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Acta Oto-Laryngologica
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Skull base surgery for removal of temporal bone tumors


Chong-Sun Kim a; Myung-Whan Suh a
a
Department of Otorhinolaryngology, Seoul National University College of Medicine, Yongon-Dong, Seoul,
Chongno-Gu, South Korea

Online Publication Date: 01 October 2007

To cite this Article Kim, Chong-Sun and Suh, Myung-Whan(2007)'Skull base surgery for removal of temporal bone tumors',Acta Oto-
Laryngologica,127:10,4 — 14
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Acta Oto-Laryngologica, 2007; Suppl 558: 414

Skull base surgery for removal of temporal bone tumors

CHONG-SUN KIM & MYUNG-WHAN SUH

Department of Otorhinolaryngology, Seoul National University College of Medicine, Yongon-Dong, Chongno-Gu, Seoul
110-799, South Korea

Abstract
Conclusion. When selecting the appropriate surgical approach the pathological type of tumor, the physiological status as well
as the functional aspects should be considered. Understanding the strengths and weaknesses of each surgical technique and
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knowledge of the particular tumor biology facilitates selection of the most appropriate surgical approach and a successful
outcome. Objectives. The purpose of this study was to review cases that underwent skull base surgery for a variety of tumors
that involved the temporal bone. We reviewed a single center’s 25-year experience for epidemiologic characteristics,
symptoms, treatment type and outcomes. Patients and Methods. The medical records and radiological images of 91 patients,
who underwent skull base surgery, were retrospectively reviewed. Results. Among the 91 patients, 61 cases had benign
disease and 30 had malignancies. A facial nerve schwannoma was the most common benign intratemporal tumor and a
squamous cell carcinoma was the most common malignant tumor. With the facial nerve schwannoma, facial nerve paralysis
and hearing loss were the most common presenting complaints; otalgia was the most common presenting symptom for
temporal bone cancer. For patients with a glomus tumor, there was a characteristic pulsating tinnitus. A majority of the
facial nerve schwannomas were resectable through the transmastoid approach. The infratemporal fossa approach type A was
usually required for lower cranial nerve schwannomas and glomus jugulare tumors. However, the fallopian bridge technique
with hypotympanectomy was another surgical option. Partial temporal bone resection and subtotal temporal bone resections
were performed in cases with temporal bone cancer. The disease free 5-year survival of the temporal bone cancers was 42%
and for the squamous cell carcinomas, it was 44%.

Keywords: Skull base, temporal bone, schwannoma, glomus tumor, squamous cell carcinoma

Introduction well as benign and malignant tumors develop in


this area. However, the numerous vital structures
The modern era of skull base surgery started by
of the inner ear and the proximity of the brain
William House who first introduced the translabyr-
inthin approach in 1961 [1]. This new approach are significant obstacles to skull base surgery. The
enabled a wider view of the cerebellopontine angle surgeon should be able to utilize all available
and a more precise removal of a large vestibular surgical options in order to approach a given lesion
schwannoma. After this surgical quantum leap by without damaging these vital structures. However,
House, Ugo Fisch opened another new field of extensive training is required to become familiar
skull base surgery with the infratemporal fossa with the various techniques currently available.
approach in 1978 [2,3]. The previously inoperable Subtotal petrosectomy, an operation that involves
tumors near the jugular foramen could be success- removal of all of the pneumatic air cells of the
fully resected by this approach. These pioneers and temporal bone, is the final step in middle ear
many others are responsible for the success of the surgery and at the same time, it is the first step in
advanced skull base surgery currently used. Yet the skull base surgery. Therefore, middle ear surgery
temporal bone presents a difficult surgical field; it and skull base surgery overlap with temporal bone
forms the auditory organ and the lateral part of the surgery. The abundant accumulated experience
skull base. Congenital and inflammatory lesions as from middle ear surgery may provide a strong

Correspondence: Chong Sun Kim, Department of Otorhinolaryngology, Seoul National University College of Medicine, Yongon-dong, Jongno-gu, Seoul
110-744, Korea. Tel: 82-2-2072-2440. Fax: 82-2-745-2387. E-mail: chongkim@snu.ac.kr

