Neurogenic tumors 12

and paragangliomas

INTRODUCTION m o n of all paragangliomas in t h e head a n d neck region. Histo-

logically it is difficult to differentiate between benign and malignant
paragangliomas. However, malignant paragangliomas are diagnosed
Neurogenic t u m o r s a n d paragangliomas f o r m a very s m a l l
by the presence of metastatic disease w h i c h occurs in less than
percentage of all neoplastic lesions of the head a n d neck region.
10% of all patients w i t h paragangliomas. Approximately 10% of
In general, these tumors are considered under the major heading
the patients have m u l t i p l e or bilateral tumors. A detailed family
of 'soft tissue t u m o r s ' . I lovvever, the u n i q u e nature of presentation
history should be o b t a i n e d , since approximately 10% of patients
of these tumors a n d the systematic w o r k u p necessary for accurate
have other members of the family w i t h a history of paragangliomas.
diagnosis to facilitate treatment warrant the need to consider
Specific genetic abnormalities are identified in members of these
these tumors as a separate e n t i t y f r o m other soft tissue tumors.
families w i t h tumors, a l t h o u g h these genetic abnormalities have
The histologic classification of neurogenic tumors of the head and
not been employed as a diagnostic test as yet.
neck is s h o w n in F i g . 1 2 . 1 .
The head a n d neck region is by far the most c o m m o n location
Benign Malignant
for benign peripheral nerve tumors ( F i g . 12.2). Schwannomas
can arise in any part of the head a n d neck region a n d can occur in Reactive M a l i g n a n t Peripheral Nerve
the head, face, scalp, cranial cavity, o r b i t , nasal cavity, oral cavity, Sheath Tumors
Traumatic neuroma (Malignant Schwannoma,
larynx, o r i n t h e m i d d l e ear. O n the o t h e r h a n d , neurofibromas o r
Neu rof i brosarcoma)
schwannomas of the cervical region are divided i n t o those arising
- Epitheloid MPNST
in the medial c o m p a r t m e n t of the neck a n d those arising in the
- Pigmented (Melanotic) MPNST
lateral compartment of the neck. Neurogenic tumors of the medial
- Peripheral Primitive
compartment of the neck arise f r o m the last four cranial nerves Neuroectodermal tumor (Askin
(glossopharyngeal, vagus, accessory, a n d hypoglossal) or t h e s y m - tumor)
pathetic c h a i n . Neurogenic tumors of the lateral c o m p a r t m e n t of H a m a r t o m a : Mucosal A u t o n o m i c nerve t u m o r
the neck arise from the cutaneous or muscular branches of the Neuromas (MEN MB, (plexosarcoma)
cervical plexus or f r o m the brachial plexus. Occasionally these Gorlin's syndrome)
tumors arise at the spinal f o r a m i n a w i t h an intraspinal a n d an Schwannoma M a l i g n a n t M e l a n o m a of soft
extraspinal c o m p o n e n t presenting as a ' d u m b - b e l l t u m o r ' . (Neurilemmoma) tissues (clear cell sarcoma)
N e u r o f i b r o m a : Solitary,
Most paragangliomas are histologically benign lesions arising
Multiple, Diffuse,
from the paraganglionic tissue related to the arterial vasculature or
Plexiform(NF-l)
cranial nerves in the head and neck region. Thus paragangliomas
P e r i n e u r i o m a (Storiform
of the head and neck region can arise in the j u g u l o - t y m p a n i c perineurial fibroma)
region, vagal body, carotid body, superior and inferior laryngeal D e r m a l nerve s h e a t h
paraganglionic tissue, in t h e nasal cavity or in (he orbit. The myxoma
distribution of paragangliomas of the head and neck region is Granular cell t u m o r
shown in F i g . 12.3. C a r o t i d b o d y t u m o r s are by far t h e most c o m -
Fig. 12.1 Histologic classification of neurogenic tumors of the head and
neck.

Fig. 12.2 The location of benign peripheral nerve tumors. Fig. 1 2 3 Distribution of paragangliomas of the head and neck region.
NEUROGFNIC TUMORS AND PARAGANGLIOMAS
CLINICAL FEATURES AND DIAGNOSIS
As indicated earlier, the majority of the patients arc asymptomatic
and present for diagnosis and treatment with the complaints of a
mass in the neck or oilier parts of the head and neck area. If the
mass is arising from or related to the arterial wall, it may he
pulsatile. On the other hand, schwannomas and neurofibromas
are firm discrete lesions which do not transmit pulsations. The
tumor is usually mobile in an axis perpendicular to the long axis
of the nerve involved and does not manifest any mobility along
the long axis of the nerve of involvement. On occasion, accurate
diagnosis can be made simply by clinical examination. Occasion-
ally paralysis of the involved cranial nerve may be the first
presenting symptom.
Fig. 12.4 Patient with pulsatile mass in the upper part of the neck.
Fig. 12.S CT scan of
the neck with contrast
enhancement.
476
RADIOGRAPHIC EVALUATION
CT and MRI scans have revolutionized the diagnostic capabilities
of neurovascular lesions in the head and neck area. Prior to the
availability of these studies, direct angiography was the only
confirmatory test for a carotid body tumor or a paraganglioma.
However, due to the increased risk of complications from direct
angiography, nowadays it is seldom performed unless preoperative
embolization of the tumor is intended. A CT scan with contrast
enhancement provides an accurate diagnosis of a neurovascular
tumor such as a carotid body tumor. The patient shown in
Fig. 12.4 has a 7 cm pulsatile mass in the upper part of the neck
on the right-hand side splaying the carotid vessels. A CT scan with
contrast enhancement shows splaying out of the carotid arteries
Fig. 12.6 Common carotid angiogram.
Fig. 12.7 Axial view of
the patient's MRI scan.
 I 2 NEUROGENIC TUMORS AND PARAGANGLIOMAS

w i t h an intense enhancement of the h i g h l y vascular lesion ( F i g . FACTORS AFFECTING C H O I C E OF TREATMENT

12.5). A direct angiogram of the same patient demonstrates the
vascularity of the lesion in relation to the carotid arteries ( F i g .
The indications for surgical i n t e r v e n t i o n for excision of neuro-
12.6). In most instances the m a j o r i t y of the b l o o d supply of the
genic or neurovascular t u m o r s depends on the symptoms of the
tumor comes f r o m the peripheral branches of the external carotid
patient, size and location of (he lesion, m u l t i p l i c i t y of the lesions,
artery. If a direct angiogram is performed, then that o p p o r t u n i t y
and the risk of surgical intervention. Since a majority of the patients
may be used to consider selective e m b o l i z a t i o n of the t u m o r if
have unifocal lesions, surgical excision should be considered to
feasible to facilitate expeditious surgical removal w i t h o u t undue
prevent progressive neurologic deficit due to an enlarging tumor
hemorrhage.
mass. Relatively small neurovascular tumors usually involve only
MRI e x a m i n a t i o n has further enhanced the capability of
o n e cranial nerve, but as the t u m o r enlarges involvement of
accurate diagnosis. T V w e i g h t e d images anil g a d o l i n i u m contrast
m u l t i p l e cranial nerves becomes a cause for concern. The disability
enhancement have greatly facilitated accuracy of diagnosis of
resulting f r o m c o m p r o m i s e of f u n c t i o n of multiple cranial nerves
these lesions w i t h an MRI (Figs 1 2 . 7 - 1 2 . 9 ) . In a d d i t i o n to this, an
is significant a n d therefore early surgical intervention should be
MRI angiogram is a non-invasive (est w h i c h accurately depicts the
considered in patients w h o are deemed satisfactory surgical can-
disposition of the carotid arteries in relation to the mass to facilitate
didates. G l o m u s Lntravagale and glomus jugulare tumors in high
surgical excision (Figs 1 2 . 1 0 a m i 12.11). MRI is also of significant
risk patients can be controlled w i t h radiation therapy which retards
help in the d e m o n s t r a t i o n of neurogenic tumors w i t h intra- a n d
the g r o w t h of the t u m o r a l t h o u g h a persistent radiologic finding
extra-spinal extension (dumb-bell tumors). A myelogram is seldom
may be present for several years after treatment. Therefore, selec-
necessary since a MRI scan provides equally good i n f o r m a t i o n
t i o n of i n i t i a l treatment depends on the size and location of the
w i t h appropriate contrast enhancement.
tumor, the surgical risk, and the risk of cranial nerve deficits
resulting from surgery versus the potential benefit to be derived
Fig. 12.8 Coronal view
f r o m non-surgical treatment.
of the patient's MRI
scan.

Fig. 12.9 Sagittal view

of the patient's MRI
scan.

Fig. 12.10 MR
angiogram showing
displacement of the
carotid vessels. Fig. 12.11 MR
angiogram showing
tumor blush.

