Otolaryngol Clin N Am

40 (2007) 625–649

Neurotologic Surgery for Glomus

Tumors
David M. Kaylie, MDa,*, Matthew O’Malley, MDa,
Joseph M. Aulino, MDb, C. Gary Jackson, MD, FACSa
a
The Otology Group of Vanderbilt, Department of Otolaryngology Head and Neck Surgery,
Vanderbilt University Medical Center, 300 20th Avenue North, Suite 502,
Nashville, TN 37203, USA
b
Department of Radiology and Radiological Sciences, Division of Neuroradiology, Vanderbilt
University Medical Center, 21st Avenue South, MCN, Nashville, TN 37232-2675, USA

Glomus jugulare tumors are benign tumors arising from paraganglia of

neural crest origin [1] near the jugular bulb. They grow along the planes of least
resistance within the temporal bone, which leads to great variability in the ex-
tent of involvement of vital structures. The safe removal of these tumors has
required great innovation and collaborative thinking among various surgical
specialties. The specialty of neurotologic skull base surgery has evolved in con-
junction with the development of surgical techniques for the management of
glomus jugulare tumors. The current surgical philosophy has developed out
of a realization that a team approach, in which neurotologists, neurosurgeons,
and head and neck/reconstructive surgeons design and implement a surgical
plan, is the optimal way to remove these tumors safely.
Glomus tumors are slow-growing tumors [2] that can become extensive
before they cause symptoms. Traditionally, surgical resection has been the
main treatment option for glomus tumors of the lateral skull base. Radia-
tion therapydexternal beam and stereotactic radiosurgerydhas been used
with success [3–5]. Complete resection is the ideal goal of surgical manage-
ment [2], and this generally can be achieved. Issues that led to incomplete
resection or unresectability in the past, such as cranial nerve deficits or in-
tracranial extension (ICE), are now manageable because of improved recon-
struction techniques.
This article reviews the important aspects of glomus tumor management.
The biology and histology of these tumors are unique and have an impact

* Corresponding author.
E-mail address: david.kaylie@vanderbilt.edu (D.M. Kaylie).

0030-6665/07/$ - see front matter Ó 2007 Published by Elsevier Inc.

doi:10.1016/j.otc.2007.03.009 oto.theclinics.com
626 KAYLIE et al

on treatment strategies. Nonsurgical options (eg, radiation therapy) are dis-

cussed. Surgical resection can be performed safely only after appropriate di-
agnostic testing is completed. Imaging and other diagnostic testing are
explored. The various resection and reconstructive strategies are discussed.

Tumor origin
The term ‘‘glomus’’ is a misnomer that was applied incorrectly before our
current understanding of the origin of these tumors. The term ‘‘paragan-
glioma’’ more descriptively represents such tumors as originating from spe-
cial neural crest elements, the paraganglion cells. The paraganglion cells,
along with autonomic ganglion cells, form the paraganglia [1]. The paragan-
glia are part of the neuroendocrine system that usually is associated with the
sympathetic ganglia and consist of the adrenal medulla and extra-adrenal
paraganglia [2]. Craniocervical paraganglia are distributed along the arteries
and cranial nerves of the ontogenetic gill arches. Derived from the branchio-
meric system, they may be jugulotympanic, intracarotid, coronary, orbital,
aorticopulmonary, pulmonary, coronary, or laryngeal. The vagal paragan-
glia are considered separate [2].
The paraganglia of the temporal bone are ovoid, lobulated bodies mea-
suring 0.1 to 1.5 mm. On average, three paraganglia can be found in each
ear, typically along the course of Jacobson’s or Arnold’s nerve. The ascend-
ing pharyngeal artery provides the primary blood supply to these paragan-
glia [1]. In contrast, the intravagal paraganglia are located within the
perineurium of the vagus nerve, generally within or approximating the jug-
ular ganglion or nodose ganglion [1].
Paraganglia contain two types of cells: type I (also referred to as chief
cells, granular cells, glomus cells, or epithelioid cells) and type II (also called
sustentacular cells, satellite cells, or supporting cells). Immunohistochemi-
cally, the chief cells are reactive for neuron-specific enolase, chromogranin
A, and synaptophysin, whereas type II cells are positive for S-100 and glial
fibrillary acidic protein [6]. Ultrastructurally, chief cells contain cytoplasmic
granules containing catecholamines.
Grossly, paragangliomas appear as deep red-purple or brown-gray en-
capsulated masses with a firm, rubbery consistency. Typically, their appear-
ance reflect their substantial vascular supply, and brisk bleeding is to be
expected should one be incised. Although carotid body paragangliomas of-
ten are clearly delineated from their surrounding structures, jugulotympanic
paragangliomas commonly exhibit some degree of local invasion, frequently
manifesting as erosion of the temporal bone [7].
Microscopically, paragangliomas resemble adrenal pheochromocytomas
(Fig. 1). The tumor contains all of the cellular elements normally present
in a paraganglion: chief cells, sustentacular cells, and a rich supporting vas-
cular network. Typically, chief cells predominatedarranged in an organoid-
nested pattern, known as zellballendand the architecture may be enhanced
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 627

