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Case Reports
. 2007 Jun;27(3):139-43.

Vagal paragangliomas: two case reports

C Caldarelli  1 , C Iacconi, C Della Giovampaola, P Iacconi, F Beatrice
Affiliations
Case Reports

Vagal paragangliomas: two case reports

C Caldarelli et al. Acta Otorhinolaryngol Ital. 2007 Jun.

Abstract
in English, Italian

Two uncommon cases of paragangliomas arising from the vagus nerve are described. The first patient underwent surgery for suspected carotid body tumour. In the second patient, computed tomography scan and digital angiography allowed a correct pre-operative diagnosis to be made. These cases confirm the prevalence of vagal paragangliomas in female sex and middle age, and the possibility of multiple similar tumours in the same patient. Histological benign features, absence of neurological symptoms, of local invasion or intracranial extension confirm the frequent benign behaviour of these neoplasms. Lack of catecholamine secretion confirms the low incidence of functioning tumours. Contrast computed tomography and digital angiography still remain the gold standard reliable instruments for diagnosis despite the success of magnetic resonance imaging, magnetic resonance angiography and octreotide scintigraphy to detect head and neck paragangliomas. A transcervical approach, without mandibulotomy, is suitable too for large tumours but complete removal, with sparing of involved segments of the vagus nerve, is rarely possible. Post-operative neurological morbidity is still an unsolved issue and, therefore, rehabilitation of deglutition and phonation is an integral part of management.

In questo articolo riportiamo due rari casi di paraganglioma vagale. La prima paziente è stata operata con il sospetto di tumore del glomo carotideo mentre, nel secondo caso, l’angio TC e l’angiografia digitale hanno consentito una corretta diagnosi pre-operatoria. L’istologia favorevole, l’assenza di sintomi neurologici, la mancanza di invasività locale e di estensione intracranica hanno caratterizzato l’andamento benigno dei tumori. L’assenza di catecolamine e derivati nel sangue e nelle urine ha confermato la bassa incidenza di forme secernenti. Queste osservazioni confermano la predilezione del paraganglioma vagale per il sesso femminile e l’età media, e la possibilità di paragangliomi multipli nello stesso paziente. Angio TC ed angiografia digitale risultano indagini diagnostiche ancora valide nonostante i successi di RM, angio RM e scintigrafia con Octreotide. L’approccio cervicale senza mandibulotomia risulta adeguato alla rimozione di tumori anche di grandi dimensioni, mentre l’asportazione completa della lesione con conservazione del tratto di vago interessato è raramente possibile. Il problema della morbilità neurologica post-operatoria è ancora irrisolto così che la riabilitazione dei meccanismi di deglutizione e fonazione costituisce parte integrante del trattamento.

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Figures

Fig. 1
Fig. 1
Contrast-enhanced CT scan of neck showing, on left side, a mass 6.4 x 2 cm. On right side: another mass 31.5 x 15.5 mm at level of carotid bifurcation reliable to the carotid body tumour removed in another hospital.
Fig. 2
Fig. 2
Carotid angiography confirming, on left side, a highly vascular lesion displacing anteriorly internal and external carotid artery with feeders from external carotid artery. A hypertrofied ascending pharyngeal artery (a). On right side: a vascular lesion in the enlarged carotid bifurcation angle (b).
Fig. 2
Fig. 2
Carotid angiography confirming, on left side, a highly vascular lesion displacing anteriorly internal and external carotid artery with feeders from external carotid artery. A hypertrofied ascending pharyngeal artery (a). On right side: a vascular lesion in the enlarged carotid bifurcation angle (b).
Fig. 3
Fig. 3
Contrast-enhanced CT scan of neck showing left mass 31.4 x 15.6 mm compressing internal jugular vein and displacing anteriorly external and internal carotid arteries.
Fig. 3
Fig. 3
Contrast-enhanced CT scan of neck showing left mass 31.4 x 15.6 mm compressing internal jugular vein and displacing anteriorly external and internal carotid arteries.
Fig. 4
Fig. 4
Digital angiography showing a tumour blush lying outside and above the carotid bifurcation (a) with feeders from external carotid artery particularly from a hypertrofied ascending pharyngeal artery (b).
Fig. 4
Fig. 4
Digital angiography showing a tumour blush lying outside and above the carotid bifurcation (a) with feeders from external carotid artery particularly from a hypertrofied ascending pharyngeal artery (b).
Fig. 5
Fig. 5
Operative view showing vagal tumour involving vagus nerve (a) and removal with segment of vagus nerve involved (b).
Fig. 5
Fig. 5
Operative view showing vagal tumour involving vagus nerve (a) and removal with segment of vagus nerve involved (b).
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