477
NEUROGENIC TUMORS A N D PARAGANGLIOMAS
PREOPERATIVE PREPARATION
All patients undergoing surgical resection for neurogenic, neuro-
vascular tumors, or paragangliomas must have an understanding
of the nature of the surgery, the risks involved, and explanation
regarding potential nerve deficits that may result following
surgery. If the lesion is of large dimensions and highly vascular,
then consideration should be given to preoperative embolization.
This should be performed ideally within 24 hours of the planned
surgical resection. Although transfusion is seldom required, blood
should be available on demand in the event of unexpected hemor-
rhage. For larger lesions, integrity of the intracranial crossover
circulation should be confirmed with occlusion of the ipsilateral
carotid artery anil cross-perfusion from the contralateral carotid
and vertebral arteries to assess the risk of neurologic deficit if
ligation of the carotid artery becomes necessary. Similarly, the
services of a vascular surgeon should be available on standby in
the event of the need for resection of a segment of the artery with
replacement if indicated.
CAROTID BODY TUMOR
A carotid body tumor, chemodectoma or non-chromaffin para-
ganglioma is a tumor of neurovascular origin arising from the
chemoreceptor cells in the carotid sheath, most commonly located
at the bifurcation of the carotid artery. These tumors are highly
vascular and are histologically benign in most instances. They are
slow growing and therefore may present with a long-standing
history. The characteristic location is in the upper part of the neck
where it may present as an external mass, or as a parapharyngeal
mass pushing the lateral pharyngeal wall medially in the
478
oropharynx. In either location, the mass usually has palpable
pulsations. If the clinical diagnosis of a carotid body tumor is
suspected, appropriate radiographic studies must be performed
prior to embarking on the surgical excision.
Although carotid angiography provides the best radiographic
picture, there is some risk involved with direct angiographic studies
and, therefore, it is usually not recommended unless embolization
of the tumor is planned. On the other band, a CT scan with
contrast is quite diagnostic and provides sufficient information for
the clinician to proceed with surgical resection. MRI is also very
accurate in demonstrating the location and extent of these tumors
as well as the relationship of carotid vessels to the tumor. How-
ever, if an angiographic study is necessary, then an MR angiogram
or digital intravenous angiography is recommended as opposed to
arteriography, since it provides adequate information to plan the
indicated surgery.
Shamblin classified carotid body tumors into three categories,
based on the relationship of the carotid arteries and the adjoining
nerves to the tumor (Fig. 12.12). In type 1 tumors, the arteries are
simply displaced by the tumor and lie on the surface of the tumor.
In type II, the tumor is indented by the external and internal
carotid arteries, making a deep groove within the tumor and the
hypoglossal and superior laryngeal nerves are on the tumor's
surface. In type III the arteries and the nerves are encased by the
tumor.
The patient shown in this procedure has a 6 cm pulsatile mass
in the upper part of the neck on the right-hand side. Radiographic
investigations included an MRI scan in axial and coronal planes as
well as an MR angiogram. An axial view of the MRI scan shown in
Fig. 12.13 demonstrates a tumor mass separating (he internal and
external carotid arteries which appear embedded within the sub-
stance of the tumor. This is a Shamblin type II lumor. An MR
angiogram demonstrates splaying of the external and internal
Fig. 12.12 Shamblin's classification.

carotid artery by the tumor on the right-hand side (Fig. 12.14).
The disposition of the tumor in relation to the common, internal
and external carotid arteries is shown on a surface marking on the
patient in Fig. 12.15.
The operative procedure is performed under general endo-
tracheal anesthesia. A biopolar cautery is essential and must be
available for safe conduct of the operation. Vascular instruments
such as vascular clamps, vessel loops and Fogarty catheters should
also be readily available. The patient is placed in a supine position
on the operating table with the neck extended and rotated to the
opposite side (Fig. 12.16). The incision is placed along an upper
neck skin crease providing generous exposure of the structures in
the upper part of the neck. Upper and lower skin flaps are elevated
in the usual fashion. Since the plane of dissection Is deep to the
sternocleidomastoid muscle, the greater auricular nerve overlying
this muscle can be safely preserved. Meticulous dissection of the
skin Haps with electrocautery is recommended to minimize blood
Fig. 12.13 An axial
view of MRI scan
showing a carotid body
tumor separating the
internal and external
carotid arteries.
Fig. 12.14 MR
angiogram
demonstrates that
the carotid arteries
are splayed by the
tumor on the right-
hand side.
12 NEUROGENIC TUMORS AND PARAGANGLIOMAS
loss. Due to the high vascularity of this tumor, rough handling or
dissection in an inappropriate plane will cause significant blood
loss. However, if gentle and careful dissection is undertaken, the
entire operative procedure can be safely accomplished without the
need for a blood transfusion.
Several hyperplastic lymph nodes are often encountered
overlying the carotid body tumor in the upper part of the neck
(Fig. 12.17). These hyperplastic lymph nodes are best excised at
the outset to provide adequate exposure of (he underlying major
blood vessels and the tumor. Following excision of overlying
hyperplastic lymph nodes, the underlying carotid body tumor
comes into the picture (Fig. 12.18). Note the internal jugular vein
skirting the posterior surface of the tumor and the common facial
vein skirting the lower border of the tumor. The initial step in the
Operative procedure at this juncture is dissection of the common
carotid artery proximal to the tumor. The lower skin flap is
elevated enough to provide the necessary exposure.
Fig. 12.15 Surface markings of the tumor in relation to the common,
external and internal carotid arteries in the patient.
Fig. 12.16 A skin incision is placed in an upper neck skin crease.
479
NEUROGENIC TUMORS A N D PARAGANGLIOMAS
The fascia on the anterior border of the sternomastoid muscle is
incised and the muscle is retracted laterally to expose the carotid
sheath. The carotid sheath is opened in the lower part of the neck
and the common carotid artery is exposed, dissected circum-
ferentially and isolated with a vessel loop.
Once proximal control of the common carotid artery is secured,
dissection of the tumor begins. The incision in the fascia overlying
the sternomastoid muscle is extended cephalad up to the tail of
the parotid gland. The sternomastoid muscle is now retracted
laterally to expose and isolate the lateral border of the tumor (Fig.
12.19). At this juncture, il is imperative thai the vagus and the
hypoglossal nerves are carefully identified and dissected to
preserve their integrity. Dissection now proceeds along the com-
mon carotid artery in a subadventitial plane until its bifurcation.
This is a very tedious, slow and meticulous dissection during
which absolute hemostasis must be maintained throughout. The
usual technique is to electrodesiccate the adventitia of the carotid
artery bearing the abnormal hypervascular tissue with the bipolar
cautery and sharply divide the coagulated tissue with fine tenotomy
scissors. Every minor bleeding point should be electrodesiccated
Fig. 12.17 Hyperplastic lymph nodes overlying the tumor are excised
first.
Fig. 12.18 Carotid body tumor encasing the carotid bifurcation.
480
with the bipolar cautery as dissection proceeds to minimize
hemorrhage. In most instances, the carotid artery and its
bifurcation are partially embedded producing indentation in the
tumor rather than circumferential encasement (Shamblin type II
tumor). Occasionally, however, the tumor surrounds the
common carotid artery and its bifurcation and the tumor may
have to be incised and bisected to dissect the artery out (Shamblin
type III tumor). If this is the case, absolute hemostasis should be
secured with the electrocautery during division of the tumor to
facilitate a bloodless procedure and the safety of the rest of the
operation.
Further dissection of the common carotid artery provides
exposure of the carotid bifurcation. At this juncture the tumor is
lifted off the carotid artery and retracted cephalad to facilitate
further dissection (Fig. 12.20). Use of sharp and blunt dural
dissectors (Penfield) greatly facilitates subadventitial dissection. It
is important to remember that nearly all the blood supply to this
tumor is derived from the branches of the external carotid artery.
However, dissection of the internal carotid artery is undertaken
fust to protect it from injury. The artery is completely isolated
Fig. 12.19 The vagus and hypoglossal nerves are dissected and
preserved.
Fig. 12.20 The tumor is lifted off the carotid bifurcation.

