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Paraganglioma, type glomus jugulare

CLINICAL HISTORY:
This 29-year old female complains of one year of pain and limited abduction of the left shoulder. Recently she noted deviation of the tongue to the left, and hoarseness with decreased volume of the voice. Now she hears a bruit in the left ear, synchronous with the pulse.

NEUROLOGICAL EXAMINATION shows atrophy of the left sternocleidomastoid and trapezius muscles, with reduced abduction of the shoulder (distribution of the accessory nerve). The left side of the tongue shows marked atrophy with deviation to the left and fasciculations (distribution of the hypoglossal nerve). The uvula is deviated to the right (distribution of the glossopharyngeal nerve). There is paralysis of the left vocal cord.

CT:
a) NON-CONTRAST CT (IMAGE 1) with bone windows shows expansion and thinning of the posterior bony edges of the left jugular foramen, with presence of scattered calcifications. The mass protrudes into the posterior fossa (arrowheads).

b) CONTRAST-ENHANCED CT (IMAGE 2) shows intense enhancement of the lesion (arrowheads).

MRI:
a) TRANSAXIAL T1 WEIGHTED SPIN-ECHO (600/14/1) SEQUENCE (IMAGES 3 - 4 AND 5): The soft-tissue mass (arrowheads) expands the jugular foramen. There is slight compression of the left cerebellar hemisphere. Some intratumoral signal dropout points to hypervascularity. The mass shows caudal extension below the bony skull base to the parapharyngeal soft tissues.

c) TRANSAXIAL (IMAGES 6 - 7 AND 8) AND CORONAL (IMAGES 9 AND 10) GADOLINIUM-ENHANCED T1-WEIGHTED SPIN-ECHO SEQUENCES: There is intense enhancement of the lesion (arrowheads). Intratumoral signal voids (arrow) reflect the hypervascularity of the tumor. Caudal extension of the tumor to the parapharyngeal space is noted (arrowheads images 9 and 10). There is tumor extension into the internal auditory canal.

ANGIOGRAPHY: Selective injection of the ascending pharyngeal artery in anteroposterior (IMAGE 11) and lateral (IMAGE 12) projections, demonstrates marked hypervascularity of the tumor.

RADIOLOGICAL DIAGNOSIS is glomus jugulare tumor based on the location of the tumor, its extension, the erosion of the bone and the hypervascularity.

THERAPY consisted of successive embolization of the ascending pharyngeal artery and other supplying vessels from the external carotid artery.

ANATOMOPATHOLOGICAL EXAMINATION (IMAGE 13).

Search also:
Paraganglioma (chemodectoma)

 

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Fig. 1

Paraganglioma, type glomus jugulare, Fig. 1
Paraganglioma, type glomus jugulare, Fig. 2
Paraganglioma, type glomus jugulare, Fig. 3
Paraganglioma, type glomus jugulare, Fig. 4
Paraganglioma, type glomus jugulare, Fig. 5
Paraganglioma, type glomus jugulare, Fig. 6
Paraganglioma, type glomus jugulare, Fig. 7
Paraganglioma, type glomus jugulare, Fig. 8
Paraganglioma, type glomus jugulare, Fig. 9
Paraganglioma, type glomus jugulare, Fig. 10
Paraganglioma, type glomus jugulare, Fig. 11
Paraganglioma, type glomus jugulare, Fig. 12
Paraganglioma, type glomus jugulare, Fig. 13