glomus jugulare paraganglioma

Glomus jugulare paraganglioma is a paraganglioma of the head and neck that is confined to the jugular fossa. While it is a rare tumor, it is the most common of the jugular fossa tumors.

Epidemiology

The relative prevalence of glomus jugulare with respect to other head and neck paraganglioma varies from publication to publication and depending on the definition of the terms jugulare, tympanicum, and jugulotympanicum. Most agree however that they are more common than glomus vagale .

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

Clinical presentation

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 the involvement of the nerves at the jugular foramen. These include :

• Vernet syndrome (motor paralysis of cranial nerves IX, X, and XI)
• Collet-Sicard syndrome (Vernet syndrome with additional involvement of cranial nerve XII)
• Horner syndrome

Pathology

For a general discussion on the pathology of these tumors please refer to the generic article pertaining to paragangliomas.

Glomus jugulare tumors are defined according to the location (i.e. origin at the jugular foramen) rather than the anatomic origin and may arise from Jacobson nerve, Arnold nerve, or the jugular bulb .

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

Radiographic features

Growth of these tumors is in a number of directions, typically extending into the mastoid air-cells, middle ear, and Eustachian tube.

CT

CT is most useful at assessing the bony margins of the tumor, which are typically irregularly eroded with a moth-eaten pattern. Eventually, as the tumor 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 . Also, erosion of the caroticojugular spine between the carotid canal and jugular fossa may be present (Phelp sign).

Angiography (DSA)

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

Angiography also has a role to play in preoperative embolization, 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 .

MRI

• T1: low signal
• T2: high signal
• T1 C+ (Gd): marked intense enhancement

Salt and pepper appearance is seen on both T1 and T2 weighted sequences; the salt representing blood products from hemorrhage 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 tumors .

Nuclear medicine

Indium-111 labeled octreotide accumulates in these tumors due to the presence of receptors for somatostatin, best visualized with SPECT, but requires the tumor to be greater than 1.5 cm in diameter.

Treatment and prognosis

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

• cranial nerve deficits
• CSF/endolymphatic leak

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

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

Differential diagnosis

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

• jugular schwannoma
  • sharply demarcated smooth bony margins
  • no internal flow voids
  • not very vascular on angiography
  • no salt and pepper appearance
  • indium-111 labeled octreotide negative
• bony metastases
  • hypervascular tumors may be very similar in appearances (e.g. renal cell carcinoma, thyroid cancer)
• meningioma
• normal anatomical variation
  • asymmetry of jugular foraminal size
  • high riding or dehiscent jugular bulb
• jugular bulb thrombosis
• endolymphatic sac tumor
  • centered in the vestibular aqueduct rather than the jugular bulb 

Siehe auch:

• jugular schwannoma
• glomus jugulare: imaging and pre-operative embolization
• jugular foramen meningioma
• dehiszenter Bulbus jugularis
• Paragangliome von Kopf und Hals
• Pfeffer und Salz Erscheinungsbild

und weiter:

• Bulbus-venae-jugularis-Hochstand
• Paragangliom
• Gradenigo-Syndrom
• Vernet syndrome
• neuroradiologisches Curriculum
• Somatostatin-Rezeptor-Szintigrafie
• jugular foramen schwannoma
• jugular spine
• collet-Sicard syndrome
• non chromaffin paraganglion cells
• jugular diverticulum
• Heiserkeit
• Paragangliom des Nervus vagus
• glomus jugulotympanicum tumour
• Pyramidenspitzen-Osteomyelitis