tumors share identical histopathologic
characteristics to paragangliomas in other head and neck lo-cations.
These features include the ‘Zellballen’ appearance of
chief cell clusters surrounded by thin-walled capillaries and
an extensive reticulin network. Electron microscopy reveals
dense secretory granules in the cytoplasm of these cells iden-tical
to those in catecholamine-producing tissue. Histochem-ical
analyses also have identified norepinephrine in tumor
specimens.27 Tumors may have various numbers of mitoses
or degrees of nuclear atypia, but these changes have not been
associated with clinically significant behavior. Glomus jugu-lare
tumors usually exhibit slow growth. Rarely, however,
these tumors grow aggressively and may even demonstrate
regional and distant metastasis.28,29
JF Schwannomas
Like meningiomas, JF schwannomas are relatively rare tu-mors
accounting for only 2.9% of all intracranial schwanno-mas.
30 More than 100 cases have now been reported in the
surgical and radiosurgical literature.30-41 Before the advent of
MRI and high-resolution CT, most patients with JF schwan-nomas
were thought preoperatively to have vestibular
schwannomas or glomus jugulare tumors because their
presentations can be similar.31
The presentation of a JF schwannoma usually depends
most on the anatomic location of the tumor. Predominantly
intracranial tumors with slight involvement of the JF tend to
present as a cerebellopontine angle mass associated with sen-sorineural
hearing loss, tinnitus, vertigo, and ataxia and often
no demonstrable lower cranial nerve deficit.31 Radiographi-cally,
these lesions can be mistaken for vestibular schwanno-mas
although they can usually be distinguished by careful
review of imaging studies. Like vestibular schwannomas,
they can displace the facial nerve significantly. According to
some authors,31 preoperative facial weakness is rare but it has
been present in 20% to 25% of patients in some large se-ries.
32,34 Predominantly extracranial tumors or those cen-tered
in the JF usually manifest with slowly progressive lower
cranial nerve deficits and may cause a classic JF syndrome.
They also may extend into the middle ear leading to a middle
ear mass and conductive hearing loss. A palpable neck mass
may be present.
Considering all JF schwannomas, the most common pre-sentations
are hearing loss and lower cranial nerve deficits.
Hearing loss is present in as many as 60 to 75% of pa-tients.
31,32,34 Hoarseness and swallowing difficulties are the
most common symptoms of lower cranial nerve dysfunction.
Preoperative lower CN (IX–XII) deficits have been present in
50% to 81% of large series31,32,34 although the incidence of
CN IX deficits may be underreported. Other initial findings
include facial numbness or pain, long tract signs, papille-dema,
42 nystagmus, and diplopia.
For intracranial schwannomas as a whole, a 2:1 female-to-male
predilection has been described.43 In large series of JF
schwannomas, however, gender distribution has been rela-tively
equal34 or even favoring a male predilection.32,35 Age of
presentation is usually the fourth through sixth decades but
outlying cases presenting in the second or seventh decade
have been reported.31,34,38
JF schwannomas may be predominantly intracranial, ex-tracranial,
or foraminal. They also may assume a dumbbell
shape and involve all of these areas. This distribution has
been hypothesized to result from variations in the tumor’s
point of origin on the lower cranial nerves.44 Thus, tumors
originating on the proximal nerve expand into the posterior
fossa. Tumors in the midportion of the nerve expand into the
bone of the JF, and tumors of the distal nerves expand ex-tracranially.
These patterns of growth have led to several anatomic clas-sifications
for JF schwannomas. Kaye and co-workers31 clas-sified
these tumors into three groups: primarily intracranial

5. 10 J.T. Kryzanski and C.B. Heilman
(type A), primarily involving bone (type B), and primarily
extracranial (type C). A type D was added to this classification
by Pellet and co-workers45 to denote dumbbell-shaped tu-mors
involving all of these areas. This expanded classification
was also used by Samii and co-workers,32 Franklin and co-workers
also classified JF schwannomas according to a glo-mus
jugulare system, which includes categories based on
intracranial tumor size and involvement of the carotid artery.
This system was subsequently modified by Mazzoni and co-workers34
to account for the size of any neck component.
The exact cranial nerve of origin was discussed in two large
series. In a review of 45 JF schwannomas by Hakuba and
co-workers38 the cranial nerve of origin was found to be the
CN IX, X, and XI complex in 19 cases, CN IX alone in 11, CN
IX or CN X in 6, CN X in 4, and CN XI in two patients. In a
series of 16 cases, Samii and co-workers32 found the CN
IX-XI complex to be the origin of the tumor in two patients,
CN IX alone in 7, CN X in 6, and CN XI in one patient.
The histopathology of JF schwannomas is similar to that of
other intracranial schwannomas and does not vary with tu-mor
location.31 Histopathologic features of intracranial
schwannomas include areas of compact spindle-shaped
neoplastic Schwann cells with occasional nuclear palisading
(Antoni A pattern), which alternate with less cellular lip-idized
tumor areas (Antoni B pattern). Verocay bodies, par-allel
arrays of tumor cell nuclei and cell processes, are often
found within Antoni A areas. Occasional mitotic figures may
be seen but do not indicate malignancy.
