Paraganglioma (chemodectoma)

Origin

Paragangliomas arise from nonchromaffin paraganglion cells derived from the neural crest.

Macroscopic appearance and general characteristics

For the purpose of this work only the glomus jugulare tumor is important. It is a highly vascular tumor originating from the adventitia of the jugular bulb that erodes and expands the jugular foramen. There is a variable extension into the infralabyrinthine compartment of the temporal bone. The vertical portion of the carotid canal can be significantly eroded. Erosion of the hypoglossal canal is possible. Intracranial extension is first extra-axial, but they can invade the cerebellum. Extension up to the cerebellopontine angle is common.

Microscopic appearance (image 2)

The lesion is composed principally of pale "chief" cells arranged in nests and lobules surrounded by flattened sustentacular cells.

Imaging features (see case reports)

- On CT the paragangliomas appear as intensely enhancing soft tissue masses, located within and expanding the bony margins of the jugular foramen. The bony erosion of the remainder of the skull base is very accurately depicted with CT.

- On MRI, the lesion has mixed signal intensity. Within the tumor areas of flow-void indicate high velocity flow. Enhancement after administration of Gd-chelates is intense. It can extend up to the cerebellopontine angle and into the infratemporal fossa (image 3).

Case report

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Paraganglioma, glomus jugulare type

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