Carotid body tumor
- Integral Diagnostics, Shareholder (ongoing)
- Micro-X Ltd, Shareholder (ongoing)
Updates to Article Attributes
Carotid body tumour, also known as a chemodectoma or carotid body paraganglioma, is a highly vascular glomus tumour that arises from the paraganglion cells of the carotid body. It is located at the carotid bifurcation with characteristic splaying of the ICA and ECA.
Epidemiology
Typically, carotid body tumours are diagnosed in the 4th to 5th decades and have a female predilection like the other paragangliomas of the head and neck 1,3. They are the most common type of paraganglioma of the head and neck (account for 60-70%). In approximately 10% of cases, they are bilateral 1,3.
A small number are familial (7-10%), and in such cases, they are frequently multicentric (35-50%) 1,3. When familial, they are usually autosomal dominant in inheritance, and associated with 3:
Clinical presentation
Clinical presentation is usually with a slow-growing rounded neck mass. It is usually located anterior to the sternocleidomastoid near the angle of the mandible at the level of the hyoid bone. Characteristically, the tumour can be moved side to side but not up or down, due to its location within the carotid sheath 1.
Cranial nerves that travel in the carotid sheath (glossopharyngeal, vagus, accessory and hypoglossal nerves) may be involved. Associated symptoms relate to their dysfunction 2.
These tumours may synthesise and secrete catecholamines, although this is less common than with adrenal paragangliomas (phaeochromocytomas) 1.
Pathology
The paraganglioma article includes a general discussion of the pathology of these tumours.
Radiographic features
Carotid body tumours are located at the carotid bifurcation with characteristic splaying of the ICA and ECA, described as the lyre sign. In all modalities, the dense vascularity of these tumours is manifested as prominent contrast enhancement.
CT
Contrast-enhanced CT is excellent at depicting these lesions. Typical appearances are:
soft tissue density on non-contrast CT (similar to muscle)
bright and rapid (faster than schwannoma) enhancement
splaying of the ICA and ECA
-
circumferential angle of contact of tumour with ICA could also be categorised under the Shamblin group system as
group I: <180 degrees of encasement
group II: 180-270 degrees of encasement
group III: >270 degrees of encasement
helps in deciding the risk of ICA adventitial involvement and possible need of ICA resection followed by grafting required in group III cases 4
MRI
-
T1
iso to hypointense compared to muscle
salt and pepper appearance when larger, representing a combination of punctate regions of haemorrhage or slow flow (salt) and flow voids (pepper) 3
intense enhancement following gadolinium
-
T2
hyperintense compared to muscle
salt and pepper appearance
DSA/angiographyAngiongraphy (DSA)
The splaying of the carotid vessels (lyre sign) is again identified with an intense blush in the tumour and 'early vein' seen due to arteriovenous shunting 3.
The ascending pharyngeal artery is the main contributing supply.
ScintigraphyNuclear medicine
Although not specific, scintigraphy shows uptake with metaiodobenzylguanidine (MIBG) and octreoscan scintigraphy and can be useful for assessing multiple lesions.
Treatment and prognosis
Surgical excision is the treatment of choice. The larger the tumour the higher the risk of operative complications 2. In patients for whom the risk of complications precludes surgery, radiotherapy may be considered 1,2.
Malignant transformation is encountered in 2-36% of cases with metastases most commonly to bone, lung and liver and regional lymph nodes 3.
Differential diagnosis
General imaging differential considerations include:
vagal schwannoma: spreads the ICA and internal jugular vein; displaces the ICA anteromedially 5
glomus vagale tumour: same pathology but located more rostrally
- +<li><p><a href="/articles/multiple-endocrine-neoplasia-syndromes">multiple endocrine neoplasia</a>: <a href="/articles/multiple-endocrine-neoplasia-type-iia-1">MEN IIa</a> and <a href="/articles/multiple-endocrine-neoplasia-type-iib">MEN IIb</a></p></li>
-<a href="/articles/multiple-endocrine-neoplasia-syndromes">multiple endocrine neoplasia</a>: <a href="/articles/multiple-endocrine-neoplasia-type-iia-1">MEN IIa</a> and <a href="/articles/multiple-endocrine-neoplasia-type-iib">MEN IIb</a>-</li>-<li>-<a href="/articles/phakomatoses">phakomatoses</a><ul>-<li><a href="/articles/tuberous-sclerosis">tuberous sclerosis complex (TS)</a></li>-<li><a href="/articles/neurofibromatosis-type-1">neurofibromatosis type 1 (NF1)</a></li>-<li><a href="/articles/vhl">von Hippel-Lindau disease (vHL)</a></li>- +<p><a href="/articles/phakomatoses">phakomatoses</a></p>
- +<ul>
- +<li><p><a href="/articles/tuberous-sclerosis">tuberous sclerosis complex (TS)</a></p></li>
- +<li><p><a href="/articles/neurofibromatosis-type-1">neurofibromatosis type 1 (NF1)</a></p></li>
- +<li><p><a href="/articles/vhl">von Hippel-Lindau disease (vHL)</a></p></li>
-<li><a href="/articles/carney-triad">Carney triad</a></li>- +<li><p><a href="/articles/carney-triad">Carney triad</a></p></li>
