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Paragangliomas in an endemic area: from genetics to morphofunctional imaging. A pictorial essay

I paragangliomi in una zona endemica: dalla genetica all’imaging morfo-funzionale. Rassegna iconografica

  • Head and Neck Radiology / Radiologia del Capo E del Collo
  • Published:
La radiologia medica Aims and scope Submit manuscript

Abstract

The aim of this pictorial essay is to illustrate the morphological [computed tomography (CT) and magnetic resonance imaging (MRI)], vascular (angiography) and functional (nuclear medicine) features of paragangliomas, uncommon lesions of the head and neck region and even more of the thorax, abdomen and pelvis, arising in an endemic area in northern Italy. These hypervascular, well-circumscribed masses usually have innocuous clinical manifestations as slowly enlarging soft-tissue lesions; however, more rarely, they can cause cranialnerve palsy, particularly lesions arising near the skull base, or symptoms related to their secreting activity. Most paragangliomas are benign and their prognosis is directly related to the location of the tumour: those arising at the carotid body have the best outcome, whereas those located at the skull base have a less favourable prognosis. Angiography is required preoperatively in larger paragangliomas for surgical planning (vascular mapping) and, rarely, for preoperative embolisation. Morphological and functional imaging is also mandatory for surgical and/or radiometabolic treatment planning and follow-up.

Riassunto

Lo scopo di questa rassegna iconografica è quello di illustrare l’aspetto morfologico alla tomografia computerizzata (TC) e alla risonanza magnetica (RM), vascolare (angiografico) e funzionale (medicina nucleare) dei paragangliomi, rare lesioni della regione della testa e del collo e ancor più, del torace, dell’addome e della pelvi, insorte in un’area endemica del nord Italia. Queste lesioni, ipervascolarizzate e ben delimitate, si manifestano solitamente in maniera innocua come masse dei tessuti molli a lenta crescita mentre più raramente causano paralisi dei nervi cranici, in particolare per le lesioni che si sviluppano a livello della base cranica, o sintomi relativi alla loro attività secernente. I paragangliomi sono per la maggior parte benigni e la loro prognosi è direttamente correlata alla posizione del tumore: quelli che si sviluppano alla biforcazione carotidea hanno esito migliore, mentre quelli situati a livello della base cranica hanno una prognosi meno favorevole. L’angiografia è necessaria prima dell’intervento nei paragangliomi più voluminosi per la pianificazione dell’intervento chirurgico (mappa vascolare) e, raramente, per l’embolizzazione preoperatoria. L’imaging morfologico e funzionale è altrettanto essenziale per la pianificazione del trattamento chirurgico e/o radiometabolico e nel follow-up.

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Authors and Affiliations

  1. Dipartimento di Radiodiagnostica, APSS di Trento, L.go Medaglie d’Oro 10, 38100, Trento, Italy

    G. Casagranda, S. Sammartano, U. Rozzanigo, P. Peterlongo & M. Centonze

  2. U.O. di Medicina II, Ospedale S. Chiara, APSS di Trento, Trento, Italy

    S. Demattè

  3. U.O. di Medicina Nucleare, Ospedale S. Chiara, APSS di Trento, Trento, Italy

    D. Donner

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  1. G. Casagranda
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  2. S. Demattè
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  4. S. Sammartano
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  6. P. Peterlongo
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Correspondence to G. Casagranda.

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Casagranda, G., Demattè, S., Donner, D. et al. Paragangliomas in an endemic area: from genetics to morphofunctional imaging. A pictorial essay. Radiol med 117, 471–487 (2012). https://doi.org/10.1007/s11547-011-0739-9

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  • DOI: https://doi.org/10.1007/s11547-011-0739-9

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