ISSN 0365-5237 print/ISSN 1651-2551 online # 2007 Taylor & Francis


DOI: 10.1080/03655230701624806
Skull base surgery 5
Table I. Pathology of the temporal bone tumors.
base of knowledge that can also be used for skull
base surgery.
Pathology No. %
The limited surgical literature in the field of skull
base surgery has provided little information for Benign tumors (N61)
otologists. There are a number of articles on skull Facial nerve schwannoma 25 41.0
base surgery for specific diseases but not many Lower cranial nerve schwannoma 9 14.8
Glomus tumor 10 16.4
articles on a large series of skull base surgery. The
Congenital epidermoid 5 8.2
purpose of this study was to review cases that Neurofibroma 2 3.3
underwent skull base surgery for a variety of tumors Petrous apex cyst 2 3.3
of the temporal bone. We reviewed a single center’s Fibrous dysplasia 2 3.3
25-year experience to determine epidemiologic Fibrous histiocytoma 1 1.6
Cementifying fibroma 1 1.6
characteristics, symptoms, treatment types, and
Ceruminous adenoma 1 1.6
outcomes. Chordoma 1 1.6
Chondrocalcinosis 1 1.6
Pigmented villonodular synovitis 1 1.6
Materials and Methods
Malignant tumors (N30)
From January 1982 through December 2006, 176 Squamous cell carcinoma 17 56.7
patients underwent skull base surgery at the depart- Adenocystic carcinoma 6 20.0
ment of Otorhinolaryngology, Seoul National Uni- Basal cell carcinoma 3 10.0
Chondrosarcoma 2 6.7
versity Hospital, for intratemporal or intracranial
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Sebaceous carcinoma 1 3.3


tumors. The senior author (C.S. Kim) performed Apocrine carcinoma 1 3.3
the majority of operations. Medical records were
Lower cranial nerve: cranial nerve IX, X, XI, XII.
retrospectively reviewed for age, gender, presenting
symptoms, operative procedures, location of the
tumor, and surgical outcomes. Radiological images to 61 years). Interestingly eight out of the nine
were also reviewed. In almost all cases temporal patients were female. Of the 10 patients with an
bone CT and/or MRI were performed. In cases with intratemporal glomus tumor, six were male and four
a glomus tumor, angiography was also carried out. were female. The mean age of these patients was
Among the 176 patients, a vestibular schwannoma 42.0 years (range 20 to 60 years). Thirty patients
was the most common tumor; it was identified in 80 were diagnosed with temporal bone cancer. The
patients. However, in this article we focused on the mean age of the patients was 53.4 years (range 29 to
91 patients with intratemporal tumors excluding 79 years) and the sex ratio was 20:10.
intra cranial vestibular schwannoma, meningioma,
endolymphatic sac adenocarcinoma, and clivus
chondroblastoma. Pathological reports confirmed Location and origin of the Tumor
that there were 61 cases with benign disease and The location of the facial nerve schwannoma was
30 with a malignancy. Facial nerve schwannoma was analyzed according to the five segments and the two
the most common benign intratemporal tumor and genu of the facial nerve (Figure 1). In four (14%)
squamous cell carcinoma was the most common cases the tumor was located in the meatal portion,
malignant tumor of the temporal bone (Table I). 10 (36%) in the labyrinthine portion, 15 (54%) in
The facial nerve function was graded according to
the House-Brackmann (HB) scale. The glomus
tumors were classified according to the Glasscock-
Jackson classification [4] and cancers of the temporal
bone were staged by the Arriaga staging system [5].

Results
Patient Demographics
The mean age of the 25 patients with facial nerve
schwannomas was 35.8 years (range 1 to 59 years). Figure 1. Location of the facial nerve schwannoma. The location
The sex ratio was 7:18 (male:female) with a slight of the 25 facial nerve schwannomas was analyzed according to the
five segments and the two genus of the facial nerve. The tympanic
preponderance of female patients. Nine patients had portion and the second genu was the most common location of a
lower cranial nerve (cranial nerve IX, X, XI, XII) facial nerve schwannoma. A large tumor can involve more than
schwannomas; their average age was 41.0 (range 26 one portion of the facial nerve.
6 C.-S. Kim & M.-W. Suh

