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Endocr Rev. 2014 Oct;35(5):795-819. doi: 10.1210/er.2014-1026. Epub 2014 Jul 17.

Current approaches and recent developments in the management of head and neck paragangliomas.

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Department of Nuclear Medicine (D.T.), La Timone University Hospital, CERIMED, Aix-Marseille Univ, F-13385 Marseille, France; Department of Radiation Oncology (A.K.), Besançon University Hospital, F-25030 Besançon, France; Department of Otorhinolaryngology/Head and Neck Surgery (C.C.B.), HELIOS Hanseklinikum Stralsund, D-18435 Stralsund, Germany; Department of Otorhinolaryngology/Head and Neck Surgery (C.C.B.), University Hospital, Freiburg, Germany; Program in Reproductive and Adult Endocrinology (V.M., K.P.), Eunice Kennedy Shriver National Institute of Child Health and Human Development and Medical Oncology Branch (T.F.), National Institutes of Health, Bethesda, Maryland 20892; Department of Neurosurgery (J.R.A.), Stanford Hospital and Clinics, Stanford University, Stanford, California 94305.


Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors belonging to the family of pheochromocytoma/paraganglioma neoplasms. Despite advances in understanding the pathogenesis of these tumors, the growth potential and clinical outcome of individual cases remains largely unpredictable. Over several decades, surgical resection has long been the treatment of choice for HNPGLs. However, increasing experience in various forms of radiosurgery has been reported to result in curative-like outcomes, even for tumors localized in the most inaccessible anatomical areas. The emergence of such new therapies challenges the traditional paradigm for the management of HNPGLs. This review will assist and guide physicians who encounter patients with such tumors, either from a diagnostic or therapeutic standpoint. This review will also particularly emphasize current and emerging knowledge in genetics, imaging, and therapeutic options as well as the health-related quality of life for patients with HNPGLs.

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