Tympanojugular Paragangliomas: Surgical Management and Clinicopathological Features

Sampath Chandra Prasad; Carlo Terenzio Paties; Francesca Schiavi; Diana Liberata Esposito; Lavinia Vittoria Lotti; Renato Mariani- Costantini; Mario Sanna

doi:10.15586/paraganglioma.2019.ch6

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Published: Jun 9, 2019

DOI: https://doi.org/10.15586/paraganglioma.2019.ch6

Keywords:

angiography, high-resolution computed tomography, infratemporal fossa approach, modified Fisch classification, tympanojugular paraganglioma

Sampath Chandra Prasad

Otology and Skull Base Unit, Gruppo Otologico Piacenza, Piacenza, Italy

Carlo Terenzio Paties

Department of Oncology-Hematology, Service of Anatomic Pathology, Guglielmo da Saliceto Hospital, Piacenza, Italy

Francesca Schiavi

Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IRCCS, Padua, Italy

Diana Liberata Esposito

Center of Aging Science and Translational Medicine (CeSI-MeT) and Department of Medical, Oral and Biotechnological Sciences, Gabriele d’Annunzio University, Chieti, Italy

Lavinia Vittoria Lotti

Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy

Renato Mariani- Costantini

Center of Aging Science and Translational Medicine (CeSI-MeT) and Department of Medical, Oral and Biotechnological Sciences, Gabriele d’Annunzio University, Chieti, Italy

Mario Sanna

Otology and Skull Base Unit, Gruppo Otologico Piacenza, Piacenza, Italy

ABSTRACT

In this chapter, we provide a focused review on a highly challenging subset of head and neck paragangliomas, that is, those arising from the skull base (jugulotympanic region). Presently, these tumors can be cured only with surgery, which must be performed in highly specialized skull base surgical centers. We review here the clinical presentation, diagnostic workup, classification, and surgical management of these rare but important tumors, together with currently available evidence concerning genetics, developmental origin, and pathology.