before further mobilization of the tumor is undertaken. Liberal use
of monopolar electrocautery for dissection and bipolar electro-
cautery for hemostasis is recommended.
During the course of dissection, near the carotid bifurcation,
bradycardia and hypotension may occur clue to stimulation of
the baroreceptors at the carotid bifurcation. Infiltration of 1%
Lidocaine (lignocaine) in the subadventitial plane of the carotid
bulb will promptly reverse the bradycardia and hypotension.
As dissection of the tumor proceeds cephalad, its blood supply
coming from the adventitial vessels of the carotid arteries is cut
off. This causes shrinkage in the size of the tumor which also feels
softer and makes subsequent dissection progressively easier (Fig.
12.21). Finally, dissection of the external carotid artery from the
tumor is undertaken. At this point, if it appears that the external
carotid artery is inseparable from the tumor, then it may be safely
cross-clamped, divided and ligateel distal to the carotid bulb.
However, in most instances, the external carotid artery with its
branches can be separated from the tumor. Each feeding vessel to
the tumor from the branches of the external carotid artery is
dissected and ligated or coagulated.
Fig. 12.21 The internal and external carotid arteries are dissected away
from the tumor.
Fig. 12.22 The surgical field following removal of the tumor shows
intact carotid bifurcation and adjacent cranial nerves.
"IT NEUROGENIC TUMORS AND PARAGANGLIOMAS
It is always desirable to have a vessel loop passed around the
distal part of the internal carotid artery and the external carotid
artery to provide retraction as well as immediate control of bleeding
should any unexpected hemorrhage ensue. Occasionally, feeding
blood vessels from the wall of the carotid artery arc so short that
ligation may not be feasible and repair of the bleeding from the
carotid artery may require a ligure of eight fine vascular suture.
Finally the specimen is removed without undue hemorrhage if
all the precautions mentioned above are undertaken. The surgical
field following the removal of the tumor demonstrates the carotid
bifurcation with intact internal and external carotid artery with its
branches as well as the preserved hypoglossal and vagus nerves
and internal jugular vein (Fig. 12.22).
The specimen removed from this patient is shown in Fig.
12.23. Note that the tumor appears almost bilobulated with deep
indentation created at the carotid bifurcation by the external and
internal carotid arteries in the substance of the tumor. This is a
classic Shamblin type II tumor. The cut surface of the tumor is
homogeneous and fleshy in consistency with areas of focal
hemorrhages (Fig. 12.24).
Fig. 12.23 External
appearance of the
tumor shows a
bilobulated tumor
showing indentation
due to encasement of
the carotid arteries.
Fig. 12.24 The cut
surface of the tumor
shows a homogeneous
fleshy lesion with focal
hemorrhages.
481
NEUROGENIC TUMORS A N D PARAGANGLIOMAS
CHEMODECTOMA OF THE SUPERIOR MEDIASTINUM
The patient shown here had multiple paragangliomas in the head
and neck region, lie presented with a family history of glomus
jugulare and glomus intravagale tumors and he had bilateral
carotid body tumors as well as right-sided glomus intravagale and
superior mediastinal paraganglioma. He had previously under-
gone sequential excision of the bilateral carotid body tumors and
glomus intravagale on the right-hand side. In the present surgical
procedure, the paraganglioma located in the superior media-
stinum posterior to the innominate artery is being resected.
The CI scan of the patient through the lower part of the neck
and the superior mediastinum shows the presence of a highly
vascular lesion posterior to the common carotid and innominate
arteries on the right-hand side (Fig. 12.25). An MR! scan vividly
demonstrates the position of the innominate artery just anterior
to the tumor mass which is wedged between the trachea medially
and the innominate artery anteriorly (Fig. 12.26). A direct angio-
gram performed through the arch of the aorta demonstrates the
tumor blush posterior to the innominate artery in an anatomic-
region between the common carotid takeoff and the vertebral
artery takeoff from the innominate artery (Fig. 12.27). During
Fig. 12.25 CT scan at the level of the suprasternal notch.
Fig. 12.26 MRI scan at the level of the suprasternal notch.
482
the venous phase of the angiogram, the rich vascularity of the
tumor is vividly demonstrated (Fig. 12.28).
Surgical approach to superior mediastinal paraganglioma
requires a cervicothoracic exposure to gain access to the great
vessels in the superior mediastinum as well as distal control of the
carotid and subclavian arteries at the root of the neck. The location
of the tumor and the position of the great vessels are shown on
the patient in relation to the exposure necessary outlined by the
incision (Fig. 12.29).
A transverse cervical incision is taken in the supraclavicular
region beginning at the anterior border of the trapezius muscle
and curving anteriorly to the midline in the suprasternal notch. At
(his point, the incision is continued vertically caudad in the mid-
line up to the third intercostal space (Fig. 12.SO). The skin
incision is deepened through the platysma and the sternocieido-
Fig. 12.27 Arterial phase of a
selective right innominate
angiogram.
Fig. 12.28 Venous phase of
angiogram.
 NEUROGENIC TUMORS AND PARAGANGLIOMAS

mastoid muscle is detached from the sternoclavicular insertion. A
median sternotomy is performed using a sternal saw d i v i d i n g the
manubrium in the m i d l i n e up to the manubrial-sternal j u n c t i o n .
At that point, the incision in the sternum is taken laterally
through the t h i r d interspace (o permit o p e n i n g of the superior
mediastinum in a 'clam-shell' fashion, hi order to gain further
exposure, it may be necessary to d i v i d e the clavicle at the j u n c t i o n
of its middle and medial t h i r d ( F i g . 1 2 . 3 1 ) . A self-retaining
sternotomy retractor is used to provide exposure of t h e anterior
superior m e d i a s t i n u m . Meticulous dissection of t h e great vessels
now begins, carefully i d e n t i f y i n g the i n n o m i n a t e artery at its
takeoff from t h e ascending aorta a n d its distal branches, i.e. t h e
c o m m o n carotid artery ( F i g . 12.32), the subclavian artery, a n d
the vertebral artery. It may he necessary to identify, d i v i d e and
ligale the internal m a m m a r y artery to facilitate further exposure.
At this point, it clearly becomes evident that the t u m o r is located
posterior and inferior to t h e i n n o m i n a t e artery. Meticulous
dissection along the subadventitial plane of the i n n o m i n a t e artery
is undertaken, carefully coagulating all the bleeding p o i n t s w h i l e
mobilizing the tumor. The right recurrent laryngeal nerve is
identified crossing the i n n o m i n a t e artery a n d c u r v i n g upward.
However, this patient had previously undergone resection of a
glomus intravagale t u m o r on the same side and therefore the
integrity of the recurrent laryngeal nerve was not of significant
importance due to the already existent paralysis of the right vocal
cord. Vessel loops are passed a r o u n d the i n n o m i n a t e artery, the
c o m m o n carotid artery, and the subclavian artery (Fig. 12.33).
W i t h alternate medial and lateral traction using the vessel loops,
the anterior attachments of the t u m o r to the posterior aspect of
t h e i n n o m i n a t e artery are d i v i d e d , f u r t h e r mobilization of the
t u m o r continues in a careful a n d delicate fashion, freeing up all
the small b l o o d vessels f r o m the loose areolar attachments around
Fig. 12.31 Median
s t e r n o t o m y is
c o m p l e t e d and the
site of division of the
clavicle is outlined.

Fig. 12.29 The

location of the tumor is
outlined on the patient
| in relation to the great
vessels.

"* Fig. 12.32 The

innominate, common
carotid and subclavian
arteries are dissected
a n d isolated.

Fig. 12.30 The

transverse cervical
incision is continued
vertically in the midline
caudad.

483
NEUROGENIC TUMORS A N D PARAGANGLIOMAS 7
the pseudocapsule of the tumor. Digital dissection often facilitates
mobilization of the tumor, keeping absolute hemostasis in m i n d
at all times. Once the anterior aspect of the t u m o r is adequately
mobilized, the t u m o r is grasped w i t h a Kelly clamp and its m o b i l -
ization begins in the prevertebral plane, f o l l o w i n g circumferential
mobilization a n d dissection of the tumor, it is delivered in a
monobloc fashion w i t h its pseudocapsule intact. The surgical field
following removal of the t u m o r is s h o w n in F i g . 12.34. A close-
up view shows the t u m o r bed between t h e i n n o m i n a t e a n d
subclavian arteries medially and the subclavian vein superiorly
( F i g . 12.35). After securing absolute hemostasis, w o u n d closure
begins using an A - 0 plate a n d screws to realign the clavicle a n d
stainless steel wires to reapproximate t h e d i v i d e d s t e r n u m . The
wound is then closed in t w o layers after a suction drain is appro-
priately positioned ( F i g . 12.36). In F i g . 1 2 . 3 7 the external
Fig. 12.33 Close-up view of the surgical field Fig. 12.34 The surgical field following removal
shows the tumor located posterior to the great of the tumor.
vessels.
Fig. 12.36 Closure of the wound. Fig. 12.37 External appearance of the tumor.
484
appearance of the t u m o r is s h o w n w h i c h measures 5 x 4 cm in
diameter. On cross-section, it appears fleshy and is l u l l of vascular
spaces, an appearance characteristic for a chemodectoma ( F i g .
12.38).
Surgical excision of carotid body tumors, chemodectomas, or
n o n - c h r o m a f f i n paragangliomas in the head and neck region and
superior m e d i a s t i n u m is a safe surgical undertaking as long as
appropriate preoperative radiographic evaluation is undertaken to
assess the a n a t o m i c dimensions a n d location of the t u m o r and
consideration is given to e m b o l i z a t i o n of the t u m o r if demon-
strable feeding vessels are identified on direct angiography. In
most instances, e m b o l i z a t i o n is n o t required. Blood transfusion is
seldom indicated a n d w i t h due diligence o f delicate conduct i n
the operating r o o m , hazards such as i n j u r y to major vessels or
their u n i n t e n t i o n a l sacrifice are avoided.
Fig. 12.35 Close-up view of the surgical field
following removal of the tumor.
Fig. 12.38 Cross-section of the tumor.