the geniculate ganglion, 16 (57%) in the tympanic Clinical Manifestations


portion, 21 (75%) in the second genu, 19 (68%)
The signs and symptoms identified at the time of
in the mastoid portion and seven (25%) in the
presentation are summarized in Table II. For the
stylomastoid foramen. The number of cases is
facial nerve schwannomas, facial nerve paralysis and
not mutually exclusive because a large tumor may
hearing loss were the most common presenting
involve more than one portion of the facial nerve.
complaints occurring in 24 (96%) and 12 (48%)
The tympanic portion and the second genu were the
patients respectively. The clinical manifestations of a
most common location of facial nerve schwannomas.
lower cranial nerve schwannoma were classified into
The lower cranial nerves, which the schwannomas
originated form, was assessed by the surgical find- otologic symptoms and non-otologic symptoms.
ings. Cranial nerve IX was the origin of the tumor in Hearing loss was the most common otologic symp-
three (33%) cases, Cranial nerve X in one (11%) tom; it was detected in five (55%) patients. On the
case, cranial nerve XII in three (33%) cases and the other hand, a hoareness (44%), shoulder weakness
origin was not identified in two cases (22%). (33%) and tongue deviation (33%) were the three
Among the 10 glomus tumor patients, seven major non-otologic symptoms. The most common
(70%) had a glomus jugulare and three (30%) had symptoms associated with an intratemporal glomus
a glomus tympanicum. The Glasscock-Jackson glo- tumor were hearing loss and tinnitus, observed in
mus jugulare classification was type I in two patients, eight (80%) and seven (70%) patients respectively.
type II in one patient, type III in one patient Tinnitus was a characteristically pulsating type in
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and type IV in three patients. As for glomus five (50%) patients. Facial nerve paralysis, intermit-
tympanicum, one patient had type I and two patients tent otorrhea, dizziness, and otalgia were also pre-
had type II. Angiography was performed in nine of sent in a number of patients (Table II). The tumor
the 10 patients. The ascending pharyngeal artery was visible through the tympanic membrane in eight
was the main feeder artery in eight (80%) cases and (80%) patients and two (20%) among them had a
the middle meningeal artery in one (10%) case. The tympanic membrane perforation. The mass was
occipital artery and posterior auricular artery also noted to be pulsating when examined by an endo-
served as subsidiary feeding arteries. The origin of scope in four (40%) patients. In one patient with
the cancer was suspected to be the external auditory hypertension, the tumor turned out to be a function-
canal in 27 (90%) patients. The mastoid cavity was ing glomus tumor that secreted vanillylmandelic acid
suspected to be the origin in one (3%) patient who (VMA). In cases with temporal bone cancer, otalgia
had a chondrosarcoma. The origin was not clear in was the chief complaint in 19 (63%) patients and
the remaining two (7%) patients. otorrhea in 17 (57%) patients. A mass in the external

Table II. Symptoms associated with temporal bone tumors.

Facial nerve Lower cranial nerve Glomus tumor Temporal bone


schwannoma (N25) schwannoma (N9) (N10) cancer (N30)
No. (%) No. (%) No. (%) No. (%)

Otologic symptoms
Hearing loss 12 (48.0) 5 (55.6) 8 (80.0) 14 (46.7)
Facial nerve paralysis 24 (96.0) 0 (0.0) 4 (40.0) 3 (10.0)
Tinnitus 6 (24.0) 3 (33.3) 7 (70.0) 5 (16.7)
Dizziness 8 (32.0) 2 (22.2) 3 (30.0) 17 (56.7)
Otorrhea 2 ( 8.0) 0 (0.0) 4 (40.0) 17 (56.7)
Otalgia 0 (0.0) 1 (11.1) 3 (30.0) 19 (63.3)
Nonotologic symptoms
Mass formation 9 (36.0) 0 (0.0) 2 (20.0) 16 (53.3)
Hoarse voice 0 (0.0) 4 (44.4) 0 (0.0) 0 (0.0)
Shoulder weakness 0 (0.0) 3 (33.3) 0 (0.0) 0 (0.0)
Tongue deviation 0 (0.0) 3 (33.3) 0 (0.0) 0 (0.0)
Ataxia 0 (0.0) 2 (22.2) 0 (0.0) 0 (0.0)
Swallowing difficulty 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)
Aspiration 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)
Dyspnea 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)
Headache 0 (0.0) 1 (11.1) 0 (0.0) 0 (0.0)

The numbers are not mutually exclusive.