Fig. 1. Histological image of a glomus tumor demonstrating zellballen architecture and

vascularity.

by reticulin stain. The cells are polygonal or spindle-shaped and exhibit cuff-
ing by sustentacular cells. A delicate capillary network also is characteristic,
sometimes producing confusion with vascular neoplasms [6]. Immunohisto-
chemical analysis can be helpful in evaluating suspected paragangliomas,
with a staining pattern similar to that of normal paraganglia. Although
electron microscopy is not usually necessary for diagnosis, ultrastructural
analysis of a paraganglioma reveals intracytoplasmic dense core (neuro-
endocrine) granules [7]. The usefulness of fine needle aspirates for the
diagnosis of paragangliomas is disputed, although some investigators dem-
onstrated successful fine needle aspiration analysis [8].
The assessment of malignancy based on pathologic findings is problem-
atic. Necrosis, nuclear pleomorphism, and vascular perineural invasion
may be seen in benign tumors. Only the presence of metastasis establishes
malignancy [9]. Although most head and neck paragangliomas are consid-
ered hormonally inactive [7], the detection of hormone-secreting tumors is
clinically significant. Morphologically, it is impossible to differentiate hor-
mone-secreting tumors from inactive tumors; thus, the distinction must be
made with specific laboratory evaluation.

Biochemistry
The chief cells of the paraganglia are one of 40 cell types classified in the
diffuse neuroepithelial system and can produce neuropeptides and catechol-
amines [2] Fewer than 4% of paragangliomas found in the head and neck
are hormonally active such that they are considered functional [10]. The
clinical presentation and biochemical behavior of secreting tumors is vari-
able, depending on the substance elaborated. Catecholamine elaboration,
dopamine secretion, carcinoid syndrome, and paraneoplastic anemia have
been reported [2].
The occurrence of familial and syndromic paragangliomas is well docu-
mented and is believed to account for 10% to 50% of head and neck
628 KAYLIE et al

paragangliomas [11]. Pheochromocytoma, thyroid and visceral neoplasms,

parathyroid adenoma, and multiple endocrine neoplasia syndromes are as-
sociated with paragangliomas. Additional tumors can be ipsilateral or con-
tralateral, located at any of the branchiomeric paraganglionic stations. The
most common association is glomus jugulare and carotid body tumors [2].
Genetic testing and counseling is available, although the usefulness and
role of these services have yet to be determined.
Paragangliomas rarely exhibit malignant degeneration, although several
cases have been reported over the past 60 years. The reported prevalence
of malignancy varies between 1% and 12%; 4% is the most commonly
quoted prevalence. The rate of malignancy is greater for vagal paragan-
glioma (19%) [12]. Certain familial or syndromic presentations may have
substantially higher rates of malignancy [13]. The determination of malig-
nancy cannot be made histologically; it is made by the detection of paragan-
glioma tissue at a site not normally associated with the paraganglionic
system. The more common metastatic sites include regional lymph nodes,
skeleton, lung, and liver [12]. A worse clinical course, greater cranial nerve
deficits, and higher morbidity and mortality are found in malignant tumors;
however, prolonged survival is possible with active disease [2].

Tumor classification
Tumor classification criteria have been reported [14–16], although none has
gained universal acceptance. The criteria proposed by Fisch and Oldring
[14,16] and Glasscock and colleagues [15] are among the more commonly ap-
plied. Both of these systems are relevant to surgical planning and are based on
an evaluation of tumor extension with specific designation for ICE. The cate-
gorization of neuroendocrine neoplasms, including paragangliomas based on
World Health Organization classification, also has been described [17].

Clinical diagnosis
Like all diagnoses, the history and physical examination are vital. They
raise the suspicion of a glomus tumor, but the diagnosis cannot be con-
firmed without imaging. Pulsatile tinnitus is the most common presenting
symptom of a glomus tumor [18]. This is caused by the turbulent flow
through the tumor and is seen in 76% of patients [19]. Hearing loss is the
second most common symptom, seen in 60% of patients [19], and usually
is a conductive hearing loss because of mass effect on the ossicular chain
[18]. Sensorineural hearing loss is caused by erosion of the tumor into the
labyrinth or cochlea [20]. Pure sensorineural hearing loss is unusual, occur-
ring in approximately 5% of patients, but mixed hearing loss is more com-
mon, occurring in 17% of patients [19].
Physical findings should alert the clinician to the possibility of a glomus tu-
mor. A vascular mass in the inferior aspect of the middle ear behind an intact
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 629