GLOMUS INTRAVACALE
The patienl shown here presented with a history of a mass in the
upper cervical region and local discomfort due to pressure from
the mass. On palpation, she had a well-defined retromandibular
mass whose lower border could be easily palpated in the upper
part of the neck, although its upper half could not be palpated due
to its location in the retromandibular space. An MRI scan in the
sagittal and coronal planes vividly demonstrates the anatomic
location and the dimensions of the tumor which are approxi-
Fig. 12.39 Sagittal view of the MRI scan showing location of the tumor.
Fig. 12.40 Coronal view of the MRI scan showing location of the tumor.
NEUROGENIC TUMORS AND PARAGANGLIOMAS
mately 4 x 7 cm (Figs 12.39 and 12.40). The tumor is located
along the carotid sheath, displacing both the external and internal
carotid arteries anteromedially and the internal jugular vein
posterolateral!}' (Fig. 12.41).
Surgical exposure for this lesion requires an incision beginning
at the tip of the mastoid process and curving along an upper neck
skin crease anteriorly up to the midline of the neck in the sub-
mental region (Fig. 12.42). The skin incision is deepened
through the platysma and upper and lower cervical flaps are
developed lo expose the tumor (Fig. 12.43). Several hyper-
plastic deep jugular lymph nodes are encountered overlying the
Fig. 12.41 Location of the tumor in relation to the carotid vessels.
Fig. 12.42 The location of the tumor and the incision are outlined.
485
NEUROGENIC TUMORS A N D P A R A G A N G L I O M A S TT
palpable tumor mass and caudad to the digastric muscle,
obscuring the view and the site of origin of the tumor (Fig.
12.44). These hyperplastic lymph nodes are initially excised to
demonstrate the anatomy of (he carotid sheath and the location
of the tumor (Fig. 12.45). Following excision of these nodes, the
common carotid artery is identified and a vessel loop is passed
around it for orientation and proximal control of the carotid
artery. The internal jugular vein is identified adjacent to the
common carotid artery and displaced posteriorly by the tumor
(Fig. 12.46).
Meticulous dissection in the carotid sheath now takes place,
carefully freeing up the internal jugular vein which is retracted
posteriorly and the common, external, and internal carotid arteries
anteriorly (Fig. 12.47). The hypoglossal nerve is carefully identified
and retracted cephalad out of harm's way. Further dissection of I he
internal carotid artery requires exposure of the upper part of the neck
medial to the digastric muscle at the jugular foramen and the
fig. 12.43 The incision is deepened through the platysma and skin flaps
are elevated.
Fig. 12.44 Hyperplastic deep jugular lymph nodes are seen overlying
the tumor.
486
carotid canal (Fig. 12.48). At this juncture it becomes obvious
that the tumor arises from the vagus nerve with olher anatomic
structures in (he carotid sheath identified and dissected free from
the tumor. The carotid vessels are retracted anteriorly and the
internal jugular vein is passed behind the tumor medially to
permit mobilization of the distal aspect of the vagus nerve (Fig.
12.49). The tumor is now grasped carefully and its dissection
continues cephalad at the jugular foramen under direct vision and
securing absolute hemostasis every step of the way. By alternate
blunt and sharp dissection, the tumor is delivered in a monobloc
fashion, leaving a large dead space between the carotid vessels
anteromedially, internal jugular vein posterolateral!}-, and the
sympathetic chain posteromedially (Fig. 12.50). After absolute
hemostasis is secured, the wound is irrigated, a Penrose drain is
inserted, and the incision is closed in two layers (Fig. 12.51). the
placement of the incision along an upper neck skin crease provides
an esthctically acceptable scar (Fig. 12.52).
Fig. 12.45 The carotid sheath is opened and the carotid artery and
vagus nerve are isolated.
Fig. 12.46 The internal jugular vein is found displaced posteriorly by the
tumor.
 12 NEUROGENIC TUMORS AND PARAGANGLIOMAS

The tumor grossly measures 6 x 4 cm and is of a fusiform shape

(Fig. 12.53). The cut surface shows a fleshy tumor with numerous
vascular spaces (Fig. 12.54). This appearance is characteristic for
a glomus intravagale tumor. The postoperative sequelae of this
surgical procedure to the patient is due to paralysis of the right
vagus nerve leading to hoarseness of voice and aspiration of fluids
and saliva due to lack of sensation in the supraglottic larynx on
the right-hand side. Most patients will compensate for this deficit
without significant functional debility. The quality of the voice
can be improved using a vocal cord medialization procedure by
laryngoplasty techniques. Such medialization procedures also
restore competency of the larynx and reduce aspiration
pneumonia.

Fig. 12.49 The internal jugular vein is retracted medially to facilitate

dissection of the distal aspect of the vagus nerve.

Fig. 12.47 The tumor is dissected from the carotid arteries and the Fig. 12.50 The surgical field following removal of the tumor.
hypoglossal nerve.

Fig. 12.48 The hypoglossal nerve is demonstrated in this close-up view. Fig. 12.51 A Penrose drain is inserted.

487
NEUROGENIC TUMORS AND PARAGANGLIOMAS rz
SCHWANNOMA OF THE VAGUS NERVE IN THE MID-
CERVICAL REGION

The patient shown in Fig. 12.55 presented with a three-year

Fig. 12.52 The incision is closed in layers.
history of an asymptomatic mass in (he eight side of his neck.
There was demonstrahle growth in its size. Clinical examination
showed that this was a 2.5 cm firm, discrete, mobile mass. No
other abnormalities were detected in a complete head and neck
examination. A CT scan of the neck clearly shows the mass, which
is located deep to the sternomastoid muscle and posterior to the
carotid artery and the internal jugular vein (Fig. 12.56). The mass
is hypodense and does not show contrast enhancement. The
anatomic relations and radiographic characteristics of this mass
lead to the possibility of a neurogenic tumor, arising from either
the vagus nerve or the sympathetic chain. Note only patchy areas
of contrast enhancement in the tumor signifying relatively poor
vascularity. The tumor is well circumscribed and appears to be
encapsulated.

TRIC1

mrlmi miliiii
Fig. 12.53 The gross appearance of t h e tumor. Fig. 1 2 . 5 5 The palpable mass a n d t h e skin incision are o u t l i n e d .

Fig. 12.54 The cut surface of the t u m o r . F i g . 1 2 . 5 6 CT scan of t h e neck shows a mass posterior to the carotid
a r t e r y a n d jugular v e i n .

488

Exposure of this lesion for resection requires a transverse incision
along an upper neck skin crease overlying the mass. The skin
incision is deepened through the platysma to expose the anterior
border of the sternomastoid muscle (Fig. 12.57). The fascia
anterior to the sternomastoid muscle is incised and the sterno-
mastoid muscle is retracted laterally to expose the mass. After
opening the carotid sheath, the tumor is found to be arising from
the vagus nerve (Fig. 12.58). The c o m m o n carotid artery with its
bifurcation as well as the descendens hypoglossi are seen medial
to the tumor. The internal jugular vein is retracted laterally to give
further exposure of the tumor, demonstrating a normal-looking
vagus nerve cephalad and caudad to the tumor.
A schwannoma expands eccentrically splaying the remaining
fibers of the nerve of origin. Thus a normal nerve bundle is usually
seen proximal and distal to the tumor. At the site of the tumor,
however, the splayed out fibers of the nerve are not clearly
identified. Removal of this benign tumor therefore requires very
Fig. 12.57 The skin incision is deepened through the platysma to
expose the anterior border of the sternocleidomastoid muscle.
Fig. 12.58 The tumor arises from the vagus nerve, seen between the
common carotid artery and internal jugular vein.
12 NEUROGENIC TUMORS AND PARAGANGLIOMAS
careful enucleation. If the tumor is small, then the entire tumor
can be removed with an intact capsule carefully preserving the
anatomic continuity of the remaining nerve bundles. The nerve
sheath over the tumor is grasped with a fine-toothed forceps and
a hemoslat is introduced underneath to dissect the pseudocapsule
of the lumor, which contains the stretched normal nerve bundles
(Fig. 12.59).
Separation of the pseudocapsule of the tumor is undertaken
circumferentially, to mobilize the tumor all around, with an intact
capsule, but with preservation of the remaining nerve bundles
(Fig. 12.60). In order to avoid inadvertent rupture of the capsule
of the tumor, meticulous gentle dissection should be undertaken.
After enucleation of the tumor, the pseudocapsule which contains
the remaining nerve bundles, maintaining continuity of the nerve,
remains behind (Fig. 12.61). A Penrose drain placed in the surgical
field is brought out at the end of the incision and the incision is
then closed in two layers.
Fig. 12.59 The nerve sheath is opened to facilitate enucleation of the
tumor.
489
NEUROGENIC TUMORS AND PARAGANGLIOMAS IZ

The specimen shows a well-circumscribed, encapsulated, firm,
solid tumor, which was confirmed as a schwannoma on histologic
examination (Fig. 12.62). Such small schwannomas are well
encapsulated and can easily be enucleated with preservation of
the continuity of the nerve. In spite of preservation of the
anatomic continuity of the nerve, its physiologic function is often
compromised.
MASSIVE SCHWANNOMA OF THE LOWER VAGUS
NERVE IN THE CERVICO-THORACIC REGION
The patient shown in Fig. 12.63 was found to have an asympto-
matic mass in the suprasternal region during a routine physical
examination. On questioning, she admitted to having local
pressure symptoms of tightness in the lower part of the neck. Her
chest x-ray in a postcroanterior view shows a fusiform mass at the
thoracic inlet displacing the trachea to the right (Fig. 12.64). This
tumor mass in the superior mediastinum extends up to the arch of
the aorta.

