26-year-old woman, who came to our hospital complaining of pulsatile tinnitus and headache.

 

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Doctor's Information

Name : Morteza
Family : Sanei Taheri
Affiliation :Radiology Department,Shohada Tajrish Hospital,SBMU
Academic Degree : Associate Professor of Radiology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : Mohsen Zakavati Avval

 

Case Section

Head & Neck Imaging

 

Patient's Information

Gender : Female
Age : 26

 

Clinical Summary

We present the case of a 26-year-old woman, who came to our hospital complaining of pulsatile tinnitus and headache.

 

Imaging Findings

A high-resolution CT scan of the skull base, showing a soft-tissue mass in the right jugular foramen. There is expansion and moth-eaten erosion of the jugular foramen. SAH specially in sylvian cistern is noted. An image obtained with DSA of the right common carotid revealing an intense tumor blush in the right jugular foramen. The tumor is seen to be supplied mainly by the ECA branches.

 

Differential Diagnosis

Jugular schwannoma(sharply demarcated smooth bony margins,no internal flow voids, not very vascular on angiography, no salt and pepper appearance, Indium-111 labelled octreotide negative). bony metastases(hypervascular tumours may be very similar in appearances). meningioma. normal anatomical variation(asymmetry of jugular foramen size, high riding or dehiscent jugular bulb). jugular bulb thrombosis

 

Final Diagnosis

Glomus jugulare tumour

 

Discussion (Related Text)

A glomus jugulare tumour is a paraganglioma of the head and neck that is confined to the jugular fossa. While it is a rare tumour, it is the most common of the jugular fossa tumours.[1] Epidemiology: The relative prevalence of glomus jugulare with respect to other head and neck paraganglioma of the head and neck varies from publication to publication and depending on definition of the terms jugulare, tympanicum and jugulotympanicum. Most agree however that they are more common than glomus vagale[2]. These tumours are seen in adults, typically between 40 and 60 years of age, with a moderate female predilection [2].
Clinical presentation:
Presentation depends on the degree of middle ear involvement. When significant involvement is present then pulsatile tinnitus and hearing loss.
Additionally a number of patterns of cranial nerve palsies have been described due to involvement of the nerves at the jugular foramen. These include [2,3]:
• Vernet syndrome (motor paralysis of cranial nerves IX, X, and XI)
• Collet-Sicard syndrome (Vernet syndrome with additional involvement of cranial nerve XII) (46), and
• Horner syndrome
Pathology:
For a general discussion on the pathology of these tumours please refer to the generic article pertaining to paragangliomas. Glomus jugulare tumours are defined according to location (i.e. origin at the jugular foramen) rather than anatomic origin and may arise from Jacobson's nerve, Arnold's nerve of the jugular bulb [2].
Radiographic features:
Growth of these tumour is in a number of directions. Typically they extend into the mastoid air-cells and middle ear and Eustachian tube.
CT:
CT is most useful at assessing the bony margins of the tumour, which are typically irregularly eroded with a moth-eaten pattern. Eventually as the tumour enlarges thejugular spine is eroded and the mass extends into the middle ear, as well as inferiorly into the infratemporal fossa. CT is excellent at assessing the integrity of the ossicles and bony labyrinth [2].
MRI:
• T1 - low signal [2]
• T2 - high signal
• T1 C+ (Gd) - marked intense enhancement [2-4]
Salt and pepper appearance is seen on both T1 and T2 weighted sequences; the salt representing blood products from haemorrhage or slow flow and the pepper representing flow voids due to high vascularity. It should be noted that this appearance is sometimes encountered in other lesions (e.g. hypervascular metastases) and is not typically seen in smaller glomus tumours [2].
DSA – angiography:
Angiography demonstrates an intense tumour blush, with the most common feeding vessel being the ascending pharyngeal [2]. Early draining veins are also noted due to intra-tumoural shunting [3]. Angiography also has a role to play in preoperative embolisation, which is typically carried out 1-2 days prior to surgery, however care must be taken to fully evaluate feeding vessels. Familiarity with vascular anatomy of the region is essential if complications are to be avoided [2].

 

References


1. Dr Yuranga Weerakkody and Dr Jeremy Jones et al., Glomus jugulare tumour. Radiopedia.org
2. Rao AB, Koeller KK, Adair CF. From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 19 (6): 1605-32. Radiographics (full text) [pubmed citation]
3. Head and neck imaging. Ed. by Peter M. Som, Hugh D. Curtin. St Louis (Mo.) : Mosby-Year Book, 2003. ISBN:0323009425 (find it at amazon.com)
4. Vogl T, Brüning R, Schedel H et-al. Paragangliomas of the jugular bulb and carotid body: MR imaging with short sequences and Gd-DTPA enhancement. AJR Am J Roentgenol. 1989;153 (3): 583-7. AJR Am J Roentgenol (abstract)[pubmed citation]

 

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