ear drum is the most common physical examination finding [18]. It is impor-
tant to differentiate between a glomus tympanicum tumor and a glomus jug-
ulare tumor. The inferior border of a glomus jugulare tumor is not visible
because the tumor extends into the hypotympanum and to the jugular bulb
[2]. A glomus tympanicum tumor may have 360 of its border visible [2]. Be-
cause glomus jugulare tumors arise from the jugular bulb, lower cranial nerve
symptoms may be encountered. Hoarseness is caused by vagus nerve involve-
ment with concomitant laryngeal dysfunction; it is seen in approximately 12%
of patients with glomus jugulare tumors [19]. Dysphagia is caused by dysfunc-
tion of one or several lower cranial nerves and occurs in less than 10% of pa-
tients [19]. Tongue weakness and shoulder weakness are the result of cranial
nerve XII and XI involvement. Headache or signs of increased intracranial
pressure can be seen in patients with large intracranial involvement of tumor.

Imaging
Paraganglioma, schwannoma, meningioma, and metastatic disease are
the primary diagnostic considerations when a jugular foramen mass is en-
countered. A schwannoma usually appears as a sharply demarcated, con-
trast-enhancing tumor centered in a smoothly enlarged jugular foramen,
with sharp, rounded bony margins. Highly vascular jugular paragangliomas
usually display intense contrast enhancement, flow voids within the tumor
are often visible, and bony infiltration or destruction typically is present
(Fig. 2). A ‘‘salt and pepper’’ appearance may be apparent on T1- and
T2-weighted MRIs [21], although this is an uncommon finding. These hy-
per- and hypointense foci are related to slow- and fast-flowing intravascular
blood and to intratumoral hemorrhage. Jugular paragangliomas produce ir-
regular erosion of the enlarged jugular foramen margins, the earliest sign be-
ing loss of the caroticojugular spine. Thin-section CT using bone algorithm
reconstruction usually allows differentiation of jugular foramen paragan-
glioma from schwannoma [22].

Fig. 2. Coronal CT image, bone algorithm, through the jugular foraminae. The symmetric sec-
tion shows the irregular, ‘‘moth-eaten’’ margins of the left jugular foramen (white arrowheads).
There is erosion of the left jugular tubercle: compare with the normal right (white arrow). The
tubercle separates the hypoglossal canal (H) from the jugular foramen. There is some tumor ex-
tension into the left hypotympanum (black arrowhead).
630 KAYLIE et al

Fig. 3. Coronal images through the right jugular foramen. CT image, bone algorithm, shows
obvious tumor within the hypotympanum (black arrow).

MRI is required to assess the extent of jugular paragangliomas. The cor-

onal postcontrast T1-weighted images are inspected carefully to assess for
extension of tumor into the hypotympanum (glomus jugulotympanicum tu-
mor) (Figs. 3 and 4) and to identify the inferior extent of the tumor below
the jugular foramen. The distal cervical internal carotid artery flow void is
identified on the axial T2-weighted images, and the extent and direction
of displacement is noted. Involvement of the hypoglossal canal may be ap-
parent clinically, but the full extent of involvement is assessed on the axial
postcontrast fat-suppressed T1-weighted images. Similarly, intracranial ex-
tent (intradural and extradural) (Fig. 5) is assessed on the axial postcontrast
images. Larger tumors involve the carotid canal, and MRI is essential to
identify petrous internal carotid artery displacement and encasement. CT
and MRI are useful to determine the extent of facial nerve and otic capsule
structure involvement.
Although routine MRI of the neck should cover the temporal bone, it is
essential that dedicated thin-section images through the jugular foramen

Fig. 4. Post-contrast fat-suppressed T1-weighted image better shows the jugular foramen
paraganglioma (P), but hypotympanum extension (arrow) is more subtle and must be searched
for diligently.
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 631

Fig. 5. Axial postcontrast T1-weighted image through the jugular foramina shows the enhanc-
ing left jugular paraganglioma (P), with transdural intracranial extension (ICE) into the left
cerebellomedullary angle.

and petrous pyramid are obtained to adequately assess the jugular paragan-
glioma. The MRI examination should include axial T1-weighted images as
well as axial T2-weighted images with or without fat-suppression. Coronal
T1-weighted images may be useful to determine the extent of bony skull
base extension with good contrast between the tumor and the T1-hyperin-
tense fatty marrow. After intravenous gadolinium contrast, axial and coro-
nal T1-weighted sequences are performed. Fat-suppressed postcontrast
images allow for improved distinction between the brightly enhancing para-
gangliomas and the bony skull base–suppressed fatty marrow. Thus, CT
and MRI of jugular foramen masses are considered complementary; CT is
useful to assess the bony margins of the jugular foramen, and MRI allows
for exquisite delineation of tumor extension. Van den Berg and colleagues
[23] are strong proponents of the time-of-flight, enhanced MR angiography
technique to depict the extent of the paraganglioma and to identify addi-
tional, small paragangliomas in other locations in the head and neck. In
contrast to conventional MR angiography, this technique does not