Fig. 1 2 . 6 3 Patient
w i t h asymptomatic
mass at the
suprasternal n o t c h .

Fig. 12.60 The t u m o r is dissected circumferentially.

Fig. 1 2 . 6 4 Chest x-ray

shows a superior
mediastinal mass w i t h
displacement of the
trachea.

Fig. 12.61 The pseudocapsule of t h e t u m o r , f o l l o w i n g excision of t h e

tumor.

Fig. 1 2 . 6 2 The surgical

specimen.

490

A CT scan through the superior mediastinum demonstrates the
inhomogeneous tumor which is well circumscribed, measuring
7 x 10 cm (Fig. 12.6S). An arch aortogram shows that it is a
relatively avascular tumor (Fig. 12.66). The left common carotid
artery is displaced medially and the left subclavian artery is dis-
placed laterally by the tumor mass which is fusiform in shape. The
diagnosis of a neurogenic tumor is quite obvious with these radio-
graphic appearances.
The surgical approach for excision of this mass requires a trans-
verse incision in the suprasternal region to gain wide exposure of
the thoracic inlet. The incision is taken through a lower neck skin
crease, and is deepened through the platysma to expose the sterno-
mastoid and strap muscles (Fig. 12.67). The upper and lower skin
flaps arc elevated deep to the platysma with an electrocautery
(Fig. 12.68).
Fig. 12.65 CT scan at the level of the thoracic inlet.
Fig. 12.66 An arch
aortogram shows
displacement of the
left common carotid
artery medially and the
left subclavian artery
laterally by a relatively
avascular mass.
-w NEUROGENIC TUMORS AND PARAGANGLIOMAS
The fascia overlying the sternomastoid muscle is incised on the
left-hand side and the muscle is retracted laterally to expose the
carotid sheath. On retraction, the left lobe of the thyroid and
the common carotid artery are exposed (Fig. 12.69). Meticulous
dissection of the contents of the carotid sheath is now undertaken,
carefully isolating and separating the common carotid artery and
the internal jugular vein from the tumor mass (Fig. 12.70).
A close-up view of the surgical field clearly shows the tumor
arising from the vagus nerve near the bifurcation of the common
carotid artery (Fig. 12.71). The tumor is fusiform in shape, but
continuity between the proximal vagus nerve and its splayed
nerve fibers over the tumor could not be demonstrated. The vagus
nerve cephalad to the tumor is thickened and nodular.
Dissection and isolation of the carotid artery is done first,
separating it from the tumor, in the neck and down in the superior
Fig. 12.67 A transverse collar incision is made in the lower part of the
neck.
Fig. 12.68 Skin flaps are elevated to expose the sternomastoid and strap
muscles.
491
NEUROGENIC TUMORS A N D PARAGANGLIOMAS

mediastinum ( F i g . 12.72). A vessel loop is passed a r o u n d the

carotid artery w h i c h permits its retraction medially. M o b i l i z a t i o n
of the cervical c o m p o n e n t of t h e t u m o r n o w begins by dissection
lateral to it and by separating it posteriorly f r o m the sympathetic
chain. Mobilization of the t u m o r on ils posterior aspect is best done
by blunt digital dissection ( F i g . 12.73). Similarly b l u n t dissection
is continued posterior to the t u m o r in the superior m e d i a s t i n u m
permitting its delivery in the neck. The dissection continues in a
circumferential fashion a r o u n d the tumor. The fully mobilized
lumor can now be pulled cephalad f r o m the m e d i a s t i n u m for total
excision ( F i g . 12.74). The lower part of the t u m o r is seen at the
thoracic inlet from the left side, being pulled o u t of t h e media-
stinum ( F i g . 12.75).
Continued dissection and gentle t r a c t i o n delivers the t u m o r in
the neck w i t h a n o r m a l appearing vagus nerve distal to it. The
distal part of the vagus is better demonstrated at the thoracic inlel
from the right-hand side of the patieni ( F i g . 12.76). Because of
Fig. 12.71 Close-up view of the surgical field shows the fusiform tumor
arising from the vagus nerve.

Fig. 12.72 The common carotid artery is dissected free from the tumor
and retracted medially by a vessel loop.

Fig. 12.70 Dissection of carotid sheath with exposure of the tumor. Fig. 12.73 Digital dissection on the posterior aspect of the tumor.

492
 NEUROGENIC TUMORS AND PARAGANGLIOMAS

the massive size of this tumor and its somewhat soft consistency,
it is not practical to enucleate the tumor and preserve any viable
nerve bundles. Therefore, the vagus nerve is transected proximal
and distal to the tumor and the specimen is delivered. The surgical
defect viewed from the left side shows the trachea, thyroid gland
and common carotid artery medial to the space created by removal
of the tumor (Fig. 12.77). Lateral retraction of the sternomastoid
muscle shows a stretched and displaced internal jugular vein. A
suction drain is left in the wound and the incision is closed in two
layers in the usual fashion (Fig. 12.78).
The specimen demonstrates removal of the intact lumor (Fig.
12.79). Note that the tumor measures at least 7 x 1 2 cm. The
bisected tumor is fleshy in consistency and somewhat irregular
with focal areas of hemorrhage (Fig. 12.80). Histologically it was
confirmed to be a benign schwannoma. Because of the sacrifice of
(he vagus nerve, the patient will develop paralysis of the left vocal
cord resulting in hoarseness of voice. If an adequate compensatory
shift from the opposite vocal cord is not observed over the next Fig. 12.76 The lower part of the tumor is pulled out of the mediastinum
with a normal appearing distal part of the vagus nerve seen from the
right-hand side.

Fig. 12.74 Mobilization of the tumor.

Fig. 12.75 The mediastinal component of the tumor is mobilized by Fig. 12.78 Wound closure.
blunt digital dissection and pulled in the neck.

493
NEUROGENIC TUMORS AND PARAGANGLIOMAS
few months, then this patient may need medialization of the
paralyzed vocal cord to improve the quality of her voice. The tech-
nical details of medialization of the paralyzed vocal cord are dis-
cussed in Chapter 8.
Dissection of a lumor extending into the superior mediastinum
through a cervical approach requires special attention to detail as
it relates to other neurovascular and lymphatic structures at the
root of the neck. On the left-hand side of Ihe neck, particular
attention should he paid lo the meticulous identification of all
lymphatic channels and their individual ligation to avoid a chyle
fistula in the neck or a chylothorax. Similarly, excessive dissection
in the prevertehral plane posterior to the carotid sheath can easily
produce trauma to the sympathetic chain and the stellate ganglion,
producing Horner's syndrome. These are avoidable complications.
If adequate mobilization of the lower border of the tumor is not
achieved through the cervical approach, then either a median
sternotomy or a 'clam-shell' sternothoracotomy should be
considered for adequate and safe monobloc resection of the
tumor.
Fig. 12.80 Cut surface of the specimen.
494
SCHWANNOMA OF THE UPPER END OF THE
SYMPATHETIC CHAIN
Schwannomas present in the sympathetic chain with almost equal
frequency as those presenting in the vagus nerve. The patient
shown in Fig. 12.81 has an ill-defined firm to fleshy mass in the
upper part of the neck in the retromandibular area. The mass does
not transmit any pulsations. An V1R1 scan in the axial, coronal and
sagittal planes clearly demonstrates a well-demarcated contrast-
enhancing lesion in the upper part of the neck reaching the
jugular foramen (Figs 12.82-12.84). An MR angiogram clearly
demonstrates an internal carotid artery and the internal jugular
vein displaced by a fusiform smooth mass lying medial and
posterior to these vessels (Fig. 12.85).
Surgical exposure for excision of this lesion requires an incision
in the upper neck skin crease extending from Ihe mastoid process
to the anterior midline in Ihe thyrohyoid region. The skin incision
is deepened through the platysma, and upper and lower skin flaps
are elevated. Structures of the carotid sheath are identified with the
exlcrnal carotid artery isolated with a vessel loop and the internal
carotid artery and internal jugular vein as well as the hypoglossal
nerve demonstrated lying superficial to the tumor (Fig. 12.86).
Meticulous dissection of each of the previously mentioned anatomic
structures now begins, carefully peeling them off the surface of the
tumor. In Fig. 12.87, Ihe completely dissected hypoglossal nerve,
common carotid artery, internal carotid artery, superior laryngeal
nerve, the rami communicantes from the cervical plexus to the
descendens hypoglossi and the vagus nerve are demonstrated. The
carotid artery and vagus nerve are retracted anteriorly and the
tumor is mobilized circumfereiitially up to the jugular foramen
cephalad and to the normal appearing sympathetic chain caudad.
At that point the tumor is removed following division of the distal
sympathetic chain in the lower part of the neck (Fig. 12.88). The
surgical field following removal of the tumor shows repositioned
location of the common, external and internal carotid arteries,
vagus nerve, internal jugular vein, and hypoglossal nerve (Fig.
12.89). The dead space created by removal of the tumor is clearly
seen posteromedial to the neurovascular structures of the carotid
sheath and the hypoglossal nerve.
Fig. 12.81 An ill-defined firm mass is palpated in the retromandibular
region.
 12 NEUROGENIC TUMORS AND PARAGANGLIOMAS

