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DOI 10.1007/s00405-012-2084-6
Received: 19 March 2012 / Accepted: 6 June 2012 / Published online: 27 June 2012
Springer-Verlag 2012
Introduction
Vascular tumors of head and neck region comprise a heterogeneous group of lesions that have a very different
histology, clinical courses, and treatment options. They are
rarely encountered in clinical practice, but a solid understanding of their diagnosis and management is very
important [1].
Paragangliomas of the head and neck are hypervascular
benign neoplasms which are derived from neural crest cells
of the autonomic nervous system. They account for 0.6 %
of all neoplasms in the head and neck region. The most
common locations are carotid space and jugular fossa.
Recently, a gene focus in families with inherited paragangliomas or glomus tumors has been identified. Despite
paragangliomas are classified as benign tumors WHO
grade I, distant metastases have been published. On the
other hand, 10 % of glomus tumors may be multicentric in
origin [2]. Combinations of head and neck paragangliomas
include vagalcarotid body paragangliomas; carotid body
glomus jugulare tumors are also reported [2].
They are usually seen in four primary locations; the
jugular bulb, middle ear cavity, vagus nerve, and carotid
body. Tumors in the jugular bulb region called glomus
jugulare and arise in adventitia of the dome of the jugular
bulb. Tumors in middle ear cavity called glomus tympanicum and it arise from the glomus bodies that run with the
tympanic branch of the glossopharyngeal nerve. They are
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MDCT examination
Performed using 16-detector MDCT scanner (Bright
Speed 16; GE Medical Systems). The entire neck and
skull base was scanned starting from the superior
border of the petrous bone till the thoracic inlet in less
\10 s in a craniocaudal direction. Contrast material
was injected with a power injector (Medrad, Stellant)
through an 18- or 20-gauge catheter into the antecubital vein. The injection rate was 4 mL/sec. A total of
90 mL of nonionic water-soluble contrast material was
used. The acquisition parameters were 120 kVp,
440 mAs, a helical pitch of 1.375:1, 0.5-second scan
time, 16 9 1.25 mm detector configuration, 1.25 mm
helical slice thickness, and 0.625 mm reconstruction
interval with a large FOV. CTA was done for all
patients.
Inclusion criteria
Image display
Exclusion criteria
Patients
Patients
Patients
stroke.
Patients
Clinical presentation
Number
Palpable mass
10
33.3
13.3
Deafness
123
Hoarseness of voice
13.3
Deviated tongue
13.3
Vertigo
13.3
Bruit
13.3
Pulsatile tinnitus
Bulbar palsy
2
2
6.6
6.6
Horner syndrome
3.3
1047
volume rendering image and MIP reconstruction (maximum intensity projection) were applied for evaluation of
tumor extension, vascularity, and detection of relation to
the surrounding vessels.
2.
left jugular fossa, extending to the middle ear and naso-pharynx (thin
white arrows) note the extensive vascular signal voids (thick white
arrow). Histopathological examination revealed jugulo- tympanicum
glomus
MRI examination
All the patients were instructed to remove all metallic
articles, e.g. hair pins and coins. They were asked
about the presence of metallic prosthesis, coils or
implants or any other articles interfering with MRI
examination.
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1048
Imaging protocol
Final diagnosis in all lesions was made histopathologically using either surgery or biopsy from the lesion.
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Image Analysis
Images were read by two radiologists in consensus with
11 years out of training, both of them were unware of
patients clinical data.
Our study included 30 patients presenting with variable
clinical presentations: the most common was palpable non
painful neck swelling that was seen in 10/30 patients
1049
c Sagittal T1WI showed the mass extending along the jugular vein till
the jugular fossa; no intra cranial extension. d DSA showed marked
tumor plush displacing the carotid artery anteriorly. Histopathological
examination revealed Glomus vegale
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1050
RK et al. and others who stated that 15 % of paragangliomas arise extra-adrenal and 80 % of them are either
carotid body or glomus tumors [4, 912]. The fact that 3/30
(10 %) of our patients showed bilateral paraganglioma
agreed with the findings of Arnlod et al. and others who
reported that 10 % of paragangliomas are multicentric in
origin [2, 5, 13, 14].
In our results MRI has been accurate in evaluating paragangliomas. Its high soft tissue resolution and multiplaner capabilities detect lesion, determine its extension, and
evaluate its relation to surrounding vessels. It demonstrated
paragangliomas as well circumscribed, expanding, intensely enhanced masses with marked internal vascularity that
appeared as multiple signal voids. This agreed with the
report by Arnold et al. and other several reports [2, 1520].