Lower cranial nerve: cranial nerve IX, X, XI, XII.
Skull base surgery 7

auditory canal, hearing loss, tinnitus, facial nerve (14%) case because multiple arteries in this patient
paralysis, and vertigo were also present (Table II). fed the glomus jugulare. Six (86%) patients, with
glomus jugulare, underwent a type A infratemporal
fossa approach. The remaining one (14%) patient,
Treatment with glomus jugulare, underwent a fallopian bridge
In 12 (48%) cases, the facial nerve schwannoma technique with hypotympanectomy. Currently eight
was resected through a transmastoid approach. (80%) patients have no evidence of disease and
An infratemporal fossa approach was performed two (20%) patients are alive with residual disease.
in seven (28%) patients, a middle fossa approach Multiple metastases were detected in one of the
in two (8%) patients, a middle fossa combined with two patients. The follow up period was 26 months
a transmastoid approach in two (8%) patients, a on average (range 4 months to 10 years).
translabyrinthin approach in one (4%) patient and a The T stage of the temporal bone cancer was T1
transcochlear approach in one (4%) patient. The in eight patients, T2 in six patients, T3 in five
location and size of the mass were the two major patients and T4 in 11 patients. The operation and
factors considered in determining the surgical ap- the treatment outcome for each patient are described
proach. No patient had a recurrence of the schwan- in Table IV. Local resections were performed in two
noma. The follow up period was 6.3 years on average (7%) patients, partial temporal bone resections in
(range from 8 months to 25 years). 16 (53%) patients, subtotal temporal bone resec-
For the lower cranial nerve schwannomas, an tions in seven (23%) patients and the infratemporal
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infratemporal fossa approach type A, with facial fossa approach type A in five (17%) patients. A
nerve rerouting, was performed in all nine cases. modified radical neck dissection was also performed
However, three cases where the temporal bone together with partial temporal bone resection in two
schwannoma was combined with a large intracranial (7%) patients who were thought to have metastatic
mass had staged operations 1 to 2 weeks later in neck nodes. The Mean follow up duration was
order to remove the intracranial portion. Six patients 65 months (range 3 months to 20 years). The five-
had no evidence of residual or recurrent disease year disease free survival was 42% (Figure 2) and
after surgery. The tumor recurred in two patients. that for squamous cell carcinoma was 44% (figure
One of the two patients underwent radiation therapy not shown). The 5-year survival of early stage (T1,
and the other underwent a second revision opera- T2) squamous cell carcinoma was 86% and that
tion. These patients are still alive with recurrent of the advanced stage (T3, T4) squamous cell
disease. Follow up was not possible in one patient. carcinoma was 20% (Figure 3).
The follow up period was 12 years on average (range
2.5 to 21 years).
Management of the facial nerve
For the three patients who had a glomus tympa-
nicum, the transmeatal approach was carried out Because the removal of a facial nerve schwannoma
in one (33.3%) patient and an extended posterior always requires complete resection of a segment of
tympanotomy approach was performed in two the facial nerve, facial nerve repair was performed in
(66.7%) patients (Table III). In cases with glomus all the cases, except one. An interposition graft with
jugulare, the feeding arteries were embolized 1 to the sural nerve was carried out in 12 (48%) patients,
5 days prior to surgery with polyvinyl alcohol (PVA) an interposition graft with the greater auricular nerve
particles. Embolization was not possible in one in four (16%) patients, a XII-VII anastomosis in
Table III. Surgical approaches and treatment outcome of glomus tumors.

Glasscock type Operation No. Outcome

Glomus tympanicum (N3)


Type I Transmeatal approach 1 NED (N1)
Type II Transmastoid approach 2 NED (N2)
Glomus jugulare (N7)
Type I Infratemporal fossa approach 1 NED (N1)
Fallopian bridge technique 1 NED (N1)
Type II Infratemporal fossa approach 1 F/U loss (N1)
Type III Infratemporal fossa approach 1 NED (N1)
Type IV Infratemporal fossa approach 3 AWD (N2), DOC (N1)

NED: no evidence of disease, DOC: dead of another cause, AWD: alive with disease, F/U: follow up.
The glomus tumors were classified according to Glasscock-Jackson classification.
8
C.-S. Kim & M.-W. Suh
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Table IV. Patient data with temporal bone cancer.