Fig. 6. Images show advantage of enhanced time-of-flight MR angiography to visualize addi-

tional paragangliomas in a patient who has undergone remote carotid body paraganglioma re-
section. Axial fat-suppressed T2-weighted image through upper neck at the level of C2 shows
a prominent, incidental right retropharyngeal lymph node (black asterisk). The right internal
carotid artery flow void is defined clearly (arrow). Posterior to the flow void, there is a T2-hy-
perintense nodule (curved arrow) whose appearance is concerning for a glomus vagale tumor.
632 KAYLIE et al

Fig. 7. Axial enhanced time-of-flight MR angiography image, again shows the right glomus va-
gale tumor (curved arrow), but also reveals a more apparent left glomus vagale tumor (double
arrows).

necessarily show the blood supply to the tumor, but it reveals a pronounced
distinction between the intensely enhancing tumor and the surrounding
dark, signal-suppressed tissues. This enhanced MR angiography technique
has proven invaluable to detect additional lesions (Figs. 6–9) and is consid-
ered an essential MR sequence when evaluating for other glomus tumors.
Conventional angiography may be useful to assess vascularity, and pre-
operative embolization can be performed at the same time. Uncommonly,
catheter angiography is necessary to confirm the preoperative diagnosis of
paraganglioma. Blood supply is primarily from the ascending pharyngeal
branch of the external carotid artery, with contribution from muscular
branches of other external carotid artery vessels and the vertebral artery.

Management
Management of glomus tumors of the lateral temporal bone requires at-
tention to the individual patient’s needs (Fig. 10). The age and health of the
patient are important considerations. Elderly and infirmed patients do not
compensate well after the abrupt loss of lower cranial nerve function. Often,
it is prudent to consider this before undertaking a large skull base resection.
The extent of the tumor, involvement in vital structures, and desires of the
patient also are major considerations. Therapy options generally fall into
two categories: curative and control. Because these are benign tumors,

Fig. 8. Oblique maximum intensity projection image shows the bilateral glomus vagale tumors
(arrowheads) in close relation to the internal carotid arteries.
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 633

Fig. 9. Reformatted axial image through the level of the thyroid gland shows an apparent ad-
ditional 3-mm carotid sheath paraganglioma in the region of the left tracheoesophageal groove
laterally (arrow).

curative therapies entail complete surgical resection. Control therapies are

designed to stop tumor growth and halt the progression of symptoms. Ra-
diation therapy does not remove the tumor; the goal is to render the tumor
incapable of further growth [5]. This requires the patient to fully understand
that the tumor is still present and likely will remain at its present size.

Radiation therapy
The radio-responsiveness of glomus tumors has been investigated. Post-
radiation tissue samples have shown no overall decrease in tumor vascular-
ity, but they did show increases in perivascular fibrosis, endothelial
hyperplasia, and subendothelial hyaline degeneration [3]. Spector and col-
leagues [24] concluded that the main effect that radiation has on glomus tu-
mors is proliferation of fibrous stroma and not tumor destruction. This
raises the question of whether radiation is curative and appropriate as a sin-
gle-modality treatment [3].
External beam radiation has been the traditional radiation therapy for
glomus tumors [3,5]. It has been difficult to make conclusions about out-
comes because of lack of uniform treatment regimens [3]. Radiation doses
were kept low by early radiation oncologists [3]. Kim and colleagues [25]
found that regrowth rates were 22% with external beam radiation lower
than 40 Gy/4 weeks and 2% for dosages greater than 40 Gy/4 weeks.
They recommended a dosage between 40 and 45 Gy. Cummings and col-
leagues [26] recommended a dosage of 35 Gy for 3 weeks.
de Jong and colleagues [27] reported the results on 38 patients receiving
external beam radiation for glomus tumors. Radiation was the primary ther-
apy for 14 patients, it was used in conjunction with surgery in 13 patients,
and it was used as salvage for recurrence after surgery in 11 patients.
When used as the sole modality, tumor growth was seen in 21% of patients.
Complete control was achieved in all patients when radiation and surgery
was used as an initial therapy, and 91% of salvage patients. These results
indicate that radiation is effective in conjunction with surgery or as salvage
for incomplete resections. Carrasco and Rosenman [3] reviewed studies of
surgical and external beam radiation therapy and concluded that tumor
634 KAYLIE et al

Fig. 10. Management algorithm.