MRA was good determinant for tumor resectability
according to Glasscock-Jackson classification. It clarifies
the relation to carotid arteries or jugular veins, splaying of
carotids and their antro-medial displacement. Also glomus
Discussion
Vascular lesions of head and neck include many different
types: benign neoplasms, malignant neoplasms, and non
neoplastic growths. They are challenging for surgical
resection. Determination of the degree of vascularization of
these lesions and their extension is very important. Imaging
studies depict the location and extent of tumor involvement, help determine the surgical approach, and help predict operative morbidity and mortality [5, 79].
In our study, paragangliomas including glomus and
carotid body tumors were the commonest detectable
lesions. This was concordant with the findings of Whalen
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T1WI showed the mass and the displaced vessels. c axial T2WI
showed the mass and the displaced vessels. Histopathological
examination revealed schwanoma
Number
Percent
(%)
Glomus tumors
12
40
20
13.3
Schwanoma
10
Hemangiopercytoma
6.6
Angiofibroma
6.6
Neurofibroma
3.3
30
100
Total
with the reports of Rao et al., Sahdev et al. and van den
Berg [5, 15, 16].
The treatment options for paragangliomas depend on
many parameters. MRA was good determinant for tumor
resectibility. In our study we found that MRA added to the
merits of MRI and helped in confirmation of the diagnosis
via providing us more details about the relation of paraganglioma to carotid arteries or jugular veins. Splaying of
carotids and their antro-medial displacement could be
accurately recorded that helps in grading of tumors. Glomus mimicking lesions such as high-riding jugular bulb,
dehiscent jugular bulb, and jugular vein thrombosis could
be confidently excluded after revision of MRA that agreed
with several recent reports by Rao et al., Sahdev et al. and
van den Berg [5, 15, 16].
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MDCT
MRI
Glomus tumor
10 (33.3 %)
Pathology
9 (30 %)
12 (40 %)
5 (16.6 %)
5 (16.6 %)
6 (20 %)
Lymphadenopathy
4 (13.3 %)
4 (13.3 %)
4 (13.3 %)
Schwanoma
5 (16.6 %)
6 (20 %)
3 (10 %)
Neurofibroma
0 (0 %)
2 (6.6 %)
1 (3.3 %)
Mucocele
1 (3.3 %)
0 (0 %)
0 (0 %)
Inverted papilloma
2 (6.6 %)
1 (3.3 %)
0 (0 %)
Angiofibroma
2 (6.6 %)
1 (3.3 %)
2 (6.6 %)
Hemangiopericytoma
0 (0 %)
0 (0 %)
2 (6.6 %)
Non specific
Total
1 (3.3 %)
2 (6.6 %)
0 (0 %)
30 (100 %)
30 (100 %)
30 (100 %)
Table 4 The sensitivity and specificity as well as the PPV and NPV
of MDCT and MRI in comparison to histopathological results
Sensitivity
Specificity
PPV
NPV
MDCT
83.33 %
100 %
100 %
80 %
MRI
77.7 %
100 %
100 %
75 %
Glomus
tumor
Carotid
body
tumor
LN
Schwannoma
Neurofibroma
Mucocele
Inverted
papilloma
Angiofibroma
HPC
NS
Total
Glomus tumor
10
Lymphadenopathy
Schwanoma
Neurofibroma
Mucocele
Inverted papilloma
Angiofibroma
Hemangiopericytoma
Non specific
Total
30
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Schwannomas, hypervascular lymphadenopathy, and nodular fasciitis showed no uniform enhancement as that of
paraganglioma and also no abnormal feeding vessels to the
tumors. This is concordant with the report of Dhiman et al.
[25]. Because of this we believe that MPR and MIP are
merits of MDCT that rise the efficacy of MDCT in diagnosis of paragangliomas and differentiate it from its
mimicking conditions.
Limitations of our study were, first, our study included
small number of patients, so further studies with larger
number of patients are needed; second, readers interpret
results in consensus and so inter-observer reliability could
not be calculated.
Conclusion
MDCT with its new utilities has near degree of accuracy in
detection and localization of paragangliomas as the same
that of MRI. Both techniques have moderate agreement
between them in differentiating paragangliomas from other
mimicking vascular lesions. So, it is better to use both of
them as complementary techniques for accurate diagnosis
and grading of paraganglioma.
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