Figs 12.82 and 12.83 Axial and coronal views of the MRI scan. Fig. 12.84 Sagittal view of the MRI scan.

Fig. 12.85 MR angiogram showing displacement of the carotid artery Fig. 12.86 The tumor is seen between the external and internal carotid
and the internal jugular vein. arteries and lying deep to the hypoglossal nerve.

Fig. 12.87 The tumor lies deep to the internal carotid artery and the Fig. 12.88 With the carotid bulb retracted the tumor bed is seen after
hypoglossal and superior laryngeal nerves and medial to the vagus excision of the tumor.
nerve and internal jugular vein.

495
NEUROGENIC TUMORS A N D PARAGANGLIOMAS

The fusiform tumor with ils tail-like component extending
caudad in the sympathetic chain is shown in Fig. 12.90. On its
cut surface, a typical fleshy tumor is identified demonstrating the
characteristic appearance of a neurogenic tumor (Fig. 12.91). The
postoperative sequelae of this operation is the development of
Horner's syndrome.
SCHWANNOMA OF THE LOWER END OF THE
SYMPATHETIC CHAIN
The patient presented in Fig. 12.92 was found to have an
asymptomatic lower cervical mass during a routine physical exam-
ination. A CT scan clearly demonstrates this well-circumscribed
mass lying posterior to the common carotid artery and internal
jugular vein adjacent to the right lobe of the thyroid gland (Fig.
12.93). A lower section of the CT scan demonstrates that the
lumor manifests areas of central necrosis and it is located postero-

Fig. 12.89 The surgical field following removal of the tumor. Fig. 12.92 An asymptomatic lower cervical mass.

Fig. 12.93 CT scan of

the neck shows a mass
posterior to the
carotid sheath.

Fig. 12.90 External appearance of the fusiform tumor.

Fig. 12.94 The mass

shows focal areas of
necrosis.

Fig. 12.91 The cut surface of the tumor.

496

Fig. 12.95 The tumor is located between the common carotid artery
and the internal jugular vein, and posterior to the vagus nerve.
Fig. 12.96 The surgical field following excision of the tumor.
Fig. 12.97 The external surface of the surgical specimen.
1 •} NEUROGENIC TUMORS AND PARAGANGLIOMAS
Fig. 12.98 The cut surface of the surgical specimen.
lateral and inferior to Ihc right thyroid lobe (Fig. 12.94). Surgical
exploration for excision of this lesion requires a supraclavicular
incision along a lower neck skin crease. The skin flaps are elevated
in the usual fashion, carefully identifying the anatomic structures
in the carotid sheath demonstrating the common carotid artery,
the vagus nerve, the internal jugular vein, and the tumor pushing
the vagus nerve anteriorly (Fig. 12.95). By meticulous dissection
of the tumor from its blood supply through small vessels along the
carotid sheath, it is removed in a monobloc fashion (Fig. 12.96).
The surgical defect demonstrates the stump of the sympathetic
chain posterior to the vagus nerve and the carotid artery and
internal jugular vein. The surgical specimen shows a 3 x 3 cm firm,
discrete, fleshy, well-encapsulated tumor which, on the cut sur-
face, demonstrates the fleshy but relatively avascular appearance,
consistent with a schwannoma of the sympathetic chain (Figs
12.97 and 12.98).
SCHWANNOMA OF THE GLOSSOPHARYNGEAL NERVE
The endoscopic photograph of a patient with a mass at the base of
the tongue and the lower pole of the tonsillar fossa on the right-
hand side is shown in Fig. 12.99. This mass was discovered by her
family practitioner due to vague symptoms of sore throat. A
nodular rubbery submucosal mass obscures the view of the
aryepiglottic fold and the vallecula on the right-hand side. Radio-
graphic studies included an MR] scan as well as a CT scan to demon-
strate the anatomic location and extent of the tumor as well as its
relation to the pharynx and other structures in the para-
pharyngeal space. A sagittal, axial and coronal view of the MRI
scan clearly demonstrates the location and disposition of the tumor
which is transversely oriented in the parapharyngeal space presenting
at the base of the tongue (Figs 12.100-12.102). The CT scan
shows that this tumor is well demarcated and encapsulated with
some areas of central necrosis presenting at the base of the tongue
(Fig. 12.103). Since the tumor is submucosal in its presentation
in the pharynx, no attempts are made at biopsy of this lesion
through the intact overlying mucosa. Instead the lesion is excised
in a monobloc fashion through an external cervical approach. An
upper neck skin incision is taken through a skin crease exposing
the sternocleidomastoid muscle, the greater auricular nerve, and
the submandibular salivary gland after elevation of the upper skin
flap (Fig. 12.104). At this juncture, it became evident that the
497
NEUROGENIC TUMORS AND PARAGANGLIOMAS 12

Fig. 12.99 Discrete Fig. 12.100 Sagittal

lobulated submucosal view of the MRI scan.
lesion of the base of
the tongue, obscuring
the view of the larynx
and pharynx.

Fig. 12.101 Axial view Fig. 12.102 Coronal

of the MRI scan. view of the MRI scan.

Fig. 12.103
Encapsulated tumor of
the base of the tongue,
extending into
parapharyngeal space.

Fig. 12.104 Exploration of the upper neck through a transverse incision

showing the sternomastoid muscle and submandibular salivary gland.

498
 12 NEUROGENIC TUMORS AND PARAGANGLIOMAS

tumor was located superomedial to the submandibular salivary fashion (Fig. 12.110). Following excision of the tumor, the space
gland and deep to the mylohyoid muscle. Therefore, the sub- created by its removal is shown in the hyoglossus muscle (Fig.
mandibular salivary gland was excised to gain exposure of the 12.111). Repair of the surgical defect is accomplished by
tumor (Fig. 12.105). A close-up view of the tumor shows the reapproximating the split fibers of (he hyoglossus muscle with
mylohyoid muscle medially, the tendon of the digastric muscle,
and the stylohyoid muscle caudad, the posterior facial vein supero-
laterally with the tumor mass in the center covered by the hyoglossus
muscle (Fig. 12.106). Palpation of the tumor reveals that it is
indeed contained within the musculature and was felt to be relatively
mobile. An incision is made into the hyoglossus muscle lo expose
the pseudocapsule of the tumor (Fig. 12.107). Using alternate
blunt and sharp dissection and securing absolute hemostasis, two
right-angled retractors are inserted around the tumor lo gain
exposure (Fig. 12.108). Further circumferential dissection of (he
tumor through the musculature of the base of the tongue allows
mobilization and delivery of the tumor, which at this point is
appreciated to be a bilobed structure (Fig. 12.109). Meticulous
attention should be paid to avoid entry into the mucosa of the
base of the tongue or the oropharynx during mobilization of the
tumor; otherwise, a mucosal closure would be required to avoid a
resultant fistula. Continued mobilization of the tumor allows its Fig. 12.107 The muscle fibers of hyoglossus are split to expose the
delivery from the deep muscular bed into the wound in an intact pseudocapsule of the tumor.

Fig. 12.105 After excision of the submandibular gland the tumor is seen Fig. 12.108 Further exposure of the tumor.
deep to the mylohyoid muscle.

Fig. 12.106 Close-up view shows the tumor embedded in the Fig. 12.109 Dissection of the tumor continues around its
hyoglossus muscle. pseudocapsule.