No. Sex Age Pathology T stage Operation Facial nerve Adjuvant treatment Recur F/U duration Outcome

1 M 29 ACC T1 PTBR None 128 NED


2 M 47 SqCC T1 PTBR None 70 NED
3 F 70 BCC T1 Excision None Distant 42 NED
4 F 34 BCC T1 PTBR RT 73 NED
5 M 40 SqCC T1 PTBR RT Local, Regional 142 NED
6 M 46 SqCC T1 PTBR RT 62 NED
7 F 48 ACC T1 Excision None Distant 39 NED
8 M 53 SqCC T1 PTBR RT Local 14 AWD
9 M 63 SqCC T2 IFTA B RT 51 NED
10 M 60 SqCC T2 PTBR, MRND IIb None 42 NED
11 F 79 SqCC T2 PTBR RT 90 NED
12 M 56 SqCC T2 PTBR, Supficial parotidectomy RT Regional 5 NED
13 F 56 SqCC T2 PTBR, Supficial parotidectomy None 161 NED
14 F 51 ACC T2 PTBR, Supficial parotidectomy None 120 NED
15 F 59 ACC T3 PTBR, MRND IIb, TRAM RT 36 NED
16 M 66 SqCC T3 ITFA A RT Regional 51 NED
17 F 79 SqCC T3 PTBR None 11 NED
18 M 48 Sebaceous carcinoma T3 STTBR Cable graft None 4 DOD
19 F 53 Apocrine carcinoma T3 PTBR Decompression RT 244 DOC

20 M 61 SqCC T4 STTBR, TRAM Hook up RT Local 63 NED


21 M 52 Chondrosarcoma T4 IFTA A Cable graft RT Distant 72 NED
22 M 58 SqCC T4 PTBR RT 53 NED
23 M 32 Chondrosarcoma T4 PTBR, ITFA A Cable graft RT 31 NED
24 M 46 SqCC T4 STTBR RT Local 129 NED
25 M 40 SqCC T4 STTBR Hook up RT 89 NED
26 F 66 BCC T4 ITFA A Hook up RT 63 NED
27 M 41 ACC T4 PTBR None 10 DOD
28 M 45 SqCC T4 STTBR Hook up RT Local 15 DOD
29 M 65 ACC T4 STTBR RT Local 26 DOC
30 M 60 SqCC T4 STTBR, Total parotidectomy, PMMC Hook up RT 3 DOC

SqCC: squamoud cell carcimoma, ACC adenoid cystic carcinoma, BCC: basal cell carcinoma, PTBR: partial temporal bone resection, STTBR: subtotal temporal bone resection, ITFA A:
infratemporal fossa approach type A, ITFA B: infratemporal fossa approach type B, TRAM: transverse rectus abdominis musculocutaneous free flap, PMMC: pectoralis major myocutaneous flap,
MRND IIb: modified radical neck dissection type IIb, RT: radiation therapy, NED: no evidence of disease, DOD: dead of disease, DOC: dead of another cause, AWD: alive with disease.
Skull base surgery 9

remaining seven (28%) patients had no change in


the performance of the facial nerve. It was also noted
that a facial nerve schwannoma can develop without
any sign of facial nerve paralysis as in two cases in
this series.
For the lower cranial nerve schwannomas, facial
nerve palsy developed in eight (89%) cases after
undergoing an infratemporal fossa approach type A.
Four patients resulted in HB grade III, while the
remaining four resulted in HB grade II. After
removing the glomus tumor via an infratemporal
fossa type A, facial nerve paralysis developed in two
(33%) cases. One case recovered from HB grade II
to grade I, four months later. Among the 30 patients
who underwent surgery for temporal bone cancer,
nine (30%) required facial nerve repair. All cases had
a T stage 3 or 4 cancer with a variety of pathologies
Figure 2. Cumulative survival of temporal bone cancer. The (Table IV). Three (30%) of them underwent a cable
disease free five-year survival was 42%.
graft with the sural nerve or greater auricular nerve.
The distal facial nerve stump was connected to the
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seven (28%) patients and a jump graft to the