-<li>soft tissue density on non-contrast CT (similar to muscle)</li>-<li>bright and rapid (faster than schwannoma) enhancement</li>-<li>splaying of the ICA and ECA</li>-<li>circumferential angle of contact of tumour with ICA could also be categorised under the Shamblin group system as<ul>-<li>-<strong>group I</strong>: <180 degrees of encasement</li>-<li>-<strong>group II</strong>: 180-270 degrees of encasement</li>-<li>-<strong>group III</strong>: >270 degrees of encasement</li>-<li>helps in deciding the risk of ICA adventitial involvement and possible need of ICA resection followed by grafting required in group III cases <sup>4</sup>-</li>- +<li><p>soft tissue density on non-contrast CT (similar to muscle)</p></li>
- +<li><p>bright and rapid (faster than schwannoma) enhancement</p></li>
- +<li><p>splaying of the ICA and ECA</p></li>
- +<li>
- +<p>circumferential angle of contact of tumour with ICA could also be categorised under the Shamblin group system as</p>
- +<ul>
- +<li><p><strong>group I</strong>: <180 degrees of encasement</p></li>
- +<li><p><strong>group II</strong>: 180-270 degrees of encasement</p></li>
- +<li><p><strong>group III</strong>: >270 degrees of encasement</p></li>
- +<li><p>helps in deciding the risk of ICA adventitial involvement and possible need of ICA resection followed by grafting required in group III cases <sup>4</sup></p></li>
-<strong>T1</strong><ul>-<li>iso to hypointense compared to muscle</li>-<li>-<a href="/articles/salt-and-pepper-sign-disambiguation">salt and pepper</a> appearance when larger, representing a combination of punctate regions of haemorrhage or slow flow (salt) and flow voids (pepper) <sup>3</sup>-</li>-<li>intense enhancement following gadolinium</li>- +<p><strong>T1</strong></p>
- +<ul>
- +<li><p>iso to hypointense compared to muscle</p></li>
- +<li><p><a href="/articles/salt-and-pepper-sign-disambiguation">salt and pepper</a> appearance when larger, representing a combination of punctate regions of haemorrhage or slow flow (salt) and flow voids (pepper) <sup>3</sup></p></li>
- +<li><p>intense enhancement following gadolinium</p></li>
-<strong>T2</strong><ul>-<li>hyperintense compared to muscle</li>-<li>salt and pepper appearance</li>- +<p><strong>T2</strong></p>
- +<ul>
- +<li><p>hyperintense compared to muscle</p></li>
- +<li><p>salt and pepper appearance</p></li>
-</ul><h5>DSA/angiography</h5><p>The splaying of the carotid vessels (<a href="/articles/lyre-sign-carotid-artery">lyre sign</a>) is again identified with an intense blush in the tumour and 'early vein' seen due to arteriovenous shunting <sup>3</sup>.</p><p>The ascending pharyngeal artery is the main contributing supply.</p><h5>Scintigraphy</h5><p>Although not specific, shows uptake with metaiodobenzylguanidine (<a href="/articles/mibg">MIBG</a>) and <a href="/articles/octreotide-scintigraphy">octreoscan</a> scintigraphy and can be useful for assessing multiple lesions.</p><h4>Treatment and prognosis</h4><p>Surgical excision is the treatment of choice. The larger the tumour the higher the risk of operative complications <sup>2</sup>. In patients for whom the risk of complications precludes surgery, radiotherapy may be considered <sup>1,2</sup>.</p><p>Malignant transformation is encountered in 2-36% of cases with metastases most commonly to bone, lung and liver and regional lymph nodes <sup>3</sup>.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>-<li>-<a href="/articles/vagal-schwannoma">vagal schwannoma</a>: spreads the ICA and internal jugular vein; displaces the ICA anteromedially <sup>5</sup>-</li>-<li><a href="/articles/vagal-neurofibroma">vagal neurofibroma</a></li>-<li><a href="/articles/high-attenuation-lymphadenopathy-1">hypervascular lymphadenopathy</a></li>-<li>-<a href="/articles/glomus-vagale-paraganglioma">glomus vagale tumour</a>: same pathology but located more rostrally</li>-<li><a href="/articles/carotid-bulb-ectasia">carotid bulb ectasia</a></li>- +</ul><h5>Angiongraphy (DSA)</h5><p>The splaying of the carotid vessels (<a href="/articles/lyre-sign-carotid-artery">lyre sign</a>) is again identified with an intense blush in the tumour and 'early vein' seen due to arteriovenous shunting <sup>3</sup>.</p><p>The ascending pharyngeal artery is the main contributing supply.</p><h5>Nuclear medicine</h5><p>Although not specific, scintigraphy shows uptake with metaiodobenzylguanidine (<a href="/articles/mibg">MIBG</a>) and <a href="/articles/octreotide-scintigraphy">octreoscan</a> scintigraphy and can be useful for assessing multiple lesions.</p><h4>Treatment and prognosis</h4><p>Surgical excision is the treatment of choice. The larger the tumour the higher the risk of operative complications <sup>2</sup>. In patients for whom the risk of complications precludes surgery, radiotherapy may be considered <sup>1,2</sup>.</p><p>Malignant transformation is encountered in 2-36% of cases with metastases most commonly to bone, lung and liver and regional lymph nodes <sup>3</sup>.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>
- +<li><p><a href="/articles/vagal-schwannoma">vagal schwannoma</a>: spreads the ICA and internal jugular vein; displaces the ICA anteromedially <sup>5</sup></p></li>
- +<li><p><a href="/articles/vagal-neurofibroma">vagal neurofibroma</a></p></li>
- +<li><p><a href="/articles/high-attenuation-lymphadenopathy-1">hypervascular lymphadenopathy</a></p></li>
- +<li><p><a href="/articles/glomus-vagale-paraganglioma">glomus vagale tumour</a>: same pathology but located more rostrally</p></li>
- +<li><p><a href="/articles/carotid-bulb-ectasia">carotid bulb ectasia</a></p></li>