recurrence (for surgical treatments) and regrowth (for radiation modalities)

were equivalent (w10%). Complications from radiation have included os-
teoradionecrosis of the temporal bone or necrosis of the brain [4]. There
also is a concern for radiation-induced malignancies [4].
Radiosurgery has emerged as an alternative to external beam radiation
for glomus tumors [4,5]. Its advantage over external beam modalities is
that the dose is delivered in a single session. Also, because a smaller volume
of normal tissue is irradiated, chronic radiation complications may be re-
duced [4]. Foote and colleagues [4] reported the results of using Gamma
Knife radiosurgery on 25 patients with a mean follow-up of 35 months.
The tumor remained stable in size or shrunk in all patients. There were
no new cranial nerve deficits other than new-onset vertigo; however, they
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 635

did not provide data on hearing. Pollack [5] performed Gamma Knife radio-
surgery on 39 patients with a mean follow-up of 44 months. He used a mean
maximum tumor dose of 31.2 Gy with a mean dose to the margin of 14.9
Gy. The tumors remained the same or shrunk in 98% of patients. Progres-
sive hearing loss was seen in 19% of patients. Other cranial nerve symptoms
were worsening balance and facial numbness. Pollack had one patient with
long-term follow-up who showed tumor growth after a period of quiescence.
He concluded that it was possible for these tumors to become active after
a period of time and regrow. These two studies show that short-term tumor
control is achievable. Glomus tumors are slow-growing tumors; showing
that the tumor did not grow after radiation for 2 to 3 years may reflect its
natural history. It is difficult to conclude from these studies that Gamma
Knife radiosurgery altered the growth patterns of these tumors.

Surgical planning
Glomus tumor growth can be multidirectional. They tend to spread along
paths of least resistance (Fig. 11). The temporal bone is largely pneumatized
and provides a wide array of pathways for these tumors to spread from their
point of origin. Vascular channels and neural foramina provide excellent
routes for glomus tumors to spread out of the confines of the temporal
bone [2]. Therefore, surgical planning is complex and critically important.
The variable extent of these tumors requires the expertise from neurotolo-
gists, neurosurgeons, and head and neck reconstructive surgeons to provide
the patient with an operation that meets their individual needs. Surgical
planning should proceed with three main goals in mind [2]. The extent of
dissection should allow for access to all tumor margins, access to ICE, prox-
imal and distal control of major vessels, and access to cranial nerves
(Fig. 12).

Fig. 11. Extent of glomus jugulare tumor growth is variable along paths of least resistance.
636 KAYLIE et al

Fig. 12. Surgical approaches are determined for each patient depending upon the extent of the
tumor. Various approaches are used to maximize surgical exposure.

The course of the facial nerve in the lateral temporal bone puts it in the
trajectory of the surgical dissection. It lies directly lateral to the jugular bulb
as it travels in the mastoid to the stylomastoid foramen (Fig. 13). Therefore,
tumors arising from the jugular bulb will be in close proximity to the facial
nerve. Mobilization of the facial nerve allows for unhindered access to the
jugular bulb. The intrinsic blood supply to the facial nerve allows it to retain
function although its extrinsic blood supply is disrupted. Mobilization of the
facial nerve from the external genu is defined as a short mobilization, and
this is consistent with excellent facial function [2]. Long mobilizations are
from the internal genu and usually result in transient facial weakness.
Long-term facial function usually is House-Brackmann grades II to IV [2].
All glomus tumors will have a relationship with the carotid artery [28].
The degree of carotid involvement varies but it usually can be determined
with preoperative imaging. Successful lateral skull base surgery is predicated
on recognizing the importance of managing the carotid artery. Proximal and
distal control of the carotid artery is of paramount importance in lateral
skull base surgery. Proximal control is achieved early in the surgery by ex-
posing the carotid artery in the neck. The jugular vein also is easily con-
trolled in the neck at the outset of the surgery. Distal control of the
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 637

Fig. 13. Facial nerve is seen directly lateral to the tumor.

carotid artery is achieved by exposing it distal to the tumor. Therefore, the

exact portion of the carotid artery that is exposed distally to the tumor is
entirely dependent upon the growth pattern of the tumor. Neurotologic sur-
gical exposures require methods to access the carotid artery in tympanic, pe-
trous, or cavernous sinus segments of the artery.

Surgical techniques for glomus jugulare tumors

The surgical incisions are designed to provide access to the lateral skull
base and vital structures in the neck, but also are designed to facilitate cere-
brospinal fluid (CSF) management and wound closure (Fig. 14). Proximal
control of the carotid artery can be achieved by the extension of the incision
into the neck. The cranial nerves and jugular vein also are readily accessed
(Fig. 15). Lower cranial nerve preservation is facilitated by identification of
the nerves where they are uninvolved by tumor in the neck [18]. They can be
dissected up to the pars nervosa or intracranially if necessary. This tech-
nique facilitates lower cranial nerve preservation.
Surgical techniques have been described in great detail elsewhere [28], and
they are described briefly here. Once the temporal bone has been exposed
and vessels and nerves have been controlled in the neck, the surgery pro-
ceeds with a complete mastoidectomy (Fig. 16). The facial nerve is identified
in the mastoid. The facial recess is opened and extended by transecting the
chorda tympani (Fig. 17). This allows for access to the hypotympanum and
the tympanic bone. The annulus of the tympanic membrane can be skeleton-
ized and followed anteriorly to the Eustachian tube. This allows access to
the tympanic segment of the carotid artery. The canal wall is preserved.
638 KAYLIE et al

Fig. 14. Diagram showing initial incisions. The solid line allows for greatest exposure of the
temperoparietal fascia. The dashed line incision provides less access to the temperoparietal fas-
cia, but a vertical limb can be added to provide increased exposure if more tissue is needed for
reconstruction.