proximal portion of the hypoglossal nerve in five
hypoglossal nerve with the sural nerve in one (4%) (56%) patients. In one (11%) patient, only a facial
patient. In the one case where a facial nerve repair nerve decompression was performed. The best result
was not performed, the tumor was peeled from the obtained after repairing the facial nerve was HB
main trunk of the facial nerve without severing the grade III.
nerve itself. In this case, the preoperative and post-
operative facial nerve functions were HB grade II.
The other 24 cases where the facial nerve was Lower Cranial Nerve Function
repaired after segmental resection resulted in a HB Additional cranial nerve palsy that was not present
grade III in interposition graft cases and HB grade preoperatively developed after removing a lower
IV in XII-VII hook up cases. Compared to the cranial nerve schwannoma via the infratemporal
preoperative facial nerve function, the facial nerve fossa approach type A. Vocal cord palsy developed
impairment worsened after surgery in four (16%) in three (33%) patients and abducens nerve palsy
patients, and it improved in 14 (56%) patients. The developed in one (11%) patient.

Case review
A 53-year-old male dentist visited the otology clinic
with a chief complaint of pulsating tinnitus. Five
years previously, the tinnitus had started intermit-
tently; however, recently the nature of the tinnitus
had changed and became continuous. A reddish
mass was visible through the tympanic membrane,
but there was no perforation noted (Figure 4). The
patient had a history of left side facial nerve paralysis
diagnosed 20 years ago, but he had fully recovered.
MRI findings were consistent with glomus jugulare
Glasscock-Jackson classification type III (Figure 5).
The preoperative left side pure tone audiogram
showed air conduction of 70 dB and the bone
conduction of 30 dB. The hearing on the right side
was normal.
Angiography and embolization were performed
Figure 3. Cumulative survival of temporal bone cancer by stage.
The 5-year survival of early stage (T1, T2) squamous cell five days before surgery (Figure 6). The tumor was
carcinoma was 86% and that of the advanced stage (T3, T4) suspected to have originated from the hypotympa-
was 20%. num based on the intraoperative findings. The mass
10 C.-S. Kim & M.-W. Suh

view of the tumor. The glomus tumor was removed


very carefully. There was no invasion noted into the
otic capsule. The tumor extended into the petrous
apex, pericarotid region and above the internal
jugular vein. The distal portion of the sigmoid sinus
and the jugular bulb was removed because the tumor
had infiltrated into these vascular structures. The
carotid artery was preserved. Cranial nerves IX, X,
XII were identified and they were all preserved. After
completely removing the tumor (Figure 7), the
Eustachian tube, middle ear and mastoid were
obliterated with an abdominal fat and temporalis
muscle flap. The immediate postoperative facial
nerve function was HB grade II, but it was recovered
to HB grade I after four months. There was no
evidence of any damage to the other lower cranial
nerves. The patient was followed for three years with
no evidence of recurrence.
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Figure 4. Tympanic membrane of the patient with glomus


