Glomus jugulare paraganglioma

Dr Dan J Bell and Dr Jeremy Jones et al.

Glomus jugulare paraganglioma 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.

The relative prevalence of glomus jugulare with respect to other head and neck paraganglioma 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 3

These tumours are seen in adults, typically between 40 and 60 years of age, with a moderate female predilection 3.

Presentation depends on the degree of middle ear involvement. When significant involvement is present then the lesion may cause 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 3-4:

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 nerve, Arnold nerve or the jugular bulb 3.

Tumours may be bilateral, and other tumours such as carotid body tumours may coexist. Up to 10% of the patients may have multiple lesions. Tumours are locally infiltrating, and may rarely metastasize 4.

Growth of these tumour is in a number of directions. Typically they extend into the mastoid air-cells, middle ear, and Eustachian tube

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 the jugular 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 3. Also erosion of the caroticojugular spine between the carotid canal and jugular fossa may be present (Phelp sign).

  • T1: low signal 3
  • 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 3.

Angiography demonstrates an intense tumour blush, with the most common feeding vessel being the ascending pharyngeal 3. Early draining veins are also noted due to intra-tumoural shunting 4.

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 3.

Indium-111 labelled octreotide accumulates in these tumours due to the presence of receptors for somatostatin, best visualised with SPECT, but requires the tumour to be greater than 1.5 cm in diameter.

Surgery is the treatment of choice and if complete resection is achieved a cure can be expected. Complications are however not uncommon due to the large number of sensitive structures in the region and include:

  • cranial nerve deficits
  • CSF/endolymphatic leak

Although most cervical paragangliomas (e.g. carotid body tumours) are considered relatively radioresistant, base of skull paragangliomas are radiosensitive, and thus large inoperable tumours or tumours in elderly and frail patients are often treated with radiotherapy.

Recurrence and local invasion is common, occurring in 40-50% of cases 3. Malignant transformation is less common, seen in 2-13% of cases 3.

For a full list of differentials see the article on jugular fossa masses. General imaging differential considerations include:

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Article information

rID: 5941
Section: Pathology
Synonyms or Alternate Spellings:
  • Glomus jugulare
  • Glomus jugulare tumours
  • Glomus jugulare tumor
  • Glomus jugulare tumors
  • Glomus jugulare paragangliomas

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Cases and figures

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    Figure 1: distribution of paragangliomas
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    Case 1
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    Case 2
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    Case 3
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    Case 4: with carotid body tumour
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