This technique is useful for smaller tumors confined to the jugular foramen
and involving the carotid artery no higher than the tympanic segment [28].
For larger tumors that extend beyond the temporal bone, more distal con-
trol of the carotid artery is required. The mastoid tip is removed to allow for
increased access to the tympanic bone (Fig. 18). It is necessary to take the canal
wall and remove the eardrum and ossicles. This sacrifices conductive hearing,
but allows for extension of the dissection to the infratemporal fossa, petrous
carotid artery and beyond, cavernous sinus, and Eustachian tube. To prevent
spinal fluid otorrhea, overclosure of the ear canal is required when the dissec-
tion is taken to this extent. The bony dissection continues into the infratempo-
ral fossa as far as necessary to get distal control of the carotid artery. The
petrous portion can be exposed readily, and the surgery can be extended to ex-
pose the middle fossa and cavernous sinus if tumor extent dictates it.
An infratemporal fossa approach is used when tumors extend into the in-
fratemporal fossa or when control of the distal petrous carotid artery is nec-
essary (Fig. 19). This approach is facilitated by anterior dislocation of the
mandible by sectioning its ligaments. The infratemporal fossa approach pro-
vides access to the cavernous sinus, clivus, nasopharynx, and deep portions
of the temporal bone. The Eustachian tube is resected in this approach. The
middle cranial fossa can be exposed readily with this approach as necessary.
The foramen spinosum and foramen rotundum also can be exposed by the
infratemporal fossa approach (Fig. 20).
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 639

Fig. 15. The vessels and cranial nerves are exposed easily in the neck. The surgical incisions are
designed to allow for access to these structures to provide adequate control.

Once the carotid artery is controlled adequately, facial nerve mobilization

is performed (Fig. 21). The jugular vein is ligated in the neck, and the prox-
imal carotid artery is secured with vascular loops. This allows for rapid con-
trol of the artery should there be injury during tumor dissection. The
sigmoid sinus is packed off to provide proximal control of the venous

Fig. 16. Simple mastoidectomy performed.

640 KAYLIE et al

Fig. 17. Extended facial recess is performed by transecting the chorda tympani.

outflow. A small window of bone is removed over the sigmoid sinus to allow
for extraluminal compression. An incision is made in the sinus and packed
with surgicel. The tumor is dissected off from the carotid artery. The sig-
moid sinus is opened down to the level of the jugular bulb. Bleeding from
the inferior petrosal sinuses is controlled with packing. Then the tumor’s

Fig. 18. Complete mastoidectomy with an extended facial recess. The mastoid tip has been re-
moved and the vital anatomy of the neck has been exposed and controlled.
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 641

Fig. 19. The infratemporal fossa approach can be used to safely expose the carotid artery in the
petrous portion of the temporal bone. This allows for adequate distal control of the artery for
tumors that extend anteriorly.

involvement with cranial nerves IX, X, XI, and XII can be assessed. The
variable tumor growth may allow for sparing of these nerves; however, if
the tumor is intimately involved with these nerves, it is prudent to sacrifice
the nerves to allow for complete (Fig. 22) tumor resection. The tumor can
then be removed completely.
ICE of tumor occurs when the tumor transgresses the dura by direct ex-
tension through the dura or by entering through cranial nerve routes
[2,29,30]. The extradural and intradural components of the tumor can be re-
moved in a single stage. By definition, dural transgression creates a defect
that requires reconstruction to prevent spinal fluid leak. Tumors of this ex-
tent require the neurotologist to overcome several challenges. There will be
more extensive bone and soft tissue defects to be reconstructed, and spinal
fluid leak is a possibility that is made more likely by increased pressures sec-
ondary to venous occlusion. These challenges are best met with careful plan-
ning and coordination with neurosurgical and head and neck/reconstruction
colleagues.