jugulare tumor. A reddish pulsating mass was visible through
the tympanic membrane. Discussion
Schwannoma
was removed by an infratemporal fossa approach
type A. After separating the incudostapedial joint, Surgical resection is the primary approach to the
the incus was removed. The long process of the incus treatment of a facial nerve schwannoma. However,
was eroded by the tumor. The retrofacial air cells this surgery is almost always associated with a
and infralabyrinthin air cells were occupied by the significant degree of facial nerve paralysis [6,7].
tumor. The facial nerve was skeletonized and Therefore, some clinicians prefer to wait or to only
rerouted anteriorly from the geniculate ganglion to decompress the tumor in order to preserve facial
the main trunk of the extratemporal portion. Re- nerve function for as long as possible [8,9]. Although
routing of the facial nerve enabled a much wider in this review, we focused only on surgical cases we
agree in part with this cautious approach. Normal or
good facial nerve function better than an HB grade
III, cannot be expected once the continuity of the
nerve is severed; as supported by the results pre-
sented in this paper. The only patient with a HB
grade II result, the best outcome in our series,
resulted from dissecting off the tumor from the
main trunk without cutting it. Preoperative counsel-
ing of the outcome of facial nerve function after
surgery is an important part of management. This is
especially important for those patients whose facial
nerve function is near normal preoperatively. We had
two patients with good facial nerve function before
surgery; however, it was not possible to remove
the tumor and preserve facial nerve function,
because the facial nerve fanned out and the full
extent of the tumor could not be completely traced.
Angeli and Brackmann [8] reported that wide
decompression is an alternative to surgical excision,
especially in cases where facial neuromas are not
suspected and in patients with normal facial func-
Figure 5. MRI findings of the patient with glomus jugulare tumor.
A glomus jugulare tumor is occupying the left middle ear, mastoid
tion. The management of facial nerve schwannomas,
cavity and petrous apex. MRI findings were consistent with glomus in our department, has become slightly more con-
jugulare tumor of Glasscock-Jackson classification type III. servative during the last two decades.
Skull base surgery 11

A B

Figure 6. Pre-embolization and post-embolization angiography findings of the patient with glomus jugulare tumor. Angiography and
embolization were performed 5 days before surgery. The ascending pharyngeal artery, occipital artery and caroticotympanic artery were
suspected to be the feeding vessels. The visible mass (A) disappeared in the post-embolization angiography (B).

There were two children with facial nerve schwan- this approach. A transmastoid approach was used
nomas in our series. The age at first visit to the when a small tumor was located distal to the
hospital was 13 and 17 months respectively. How- geniculate ganglion. For tumors, involving the labyr-
ever, all the photos and video films taken by their inthine segment, a middle fossa approach or com-
bined middle fossa and transmastoid approach was
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parents showed that these babies have had facial


paralysis from birth, indicating the possibility of used. The postoperative facial nerve function was
prenatal presence of the facial nerve schwannoma. HB grade II in the case of combined middle fossa
Although many cases have been reported in the and transmastoid approach.
world literature, facial nerve tumors in such young
patients are very rare. A detailed history of these two Glomus Tumor
patients has been reported elsewhere [10]. A facial According to Sanna et al [11] the most common
nerve schwannoma should also be considered in the symptoms caused by a glomus tumor were hearing
differential diagnosis when a young child presents loss and pulsatile tinnitus, consistent with our
with unilateral facial nerve paralysis. results. Among these symptoms, unilateral pulsating
The preoperative hearing level, location and size tinnitus was a unique characteristic associated
of the tumor were important factors to consider with these tumors. We treated five patients with
when selecting an operative technique. When the this symptom. Other causes that can also produce
hearing level was poor and the tumor was big, the pulsation tinnitus should be considered such as an
infratemporal fossa approach was frequently used. aberrant carotid artery, high jugular bulb and
However, in those patients who had serviceable arteriovenous malformation. O’Leary et al [12]
hearing it was difficult to preserve the hearing with reported that an aberrant carotid artery is more

Figure 7. Operative findings after removing the whole mass (left eat). The glomus tumor was completely removed together with the jugular
bulb. The sigmoid sinus and carotid artery are exposed and the facial nerve rerouted anteriorly. VII: facial nerve, ICA: internal carotid
artery, SS: sigmoid sinus, C: cochlea, L: labyrinth, Et: Eustachian tube, Ant: anterior, Post: posterior, Sup: superior, Inf: inferior.
12 C.-S. Kim & M.-W. Suh

