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Bilateral carotid body tumors and right jugular paraganglioma

Case contributed by Vinay Shah
Diagnosis almost certain

Presentation

Swelling over right side neck region.

Patient Data

Age: 35 years
Gender: Female

 Well defined intensely enhancing lesion is seen in the right side of neck near bifurcation of right CCA, predominantly involving the carotid triangle. The lesion is displacing the right internal and external carotid arteries and compressing the internal jugular vein. The lesion causes splaying of right internal and external carotid arteries and extends upto the carotid bifurcation. Another Similar signal enhancing lesion noted in the left carotid triangle near left CCA bifurcation.  

Another illdefined hyperintense signal lesion is seen  in the right jugular foramina, which inferiorly extends upto the upper neck and superiorly it is reaching upto the level of the middle ear. The lesion shows heterogeneous postcontrast enhancement. The lesion is causing enlargement of the right jugular foramina. The right IJV near jugular bulb is seen in close relation to the lesion.    

On MR angiography the lesion causes splaying of right internal and external carotid arteries and extends upto the carotid bifurcation. Few vascular channels are seen in the right jugular foramen lesions on MR non-contrast 3D TOF angiography.

On venography the right internal jugular vein is compressed by the above mentioned lesion. Left internal jugular vein appears normal.  

Case Discussion

 The likely differential diagnosis are 

A) Bilateral carotid body paraganglioma and right jugular paraganglioma (more likely)

B) Multiple nerve sheath tumor (schwannoma / neurofibroma) 

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