Jugular paraganglioma

Case contributed by Prashant Mudgal
Diagnosis certain

Presentation

Middle-aged lady presented with sensorineural hearing loss, tinnitus, and vertigo.

Patient Data

Age: 45 years
Gender: Female
ct

An intensely enhancing hyperdense lesion is seen involving the left jugular foramen, measuring approximately 3.5 x 3.5 x 3.5 cm. The lesion is causing destruction and widening of the jugular foramen.

Medially, the lesion has intracranial extradural extension in the posterior fossa, indenting the cerebellar cortex.

Laterally, the lesion extends into the middle ear cavity (epi, meso, and hypotympanum) and external auditory canal. There is erosion of the long process of the incus, the inferior semicircular canal, the basal turn of the cochlea, and the lateral wall of the tympanic segment of the facial nerve.

The lesion infiltrates the jugular bulb, and inferiorly extending into the proximal jugular vein. Posterior inferiorly, there is the destruction of the basiocciput and lateral aspect of the foramen magnum.   

Case Discussion

The most common tumor to develop in the jugular foramen is a jugular paraganglioma. Paragangliomas in the skull base are ubiquitous in their distribution and arise from paraganglia or glomus cells situated at the following sites:

(1) in the adventitia of the jugular bulb beneath the floor of the middle ear 

(2) in the bony walls of the tympanic canals related to the tympanic branches of the IX and X nerves

(3) in the bone of the promontory, close to the mucosal lining of the middle ear.

Imaging studies are necessary to depict the location and extent of tumor involvement, to help determine the surgical approach, and to predict operative morbidity and mortality 1.

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