Surgery of Carotid Body Tumors: Clinical Experience with 10

Cases

ArminGeiger, M.D.
Rasjid Soeparwata, M.D.
Johannes Schmidt, M.D.
and
Hans H. Scheld, M.D., F.I.C.A.

MUENSTER, GERMANY

Abstract
Within population of 1150 vascular patients only a small percentage of the
a
correct preoperative diagnosis of carotid body tumors (synonym chemodectoma)
was found (2 patients). Before being treated by a specially trained vascular

surgery team 8 patients did undergo inadequate operations. These were per-
formed with high risk of local complications, even though by simple use of
intravenous angiography (digital subtraction technique) the typical intercarotid
widening with rich vascular filling could have led to the correct diagnosis.
Malignancy was detected in 1 case (pulmonary metastasis). In 2 cases concom-
itant tumors of the jugular vein were seen.
Since the blood supply from carotid body tumors is derived nearly exclusively
from the external carotid artery, interruption of blood flow in this vessel simplifies
the surgical approach substantially.
Ligature of the external carotid artery (5 patients) and interposition of venous
grafts (10 patients) were employed as surgical therapies.
In 1 case extirpation of a larger tumor (18 x 11 x 9 cm) extending from the
skull base and almost reaching the left clavicular bone was successfully carried
out. At present there is no other satisfactory therapeutic approach to benign
carotid body tumors available. For this reason surgical intervention, even in far
advanced stages, is always justified.

Introduction

The carotid body belongs to the so-called nonchromaffin paraganglia. Physiologically it acts as a
vascular chemoreceptor stimulated by changes in pH, oxygen, and carbon dioxide pressure of the arterial
From the Department of Thoracic, Cardiac, and Vascular Surgery, Westphalian Wilhelms University, Muenster, Germany
Presented at the 32nd Annual Meeting, International College of Angiology, Toronto, Canada, June, 1990

315
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blood. ~ Tumors arising from the carotid body were rare conditions. Since 1743, when Albrecht von Haller
described the discovery of this tissue, there have hardly been 1200 known cases all over the world.2’j. In
cases without surgical treatment the mortality rate reaches 30%. A general account of the relevant

literature shows the incidence of malignancy in a wide range from 1.5% to 50% of cases. 1-12 Only in those
cases in which metastasis to lymph nodes or other organs are detected should the diagnosis of malignancy
2,4-S.11I
b .
be given.
Etiology
The etiology of carotid body tumors is not exactly known. The familial tendency of these tumors
suggests a genetic predisposition. ~°’~3 A very interesting hypothesis suggests chronic lack of oxygen leads
to a high frequency of carotid body tumors in persons born and living at high altitudes.

Symptoms
It is a remarkable that many carotid
phenomenon body tumors are at first not correctly diagnosed.
Nevertheless, it is very easy to get the right diagnosisby simply thinking of it. Usually the patients are
asymptomatic. The extremely slow, nearly unrecognized increase of the tumor in the lateral aspect of the
neck is a most characteristic feature. On the other hand the tumors, above all the large ones, may cause
symptoms like dysphagia, disorders of the language or breathing., and continuous pain of the cervical
vertebral column. Dependent on local alteration or infiltration the cranial nerves could be injured. Thus
paresis of the facial nerve, glossopharyngeal nerve, vagal nerve, accessory nerve, and hypoglossal nerve
could occur. Caused by injury of the cervical sympathetic nerve, Horner’s triad could appear. Transitory
ischemic attacks, even apoplexia, could occur as a consequence of carotid artery compression . 6,9

Diagnosis
The most important clinical examination methods for investigation of tumors of the carotid bifurcation
were described by Fontaine and Kocher ~2: the sign of mobility (Fontaine), the sign of pulsation (Kocher
I), and the sign of localization (Kocher II).
If the tumor is mobile in a horizontal level, but there is no mobility in a vertical level, the sign of
Fontaine is defined as positive. These findings are dependent on the fixation of the tumor in the carotid
bifurcation.
Fixating the tumor between three fingers shows pulsation of the internal carotid artery under the thumb,
of the external carotid artery under the third finger, and missing pulsation at the upper pole of the tumor
beyond the index finger. These findings describe the first sign of Kocher.
The second sign of Kocher is defined as positive if simultaneous external and transoral palpation allows
the localization of a tumor at the tonsillar fossa.
Furthermore, there are several clinical signs indicating a chemodectoma; eg, auscultation of a buzzing
murmur marks an ateriovenous shunt, which could be found in any case of large carotid body tumors.

