every extra‘pear’ counts a 3year retrospective study on imaging in extra-adrenal paragangliomas...
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Every extra‘pear’ counts
A 3year Retrospective Study on Imaging in Extra-Adrenal Paragangliomas with A Select Display of Exquisite Cases
Abstract Id no 1207
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Aim
To review the location, distribution and imaging features of extra-adrenal paragangliomas
presenting to a quarternary care hospital in southern India for the past 3 years and
illustrate the imaging features of selective unusual presentations.
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Methodology and Materials
A 3 years retrospective study from December 2011 to November 2014
Data collection methods: Institutional PACS server was searched for the key words ‘paraganglioma,’ ‘glomus’ and ‘carotid body’
Data collection sources:• Institutional PACS• Surgical records• Medical records • Institutional laboratory results
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Results
• Total no. of lesions = 67• Male : Female = 1.48 : 1• Average age at diagnosis = 44.6years[Range from 14 to 76 years]• Total lesions biopsied = 38 (57%)• Biopsy proven paragangliomas = 35[Malignant paraganglioma = 1]
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Radiological operationsColour Doppler
1%
CT52%MRI
36%
MRCT9%
DSA1%
Diagnostic modality
PVA; 3Gel-foam, 3
Embolisation
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Distribution of lesions in the body
Carotid Body Tumours44%
Glomus Jugulare27%
Para-aortic6%
Spinal6%
Glomus Jugulo-
tympan-icum5%
Glomus Tympan-
icum5%
Urinary Bladder3%
Glomus Vagale3%
Carotid sheath2%
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Size distribution of lesions (cm)
< 1
1 to 2
2 to 3
3 to 4
4 to 5
5 to 6
6 to 7
7 to 8
8 to 9
> 9
3
7
7
12
9
14
5
6
1
1
Average size of lesions = 4.4cmRange = 0.3cm to 9.7cm
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Carotid body; 18Other head &
neck; 6
Spine; 3
Para-aortic; 1.2
Treatment
excision Stereotactic Radiosurgery
Radiotherapy Embolisation0
5
10
15
20
25
30
35 32
8
3
6
Excised lesions
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Features of common conditions
Carotid body
Other head & neck
Total0%
10%20%30%40%50%60%70%80%90%
100%
Gender Distribution
Male Female
Carotid body
Other head and neck
Total 05
101520253035404550
Average size of lesions
mm
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Carotid body lesions vs head & neck lesions
Male Multiple Biopsy Excision Embolisation RT SRS0%
10%
20%
30%
40%
50%
60%
70%
80%
Carotid body tumours Other Head & Neck
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Para-falcine paraganglioma• MRI of a 41year old man suspected to
have a glioma shows a heterogeneously hyperintense lesion to the right of the posterior part of the falx with significant peri-lesional oedema.
• T1 plain and post contrast images show moderate enhancement of the solid components.
• MRI diagnosis was meningioma.
Patient underwent excision and biopsy was reported as paraganglioma. On follow up, he had his latest MRI after 9years in December 2013 that shows no disease.
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Carotid body chemodectoma11year old with right neck swelling and pain.
• Colour Doppler neck showed a well-defined heterogeneous lesion at right CCA bifurcation with intralesional arterial flow.
• Diagnosis of carotid body tumour was suggested and no other imaging was done. Patient underwent excision, biopsy reported as paraganglioma.
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Tracheal paraganglioma22years old man presented with
haemoptysis, dyspnoea and voice change.
• He presented 2 years later with breathing difficulty and was haemoptysis and was found to have post-op recurrence. He underwent excision with crico-tracheal resection anastomosis.
• Neck radiograph, lateral view shows a soft-tissue density within the tracheal lumen compromising the airway.
• CECT axial sections showed a well-defined, intensely enhancing, polypoidal lesion with infiltration of the posterior tracheal wall. Excision and biopsy showed paraganglioma.
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Atrio-ventricular groove paraganglioma
• 36years old man who was incidentally detected to have a mediastinal mass. CECT axial sections show a brightly enhancing mass in the left AV groove. Peripheral curvilinear calcifiations are seen in the lateral and posterior aspect. Eccentric non-enhancing areas suggest necrosis. Patient underwent CT guided FNAC suggestive of paraganglioma.
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Para-vertebral Paraganglioma51 year old diabetic and hypertensive with back pain.
CECT arterial phase axial section show an intensely enhancing lobulated soft tissue density lesion in the left para-aotic region
Patient underwent CT guided biopsy and pre-operative embolisation with gelfoam Plain CT prone axial section with needle
Post operative Ga-68 DOTATATE PET CT showed a DOTATATE avid residual functioning tumour and is
planned for Lutetium-177 DOTATATE therapy.
Patient undewent partial excision as it was adherent to the aortic wall. Surgical
pathology specimen showed paraganglioma.
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Metastatic Paraganglioma of the Urinary Bladder
32years old man presented with fatigue sweating and palpitations.
• Biopsy from the bladder mass was reported as paraganglioma. MIBG scan was negative. Hence, he was advised DOTA scan followed by Lutetium therapy but was discharged at request.
• CECT venous phase axial image shows a large, lobulated moderately enhancing mass involving the base, posterior and right walls of the urinary
bladder.
• Coronal section shows central calcification and a mixed lytic-sclerotic metastasis in the left acetabulum. Multiple other lung, nodal and skeletal metastases were present.
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Literature review - location
• Extra-adrenal paragangliomas can be found in every site in which healthy paraganglia are known to occur
• Majority are seen in relatively predictable regions of the body:
1. Carotid body,2. Jugular foramen, 3. Middle ear, 4. Aorticopulmonary region, 5. Posterior mediastinum, and 6. Abdominal paraaortic region including Zuckerkandl’s body.
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Literature review - imaging
Nearly identical imaging features:• CT: homogeneous or heterogeneous
hyperenhancing soft-tissue mass • MRI: multiple areas of signal void interspersed
with hyperintense foci (salt-and-pepper appearance) within tumour mass
• Angiography: intense tumour blush with enlarged feeding arteries
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Conclusions of our study
• CT and MRI are the mainstay modalities of diagnosis• Over 80% are in the head and neck and over half of
these are carotid body tumours• Majority of the lesions are between 3 and 6cm• Carotid body tumours are larger at presentation and
more often excised than other head & neck paragangliomas, while the latter are more often treated with embolisation, radiotherapy and stereotactic radiosurgery.
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Radiological imaging is always indispensible and often sufficient for the diagnosis of extra-adrenal paragangliomas and an attractive option for treatment of the same
• References:• Extraadrenal Paragangliomas of the
Body: Imaging Features AJR 2006; 187:492–504
• Radiopaedia
All images are from our own institutional PACS