Reconstruction and cranial nerve rehabilitation

The bone and soft tissue defects are the necessary result of the surgical
resection. Large tumors can be removed safely because reconstruction tech-
niques have been developed to allow it. It is not prudent for a neurotologist
to attempt resection of a glomus jugulare tumor unless there is a plan for
642 KAYLIE et al

Fig. 20. The carotid artery has been exposed up to the cavernous sinus. Middle fossa exposure
has facilitated this approach. There is a medium-sized dural defect.

reconstruction. The principles of defect reconstruction are designed primar-

ily to provide adequate closure of spinal fluid–containing spaces and second-
arily to provide an acceptable cosmetic result. The wound closure also plays
an important role in preventing spinal fluid leaks. The principles of cranial
nerve rehabilitation primarily are to allow the patient to take nutrition by
mouth and prevent aspiration and secondarily to provide a good voice. Fa-
cial nerve weakness often occurs after these surgeries, mostly because of
nerve mobilization. Eye injury because of poor lacrimation and inability
to close the eye are the most worrisome outcomes from facial weakness.
This can be prevented with diligent eye care and placement of a gold weight
to facilitate eye closure.
The method of defect reconstruction is dependent upon the size of the de-
fect. The extent of the defect usually can be determined with careful study of
the preoperative imaging. This allows for a general plan for reconstruction.
ICE usually can be anticipated. When ICE is expected, a lumbar drain is
placed preoperatively. It is left in place for 5 to 7 days to allow for spinal
fluid decompression to reduce pressure on the reconstruction. The Eusta-
chian tube is sewn closed when the infratemporal fossa approach is used,
and the middle ear is packed with muscle when the canal wall and middle
ear are intact. These maneuvers help to reduce CSF rhinorrhea.
The reconstruction is dependent upon the size of the defect. Using vascular-
ized tissue is the key to successful reconstruction and CSF management. Small
defects may have the labyrinth intact and a minimal dura defect at the pars
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 643

Fig. 21. Facial nerve is mobilized from the geniculate ganglion.

nervosa. Commonly, these are reconstructed with free abdominal fat. This re-
construction may be augmented with fibrin glue or a pedicled tissue graft (eg,
temporoparietal fascia flap). The temporoparietal fascia is a vascularized fas-
cia that is supplied by the superficial temporal artery [31,32]. The artery
branches into anterior and posterior arteries, which allows for a large flap
to be mobilized (Fig. 23). Medium-sized defects occur with larger bone dissec-
tion and involve large dura defects. These are closed with various flaps, includ-
ing the temporoparietal fascia flap (Fig. 24), trapezius flap, or latissimus dorsi
flap. The reconstruction is augmented with free abdominal fat to provide bulk
to bolster the flap and prevent CSF leak. It also helps to fill in the defect and
improve cosmesis. Large defects can occur with large glomus tumors, previ-
ously radiated fields, or other large skull base tumors. The flaps mentioned
above are used often. These defects also might require a microvascular free
flap for reconstruction. It is imperative to bring vascularized tissue into the
defect, which may not be possible with large resections or previously radia-
ted fields. The wound closure is facilitated by the initial incisions. The sterno-
cleidomastoid muscle is reapproximated to the temporalis muscle fascia. This
helps to hold the abdominal fat graft in place and helps to bolster the dura clo-
sure (Fig. 25).
The lower cranial nerves, IX, X, XI, and sometimes XII, are sacrificed
commonly during glomus tumor surgery. The most worrisome complication
is aspiration secondary to laryngeal incompetence. Vocal cord medialization
with or without arytenoid adduction has been a major advancement. It pro-
vides patients with the ability to take nutrition by mouth and virtually
644 KAYLIE et al

Fig. 22. The tumor has been devascularized and is resected, leaving a medium-sized defect.

eliminates the need for tracheostomy [33]. Velopharyngeal incompetence

can be addressed with palatal adhesion surgeries [32]. Elderly patients
have much greater difficulty compensating for the loss of lower cranial
nerves. It is important to discuss this preoperatively, because they may
not be surgical candidates. Partial resection with sparing of the cranial
nerves also may be considered.

Results
Between January 1, 1971 and October 31, 2006, 238 patients underwent
surgery for glomus tumors at our institution. There were 202 patients
with glomus jugulare tumors, 33 with glomus vagale tumors, and 3 with ca-
rotid body tumors with skull base involvement. The median age was 41
years (range: 9 to 78 years). The male/female ratio was 2.5:1. Tumor size
ranged from less than 1 cm to 7 cm. Multiple tumors were seen in 9.7%
of patients.
Total resection was achieved in 90% of patients. Subtotal resection was
performed mostly in elderly patients with carotid artery involvement or cra-
nial nerve involvement. Those with partial resection were elderly patients
with extensive carotid artery involvement or cranial nerve involvement. Tu-
mor recurrence rate was 6%. Follow-up ranged from 9 to 235 months with
a mean of 54 months. Preoperative cranial nerve deficits were seen in 47% of
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 645

Fig. 23. The superficial temporalis fascia flap has great flexibility and size potential. It is a ped-
icled flap supplied by the superficial temporal artery.