Ultrasound investigation, a well-known screening method, is able to show the close relationship of the
2
tumor with the carotid arteries.2
In our institution angiography with digital subtraction technique is carried out in any case of suspected
carotid body tumor. Angiography shows the typical intercarotid widening of the bifurcation and images
the size of the tumor by intensive opacification (Fig l.). About twenty years ago the need for carotid
angiography in context with differential diagnosis of chemodectomas was disputed because of dangerous,
 317
even life-threatening, side effects caused by the investigation.b Today the possibility of intravenous
application of the contrast medium and employment of digital subtraction technique allows a maximum

Fig. l. Angiogram shows typical intercarotid widening of the bifurcation.

of safety for patients and gives a satisfying qualitative diagnosis.

Finally the findings of axial computed tomography are essential for estimating the correlation of the
tumor with its adjacent structures (Fig. 2). The determination of operability is dependent on the rate of
infiltration by the tumor in these structures.
Patients and Methods

During the last ten years we have seen 10 patients with 10 chemodectomas. In the period from April,
1989, to April, 1990, alone 6 patients with carotid body tumors underwent surgical treatment in our
institution.
Today we are not able to state whether this cluster of chemodectomas is accidental or represents a
general increase in this clinical entity. In our patients we did not find the slightest indication of etiologic
factors; 8 of our 10 patients had had previous operations elsewhere, either to establish a diagnosis or in
an attempt to remove the tumor.
In all cases the tumor was far progressed, belonging to type III of Linder’s classification. Subadventi-
tial resection in the &dquo;white line,&dquo; as described by Sir Gordon-Taylor, was not possible in all cases
because of the size of the tumor or consecutive adhesions caused by the previous operations. Therefore,
we performed en-bloc resection of the carotid bifurcation in all patients and replaced the arterial defects
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Fig. 2. Computed tomography shows correlation of tumor with its adjacent structures.

by autologeous graft interposition. In the first series of patients we replaced the internal and the
venous

external carotid artery by saphenous vein grafts in 5 cases. In the later series we ligated the external carotid
artery and reconstructed only the internal branch of the carotid artery. Postoperative differences between
the two series were not seen.

Registration of evoked potentials and continuous electroencephalography separated for each hemi-
sphere were performed as intraoperative monitoring. The use of an intraluminal shunt was not necessary
in any of the cases.
In I patient there was a large tumor measuring 18 x 11 x 9 cm extending from the skull base and almost
reachingthe left clavicular bone (Fig. 3 A-C). We have not read anything about a tumor of similar
dimension in all the literature. The anamnesis of this patient went back to about thirty years ago.

Results
All patients left the hospital two to three weeks after operation. Nerve palsy was seen only in those
cases that had had previous operations. Two of these patients had lesions of the recurrent laryngeal nerve
as a result of the first operation. In detail we recognized lesions of the hypoglossal nerve, facial nerve and

vagal nerve. In I case there was a Horner’s triad. As a rule nerve palsy was only temporary and occurred
in those cases with extremely large tumors.
In 2 cases concomitant tumors of the jugular vein were detected. Half a year after the operation we
discovered lung metastasis in a routine chest x-ray in 1 patient. ’

Bleeding complications or infections were not seen in any of the cases.

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3. A.Large tumor measuring 18 x

Fig.
11 9 cm extending from the skull base and
x

almost reaching the left clavicular bone. B.

Intraoperative view to the vein graft.
C. Postoperative view.
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One week after the operation the function of the vein grafts was checked by intravenous digital
subtraction angiography.
Dipyridamole and acetylsalicylic acid were administered as medication.
Discussion

Since Rieger did the first resection of a carotid body tumor in 1862, surgery of these tumors has

changed in a fundamental manner. In large tumors and in complex situations it has been standard for a
long time to ligate the common carotid artery. Hemiplegia and a high mortality rate (20-40%) were the
consequences4’9 Already by the end of the first decade of our century en-bloc resection and replacement
of arterial flow by end-by-end anastomosis of the common and internal carotid arteries was being
performed successfully. Moreover, the possibility of interposition of an autologous vein graft was later
described as an alternative. 16
Conclusions

In cases of well-founded suspicion of carotid body tumors we consider treatment at a special vascular
surgical unit very important. In our opinion it is a dangerous mistake to try to get a histologic diagnosis
by means of biopsy or exploratory exposure. This may cause uncontrolled bleeding and danger for the
13,17
patient’s life. The possibility of malignancy and the uncertain natural history of carotid body tumors
&dquo;-19
seem to be an indication for operation in all cases.

Armin Geiger, M.D.

Westfälische Wilhelms
Universität Münster
Albert-Schweitzer-Str. 33
D-4400 Miinster, Germany

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