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Review
. 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

David Taïeb  1 , Alexandre Kaliski, Carsten C Boedeker, Victoria Martucci, Tito Fojo, John R Adler Jr, Karel Pacak
Affiliations
Review

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

David Taïeb et al. Endocr Rev. 2014 Oct.

Abstract

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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Figures

Figure 1.
Figure 1.
Relationship between SDH complex dysfunction and tumorigenesis. Abbreviations: IMM, inner mitochondrial membrane; 2-OG, 2-oxoglutarate.
Figure 2.
Figure 2.
Proposed imaging algorithm.
Figure 3.
Figure 3.
Radiosurgical planning in a patient with sporadic multifocal PGL (vagal and jugular locations). [18F]FDOPA PET/CT showing vagal (arrows) and jugular (arrowheads) locations in a sporadic case (axial view) centered over the vagal PGL (A) and maximum intensity projection (MIP) (D). 3D reconstruction of single-fraction dynamic conformal arc RS planning (B) based on 3D [18F]FDOPA PET/MR fusion image using the iPlanDose 4.5 (BrainLab) treatment planning software. Organs at risk (brainstem, eyes, optic nerves, optic chiasm, and optic tracts) as well as the 2 tumors are auto-segmented (on T1-weighted MRI) and manually delineated (on [18F]FDOPA PET/CT images) and are represented in 3D. Beams and isodoses are represented using color wash display on axial (C), sagittal (E), and coronal (F) views.
Figure 4.
Figure 4.
Clinical algorithm for management of HNPGLs.
See this image and copyright information in PMC

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