patients. The most commonly involved nerves were X, XII, and IX. Of those
with cranial nerve deficits, 95% had multiple cranial nerve deficits. Preoper-
ative facial nerve weakness indicated that the facial nerve was involved with
tumor in 100% of patients. Sensorineural hearing loss was caused by inner
ear involvement. New cranial nerve deficits were seen in 60% of patients,
with cranial nerves IX, X, XI, and XII deficits in 40%, 24%, 26%, and
21%, respectively. Facial nerve weakness not attributed to mobilization of
the nerve occurred in 5% of patients.
Thirty patients underwent revision surgery from January 1, 1987 until
October 31, 2006: 24 for recurrent glomus jugulare tumors, 5 for glomus va-
gale, and 1 for a carotid body tumor. Twenty-one patients had one prior
surgery, 5 patients had two prior surgeries, and 2 patients had three or
more surgeries. Total resection was possible in 93% of patients. Of those
with complete resection, there was no evidence of recurrence in 97% of pa-
tients. The tumors ranged in size from 1.7 to 4.2 cm. Of the patients requir-
ing revision surgery, 66% had preoperative cranial nerve deficits. The most
common deficits were seen in cranial nerve IX (37%) and X (48%). Postop-
eratively, 95% of patients had cranial nerve deficits. Cranial nerves IX
(77%) and X (74%) were the most commonly affected. Multiple cranial
nerve deficits were seen in 75% of patients.
Twenty-six patients had glomus tumors with ICE between January 1,
1987 and October 31, 2006. The tumor size ranged from 1.5 to 7 cm with
646 KAYLIE et al

Fig. 24. The pedicle is isolated and the flap is rotated into the defect to provide vascularized
tissue. The length of the flap allows for excellent coverage of the dural defect.

a mean size of 4.3 cm. The size of the intracranial component ranged from
0.3 to 4 cm with a mean size of 1.6 cm. Total resection was achieved in 89%
of patients. Reconstruction varied depending upon the size of the dural de-
fect. All patients had a temporoparietal fascia flap. Sixteen patients had a du-
ral patch placed with bovine pericardium in addition to the temporoparietal
fascia flap. All patients had a lumbar drain placed before the procedure that
was left in place for 5 to 7 days. CSF leak occurred in 6% of patients, and
4% had pseudomeningocele formation.
The mortality was 2% (5 out of 232 patients). Three patients died of
stroke after carotid artery resection, and 2 patients died of pulmonary
embolus.

Summary
Glomus tumors of the lateral skull base present a surgical challenge be-
cause of the variable extent of their growth. The specialty of neurotology
has evolved, in great part, out of a need to develop surgical strategies to re-
move these tumors safely. The current concept for surgical removal is cen-
tered on the team approach. The expertise of head and neck/reconstruction
 NEUROTOLOGIC SURGERY FOR GLOMUS TUMORS 647

Fig. 25. The sternocleidomastoid muscle flap is sewn to the deep temporal fascia and temporalis
muscle. This holds the free fat graft in tightly to apply pressure on the dural closure to prevent
spinal fluid leak.

surgeons has allowed for greater surgical dissection. The defects that are
necessarily created to provide access to all tumor margins can be closed
and reconstructed with minimal morbidity in carefully selected patients.
All glomus jugulare tumors involve the carotid artery to some degree. The
extent of involvement dictates the surgical planning and extent of dissection.
Proximal and distal control of the carotid artery is necessary to help prevent
catastrophe.
Surgical removal is the only way to achieve total tumor removal. Radia-
tion therapy, including stereotactic radiosurgery, does not remove the tu-
mor. The goal is to render the tumor incapable of further growth
secondary to perivascular changes in the tumor. Long-term data on large
numbers of patients are not available at this time. Surgery remains the
most appropriate modality for achieving complete cure.
Surgery can be performed safely in a single stage in properly selected pa-
tients. Postoperative cranial nerve deficits are common, and preoperative
counseling is imperative. Elderly and infirmed patients do not compensate
well for loss of cranial nerve function and generally do not make good sur-
gical candidates. Tumors with ICE can be removed in a single setting. Al-
most all patients who undergo revision surgery for glomus tumors have
one or more cranial nerves sacrificed. Revision surgery is made more diffi-
cult by scarring and altered surgical planes. Glomus tumors are benign
648 KAYLIE et al

and slow growing, so complete surgical excision provides a cure. Patients

who had prior surgery, in which tumor was left to spare cranial nerves,
have the potential for tumor regrowth. If revision surgery is necessary, it
is almost assured that cranial nerve function will be lost. We conclude
that the primary surgery is the best setting for providing a cure, and loss
of cranial nerve function in an appropriate patient is tolerated and often
necessary.
Neurotologic surgery for glomus tumors of the lateral skull base is a safe
and effective method for achieving a complete cure. The quality of life
afforded to these patients is good, especially considering the complexity of
the surgery and reconstruction.

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