Grossly, JF schwannomas may be solid or cystic. Kaye and
coworkers31 noted two of their 13 cases were predominantly
cystic tumors and both were primarily intracranial lesions.
Carvalho and coworkers36 described five cystic tumors
among 21 cases of JF schwannomas. These tumors occurred
in younger patients and also were predominantly intracranial
tumors; all extended to the cerebellopontine angle and com-pressed
the brainstem. They further subdivided tumors into
two categories: Type 1 cystic lesions were single large cysts
with a thin, enhancing tumor wall. Type 2 cystic lesions had
multiple cysts with a thick, irregularly enhancing cyst wall.
Although cystic schwannomas have a tenacious and adherent
capsule, there was no significant difference in outcomes com-pared
to patients with solid JF schwannomas.
Chordomas and Chondrosarcoma
Several cases of chordomas and chondrosarcomas, rare
causes of tumors centered in the JF, have been reported.46-49
These tumors more commonly involve the JF through sec-ondary
extension. Chordomas arise from notochordal rem-nants
(ecchordosis physalliphora) found along the clival
bone marrow. They usually arise extradurally in the clivus
near the midline but may grow to involve the paramedian
skull base extensively. In addition to the conventional chor-doma
subtype, which makes up 70% to 90% of tumors, there
are dedifferentiated and chondroid subtypes.50 The dediffer-entiated
subtype has a significantly poorer prognosis while
the chondroid subtype contains cartilaginous and chon-dromatous
elements. Whether the latter is a true chordoma
or a chondrosarcoma variant is debated.
Chondrosarcomas are slow-growing malignancies derived
from cartilage that usually arise from skull base synchondro-ses,
typically from the petrooccipital synchondrosis.50 Unlike
chordomas, chondrosarcomas usually arise in a paramedian
location. Like chordomas, they may grow extradurally to in-volve
the JF. They also have three pathologic subtypes: con-ventional,
mesenchymal, and dedifferentiated. The mesen-chymal
and dediffererentiated subtypes contain various
amounts of small undifferentiated stromal cells and have a
worse prognosis than conventional subtypes.
Chordomas and chondrosarcomas usually manifest with
headaches and diplopia. In 50% of cases, the diplopia is
because of CN VI palsy.51 Trigeminal numbness is the next
most common cranial neuropathy. Dysfunction of CNs VII–
XII is less common, occurring in approximately 20%.50 Mul-tiple
ipsilateral cranial neuropathies are more common to
chondrosarcomas than chordomas.51 Radiographically,
chordomas and chondrosarcomas display highly variable
heterogeneous enhancement, lobulation, calcification, bony
erosion, and high signal intensity on T2-weighted MRI. Com-bined
with the previous characteristics, involvement of the
clivus or the petrooccipital synchondrosis is highly sugges-tive
of these tumors. Chondrosarcomas and chordomas
themselves may be difficult to distinguish radiographically
except that chordomas tend to occur in the midline and
chondrosarcomas in paramedian locations.
Other JF Lesions
Primary bone tumors may present as JF lesions. They are
uncommon and include a large number of pathologic entities
such as giant cell tumors, aneurysmal bone cysts, osteoblas-tomas,
plasmacytomas, ossifying fibromas, nonossifying fi-bromas,
fibrous dysplasia, osteoid osteomas, solitary bone
cysts, and osteitis fibrosa cystica of hyperparathyroidism.52
Few lesion on this list merit additional discussion. Giant cell
tumors of the JF may mimic glomus jugulare tumors, pre-senting
with pulsatile tinnitus, hearing loss, and a hypervas-cular
mass behind the tympanic membrane visible on oto-scopy.
Importantly, angiography reveals small, intricate
feeding vessels to a giant cell tumor in contrast to the large
vessels that usually supply glomus jugulare tumors and other
paragangliomas.52 Cranial plasmacytomas also may present
as vascular lesions of the JF. Though arteriovenous shunting
is not usually seen on angiography, it has been reported53 and
closely resembles the angiographic appearance of a glomus
jugulare tumor.
Metastatic lesions are an important cause of JF lesions and
are a more common cause of a classic Vernet or Collet-Sicard
syndrome than primary JF tumors.1 A review of nine patients
with JF metastases by Greenberg and co-workers54 under-scores
the aggressive nature of these lesions. Of nine patients,
eight had significant lower CN deficits and five had pain as a
prominent presenting feature, including glossopharyngeal
neuralgia in two patients. Head and neck tumors, accounted
for four of the nine cases. Head and neck cancers may reach
to the JF by direct extension or by hematogenous or perineu-ral
spread.55 The radiologic appearance of other metastatic
lesions may closely resemble that of glomus jugulare tumors:
metastatic melanomas,56 renal cell carcinomas,57 and adeno-carcinomas.
58

6. Clinicopathologic features of JF tumors 11
Conclusion
A wide variety of tumors can affect the JF, including glomus
jugulare tumors, meningiomas, schwannomas, chordomas,
chondrosarcomas, primary bone tumors, and metastases. In
general, radiographic and clinical features of each tumor type
allow preoperative identification with reasonable accuracy.
Accurate preoperative diagnosis is important because of the
complexity of surgical treatment and to the growing availabil-ity
of nonsurgical treatment modalities such as stereotactic
radiosurgery, which may be undertaken without tissue diag-nosis.
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