likely when the mass is located at the anterior gery. Postoperatively, one patient suffered from
portion of the tympanic membrane and a glomus multiple lower cranial nerve palsies including cranial
tumor or a high jugular bulb are more likely nerves IX, X, XII; the rest of the patients were
when the mass is located at the posterior portion. successfully managed without damaging other lower
Although this point may be straightforward, it cranial nerves. However, as demonstrated by our
cannot be applied to all cases. There were seven series of lower cranial nerve schwannomas, the
patients in our series who were operated on due to a infratemporal fossa approach type A has the poten-
glomus jugulare but only two patients had the typical tial risk of facial nerve impairment. During the
postero-inferior tympanic mass. However, it seems standard infratemporal fossa approach type A, the
to follow that the mass is located at the inferior facial nerve was removed from the fallopian canal
portion of the tympanic membrane. The location of and rerouted anteriorly. Devascularization of the
the tympanic mass of the glomus tumor is rather vasa nervorum is the suspected cause of facial nerve
dependent on the stage of the disease process. palsy during this procedure. Although the facial
Although the majority of glomus tumors are thought nerve impairment may be mild and/or transient,
to secrete catecholamine, only 2% of glomus tumors it should be considered as a specific risk associated
are reported to produce clinical symptoms [13]. with this type of surgery. We currently use the
Typical symptoms may be severe headache, pallor, fallopian bridge technique with hypotympanectomy
palpitation, nausea, and vomiting. When the tumor in selected cases. This is an approach that removes
is functioning, it can abruptly release catecholamine the temporal bone inferior to the vestibule and
cochlea leaving the fallopian canal in situ [21].
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into the bloodstream during surgery and cause


disastrous cardiovascular events [14]. Twenty-four There is a better chance of preserving the facial
hour urine VMA and metanephrine should be nerve function and hearing compared to the infra-
evaluated before surgery in order to prevent such temporal fossa approach type A. A patient who
emergencies. We also had one patient with a underwent this procedure currently has a facial nerve
functioning tumor in our series of patients. function of HB grade I and her postoperative hearing
Recent advances in stereotactic radiosurgery sug- is excellent with no air bone gap.
gest that radiation therapy has similar or better
results than surgery for management of glomus
Temporal bone cancer
tumors. Although these tumors are typically radio-
sensitive, radiation therapy can eliminate the entire Squamous cell carcinoma and adenocarcinoma were
tumor in only 30% of cases [15]. The goal of the two most common histological types of temporal
radiation is growth inhibition rather than elimination bone carcinomas, consistent with other studies [22].
of the tumor [16]. Therefore, surgery is superior to Of the various symptoms of temporal bone cancer,
radiation for immediate and complete tumor elim- otalgia is among the most common. In addition,
ination [17]. Moreover, residual tumor may remain investigators have suggested that the character of
dormant for the rest of the patient’s life in some pain becomes more deep seated and unremitting
cases, but in others, it may become a malignant as the disease advances [23,24]. Many patients
tumor. It is reported that 1 to 5% of glomus jugulare visit the otology clinic with nonspecific ear pain.
tumors are malignant and exhibit metastatic spread Although the majority of clinic patients do not have
[18,19]. We treated one such patient out of 10 in any specific abnormality, clinicians must be able to
our series that had distant metastasis after malignant distinguish those among them that may have a
transformation. Complete resection of the tumor temporal bone malignancy. Deep seated and unre-
can be safer than leaving residual tumor after mitting pain may be a sign that alerts the clinician to
radiation therapy. Radiation may have a more a more serious problem.
important role in salvage treatment for patients Various staging systems for temporal bone cancer
who cannot undergo surgery because of unresectable have been proposed. However, there is no consensus
tumors or residual disease. on any of the staging systems proposed to date.
In our experience and from other reports, glomus Some investigators have suggested the need for a
tympanicum was usually accessible via a transmeatal multi-institutional effort to develop a staging system
approach or an extended posterior tympanotomy and prospective treatment protocols [25]. The lack
approach [12,20]. However, glomus jugulare re- of a unified staging system makes it difficult to
quired greater consideration of residual functions compare treatment outcomes between studies. In
of hearing and the lower cranial nerves. Six of the this article, we used the Pittsburgh staging system
seven patients with glomus jugulare were treated proposed by Arriaga in 1990. Originally, this staging
with the infratemporal approach type A. Most of system was proposed to classify cancer of the
them did not have serviceable hearing before sur- external auditory canal. It may not be appropriate
Skull base surgery 13

for classifying temporal bone cancers of other strength and weakness of each surgical technique
origins. Although there are limitations using this and knowledge of the particular tumor biology
staging system, the Pittsburgh staging system seem may facilitate selection of the appropriate surgical
to be the most widely used for similar cases approach and result in a successful outcome.
currently.
The 5-year survival rate for patients with temporal
bone cancer in other studies has been reported to be
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