499
NEUROGENIC TUMORS AND P A R A G A N G L I O M A S
interrupted chromic catgut sutures. A Penrose drain is inserted
and the skin incision is closed in two layers (Fig. 12.112). Gross
appearance of the tumor shows that it is a 4 x 2.5 cm rubbery
nodular tumor which shows areas of hemorrhage and necrosis on
its cut surface (Figs 12.113 and 12.114). The final pathologic
Fig. 12.111 The surgical field following excision of the tumor.
Fig. 12.113 External appearance of the tumor. Fig. 12.114 The cut surface of the tumor.
500
\ JL
diagnosis of this tumor proved that I his was a schwannoma,
probably arising in the glossopharyngeal nerve. A postoperative
endoscopic view of the larynx and oropharynx shows completely
normal restored anatomy of the supraglottic larynx and the base
of the tongue (Fig. 12.115).
Fig. 12.110 The tumor is delivered intact.
Fig. 12.112 Wound closure is completed with a Penrose drain in place.
Fig. 12.115 Postoperative endoscopic view of
the larynx and pharynx.
MULTIPLE NEUROFIBROMAS OF THE CRANIAL NERVES
Neurofibromas and paragangliomas are often multifocal. They
may present as multiple tumors in the same nerve or as multiple
tumors involving several cranial nerves. In Fig. 12.116, the right-
hand side of the neck of a patient with multiple neurofibromas of
the cranial nerves is exposed. For orientation, the patient's head is
towards the left side of (he picture. Several neurogenic tumors
involving the vagus nerve and the sympathetic chain are seen.
A close-up view of the field shows two large neurofibromas, one
involving the vagus nerve and the other involving the sym-
pathetic chain (Fig. 12.117). In addition, she has other multiple
small tumors involving both the vagus and the sympathetic chain
lower down. Four neurofibromas were enucleated from the vagus,
three from the sympathetic chain, and one from the cervical
plexus. The surgical field following excision of all these tumors is
Fig. 12.116 Multiple neurogenic tumors of the cervical region.
Fig. 12.117 Close-up
view showing two
neurofibromas of the
vagus and one of the
sympathetic chain.
shown in Fig. 12.118, demonstrating preservation of the con-
tinuity of the vagus nerve and the sympathetic chain.
The surgical specimen is shown in Fig. 12.119. The neuro-
fibromas of the vagus are shown in the upper pari of the photo-
graph, the three neurofibromas excised from the sympathetic chain
in the middle, and the one from the cervical plexus is shown at
the bottom. If very meticulous and careful dissection is performed
for enucleation of these tumors, then the patient should not have
vocal cord paralysis or Horner's syndrome. Optical magnification
should be used to spread and separate nerve fibers during
enucleation of the lumor avoiding inadvertent injury or division of
the normal nerve bundles. Thus, small neurogenic lesions such as
these can be safely excised with preservation of the function of the
involved nerve. However, when they are large, the probability of
saving the function of the involved nerve is very small, even
though its anatomic continuity may be preserved.
Fig. 12.118 The
surgical field following
enucleation of multiple
neurofibromas of the
vagus, sympathetic
chain and cervical
plexus.
Fig. 12.119 The surgical specimen of multiple neurofibromas.
501
NEUROGENIC TUMORS AND PARAGANGLIOMAS
DUMB-BELL SCHWANNOMA OF A CERVICAL NERVE
ROOT
The patient shown here presented to her local physician with the
complaint of an ill-defined mass in the posterior triangle of the
neck with tingling and paresthesia in the supraclavicular region. A
CT scan performed through the mid-cervical region demonstralcs
a well-defined dumb-hell shaped tumor extending from the cervical
region through the vertebral foramina into the paraspinous space
(Fig. 12.120). The tumor does not show contrast enhancement
signifying that it is not a highly vascular lesion. Surgical exposure
for removal of this lesion requires a transverse incision in the
posterior triangle of the neck to expose the tumor. The carotid
sheath and sympathetic chain are carefully identified and retracted
anteriorly to expose the tumor which is seen arising from the fourth
cervical root (Fig. 12.121). Circumferential mobilization of
this tumor is accomplished with meticulous hemostasis towards
the medial extension of the tumor into the paraspinal space
Fig. 12.120 CT scan of the neck showing a dumb-bell schwannoma at
the vertebral foramen.
Fig. 12.121 Cervical exploration through a transverse incision shows the
carotid artery and sympathetic chain anterior to the tumor in the root of
C4.
502
(Fig. 12.122). At Ibis juncture, the tumor is found to be entering
the vertebral foramen in which it is lightly packed. With adequate
careful mobilization, the tumor is freed up in the vertebral
foramen up to its medial end where it is transected (Fig. 12.123).
The surgical field following removal of the tumor shows the upper
cervical roots carefully preserved and the contents of the carotid
sheath carefully dissected free from the area of surgical resection
(Fig. 12.124). The surgical specimen clearly shows a dumb-bell
shaped tumor with the intraspinal component of the tumor on
the left-hand side and the extra-spinal component on the right-
hand side on gross examination (Fig. 12.125). The cut surface of
the tumor shows that it is a fleshy, poorly vascularized tumor
showing areas of hemorrhage and necrosis (Fig. 12.126).
Resection of a schwannoma of the cervical root leads to anesthesia
in the distribution of the cutaneous nerves arising from this
cervical root as well as partial paralysis of the diaphragm on that
side. Other than that, the impact of the surgical procedure on
appearance or function is minimal.
Fig. 12.122 The tumor is circumferentially mobilized up to the vertebral
column.
Fig. 12.123 The medial end of the tumor is removed from the vertebral
foramen.
 NEUROGENIC TUMORS AND PARAGANGLIOMAS

MASSIVE DUMB-BELL NEUROFIBROMA OF THE

CERVICAL REGION

A neurofibroma of the cervical plexus may occasionally present as

Fig. 12.124 The surgical field following removal of the tumor.
a bilobulated mass with a palpable tumor in the neck and a dumb-
bell shaped extension through the neural foramen in the spinal
canal. The tumor may thus cause compromise of the function of
the involved nerve and occasionally spinal cord compression. The
patient seen in Fig. 12.127 presented with the history of a mass
in the cervical region of several years' duration. The mass was
asymptomatic but had gradually increased in size to its present
dimension over the years. Because of the obvious deformity, he
decided to have it removed.
On physical examination, the mass is situated deep to the
sternomastoid muscle in the posterior triangle of the right side of
the neck. It is firm in consistency and mobile over the deeper
soft tissues. An MRI in the sagittal plane demonstrates a well-
circumscribed homogeneous round tumor involving the upper
part of the posterior cervical region (Fig. 12.128). The mass
appears to be well encapsulated and relatively avascular. The MRI
in the axial plane shows the mass deep to the sternomastoid
muscle with its extension through the neural foramen in the
spinal canal (Fig. 12.129). The tongue-shaped projection of the
tumor in the spinal canal is very characteristic of a dumb-bell
neurofibroma. A CT scan at the same level confirms continuity
between the cervical component of the tumor and its extension
through the widened vertebral foramen into the spinal canal (Fig.
12.130). A carotid angiography shows that the tumor is hypo-
vascular since no abnormal tumor blush is seen (Fig. 12.131).
The surgical procedure for excision of this tumor requires a
cervical laminectomy for exposure of the intraspinal component

Fig. 12.127 An
asymptomatic
enlarging mass in the
posterior triangle of
net! 61 "71 51 §T" the neck.
mil! : , iiinluiihiiimimimuulinu
Fig. 12.125 The surface appearance of the dumb-bell tumor.

Fig. 12.128 Sagittal

view of the MRI scan.

Fig. 12.126 The cut surface of the specimen.

503
NEUROGENIC TUMORS A N D P A R A G A N G L I O M A S
17
of the tumor. The involved cervical root is transected proximal to
the intraspinal component of the tumor. In addition, a lateral
neck exposure is required for excision of the cervical component
of the tumor simultaneously.
The patient is placed in a left lateral position with the head fixed
with neurosurgical tongs (Fig. 12.132). A T-shaped incision is
marked for exposure of the vertehral column and the posterior
triangle of the neck. The vertical incision extends from the
occiput up to the spinous process of the seventh cervical vertebra.
At approximately the level of the involved vertebral foramen, the
transverse incision begins at right angles to the vertical incision
and overlies the cervical tumor mass extending anteriorly up to
the sternomastoid muscle.
The cervical laminectomy is performed first. The midline
vertical skin incision is deepened through the soft tissues and
musculature which arc retracted laterally to expose the spinous
process and the posterior lamina of the fifth cervical vertebra (Fig.
12.133). The paraspinal muscles are retracted laterally with self-
Fig. 12.129 Axial view
of the MRI scan shows
paraspinal extension of
the dumb-bell tumor.
Fig. 12.130 CT scan
shows the dumb-bell
tumor with paraspinal
extension through the
vertebral foramen.
504
retaining retractors to maintain exposure of the vertebral column.
The spinous process is excised with a rongeur, providing further
exposure of the lamina (Fig. 12.134).
It is important to emphasize that the laminectomy component
of the surgical procedure is best performed by a neurosurgeon.
Excision of the posterior lamina of the vertebra exposes the dura
of the cervical spinal cord (Fig. 12.135). The dura is incised in the
midline to expose the cervical roots and the spinal cord (Fig.
12.136). Meticulous mobilization of the intraspinal component
of the tumor is now undertaken with neurosurgical technique.
The cervical root proximal to the tumor is transected and the
tumor is mobilized for delivery (Fig. 12.137).
Cervical exploration begins with extension of the transverse
part of the incision anteriorly up to the sternocleidomastoid muscle.
The superior and inferior skin flaps are elevated. The accessory
nerve is dissected first and carefully preserved (Fig. 12.138). It is
identified as it emerges from the posterior surface of the sterno-
mastoid muscle and traced until its entry into the trapezius
Fig. 12.131 Arch
aortogram showing
medial displacement of
the right common
carotid artery by this
relatively avascular
tumor.
Fig. 12.132 The patient is positioned on the operating table and the
incisions are outlined.
 12 NEUROGENIC TUMORS AND PARAGANGLIOMAS

muscle. It is then retracted anteriorly to facilitate mobilization of

the neurofibroma of the cervical plexus in the posterior triangle
(Fig. 12.139).
Careful dissection of the soft tissues and fibroconnectivc tissue
around the tumor is undertaken to mobilize and remove it in a
monobloc fashion. Thus cervical and intraspinal components of
the dumb-bell shaped tumor arc removed intact. Complete hemo-
stasis must be achieved prior to wound closure. The dura of the
spinal cord is closed with 4-0 Neurolon sutures. The paraspinal
muscles are reapproximated in the midline. The neck wound is

Fig. 12.133 The

spinous process and
posterior lamina of the
fifth cervical vertebra
Fig. 12.135 The posterior lamina is excised and the dura is exposed.
are exposed.

Fig. 12.136 The dura is opened and the spinal cord and cervical roots
are exposed.
Fig. 12.134 The
spinous process is
excised with a rongeur.

Fig. 12.137 The cervical root is transected intraspinally.

505
NEUROGENIC TUMORS AND PARAGANGLIOMAS 12
irrigated with Bacitracin solution. Suction drains are placed in the
wound and the skin incision is closed in layers (Fig. 12.140).
Since only a posterior laminectomy is performed, rigid tixalion of
the spine is not required.
The specimen vividly demonstrates the dumb-hell shaped tumor
with its intraspinal extension and a second neurofibroma removed
from the lower branches of the cervical plexus (Fig. 12.141). The
bisected specimen shows that the tumor is not homogeneous on
its cut surface. Areas of focal hemorrhage arc present throughout
the tumor (Fig. 12.142).

Fig. 12.138 The

accessory nerve is
Fig. 12.140 The incision is closed in layers after suction drains are put in
dissected as it emerges
place.
from the
sternomastoid muscle
and is preserved.

Fig. 12.141 The external appearance of the dumb-bell neurofibroma

and a second, small incidental neurofibroma of the cervical plexus.
Fig. 12.139 The
accessory nerve is
retracted anteriorly
and dissection of the
tumor in the posterior
triangle of the neck
continues.

Fig. 12.142 The cut surface of the specimen shows areas of focal
hemorrhage.

506
 NEUROGENIC TUMORS AND PARAGANGLIOMAS

In the immediate postoperative period, the cervical spine is Fig. 12.145 Outline of
stabilized with a halo splint. The patient is advised to wear a the T-shaped incision.
cervical collar for three months. The postoperative photograph of
the patient shows a well-healed scar (Fig. 12.143).

Fig. 12.143
Postoperative view of
the patient.

Fig. 12.146 Exposure

1 of the CI root through
* the cervical
laminectomy.

Another patient with a dumb-hell neurofibroma involving the

craniocervical junction is shown here. An MR I scan at the level of
CI and C2 in an axial plane shows a multilohulalcd tumor with a
paraspinal extension of the tumor ahutting the spinal cord (Fig.
12.144). The surgical approach for this patient is essentially
similar, with a vertical incision from the inion to C4, and a
transverse incision from the mastoid process up to the midline
(Fig. 12.145). A cervical laminectomy is performed with resection
of the left half of the posterior arch of CI to gain exposure of the
root of CI in the region of its dural sleeve (Fig. 12.146). In this
patient a normal nerve root could be demonstrated medial to the
tumor and therefore the dura did not require opening. The nerve

Fig. 12.144 Axial view

of the MRI scan
showing a dumb-bell
tumor of the CI nerve
root abutting the
spinal cord.

Fig. 12.147 The posterior compartment musculature is divided to

mobilize the cervical component of the tumor.

507
NEUROGENIC TUMORS AND PARAGANGLIOMAS
root is transected and its stump is ligated with a silk suture to
prevent CSF leakage. Mobilization of the cervical component of
the tumor begins next, through the posterior compartment
musculature (Fig. 12.147). Meticulous dissection of the entire
tumor permits a monobloc resection of the multilobulated tumor
(Fig. 12.148). The surgical specimen shows complete excision
Of the multilobulated dumb-bell neurofibroma with paraspinal
extension at the level of CI (Fig. 12.149). Excision of a dumb-bell
neurofibroma of the cervical region requires close cooperation
between the neurosurgeon and the head and neck surgeon for safe
monobloc removal of the tumor and smooth postoperative
recovery.
Fig. 1 2 . 1 4 8 The t u m o r is delivered by c i r c u m f e r e n t i a l dissection f r o m
the surrounding soft tissue.
508
T2
RESECTION OF SCHWANNOMA OF THE SYMPATHETIC
CHAIN IN THE SUPERIOR MEDIASTINUM
The patient whose contrast-enhanced CT scan of the superior
mediastinum is shown in Fig. 12.150 has a soft tissue mass lateral
to the esophagus and the tracheoesophageal plane in the superior
mediastinum lying posterior to the carotid sheath. The pre-
operative clinical diagnosis of a neurogenic tumor was based on
the radiologic findings and the homogeneous appearance of the
tumor on the CT scan.
The surgical procedure is undertaken with a transverse incision
along a lower neck skin crease exposing the lower end of the left
sternocleidomastoid muscle and the strap muscles in the central
compartment of the neck (Fig. 12.LSI). The lower skin flap is
elevated to expose the suprasternal notch as low as possible. The
strap muscles on the left-hand side are excised to gain satisfactory
exposure of the lower pole of the left thyroid lobe, the tracheo-
esophageal groove and the carotid sheath (Fig. 12.152). The
Fig. 1 2 . 1 5 0 Contrast-enhanced axial CT scan of the superior
mediastinum.
Fig. 1 2 . 1 5 1 The l o w e r e n d of t h e left s t e r n o c l e i d o m a s t o i d muscle and
the strap muscles are exposed t h r o u g h a transverse incision along a
l o w e r neck skin crease.
 NEUROGENIC TUMORS AND PARAGANGLIOMAS

sternocleidomastoid muscle is retracted laterally to expose the
underlying carotid sheath. By alternate blunt and sharp dis-
section, the common carotid artery and the internal jugular vein
are identified (Fig. 12.153). The internal jugular vein, the carotid
sheath, and the vagus nerve arc retracted laterally. The recurrent
laryngeal nerve on the left-hand side has been identified and the
trachea and the esophagus arc retracted medially to expose the
prevertebral plane. The upper end of the tumor is just visible at
this point.
At this juncture, the tumor was found to be arising from the
lower end of the sympathetic chain. The sympathetic chain is
divided and by alternate blunt and sharp dissection, the tumor is
circumferentially mobilized (Fig. 12.154). A liberal use of nemo-
d i p s facilitates hemostasis and permits expedient mobilization of
the tumor, lixtrcmc care must be exercised to avoid injury to the
confluence of the internal jugular and subclavian veins as well as
the innominate vein in the superior mediastinum. Similarly, the
lower end of the left recurrent laryngeal nerve ascending from

Fig. 12.152 Excision of the strap muscles on the left-hand side provides
satisfactory exposure of the lower pole of the left thyroid lobe, the
tracheoesophageal groove and the carotid sheath.

Fig. 12.153 The common carotid artery and the internal jugular vein are
identified.

509
NEUROGENIC TUMORS A N D P A R A G A N G L I O M A S

behind the arch of the aorta should be protected. Willi con-

tinuous traction on the specimen and gradual circumferential dis-
section, the tumor is delivered intact in a monobloc fashion. The
surgical defect following removal of the tumor shows the origin of
the left common carotid artery from the arch of the aorta as well
as the origin of (he innominate artery (Fig. 12.155). Suction
drains are employed for drainage and the wound is closed in
layers. The surgical specimen shown in Fig. 12.156 demonstrates
a homogeneous tumor mass with multiple lobulations seen on the
cut surface. Histological analysis confirmed the diagnosis of a
benign schwannoma.

Fig. 12.155 The surgical defect.

Fig. 12.154 The tumor is circumferentially mobilized by alternate blunt Fig. 12.156 The surgical specimen.
and sharp dissection.

510