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Head Neck. 2018 Dec 24. doi: 10.1002/hed.25604. [Epub ahead of print]

Tumor multifocality with vagus nerve involvement as a phenotypic marker of SDHD mutation in patients with head and neck paragangliomas: A 18 F-FDOPA PET/CT study.

Author information

1
Department of Endocrinology, Conception University Hospital, Aix-Marseille University, Marseille, France.
2
Laboratory of Molecular Biology, Conception Hospital & CNRS, CRN2M UMR 7286, Aix-Marseille University, Marseille, France.
3
Department of Radiology, Conception Hospital, Aix-Marseille University, Marseille, France.
4
Department of Otorhinolaryngology-Head and Neck Surgery, Conception Hospital, Aix-Marseille University, Marseille, France.
5
Department of Endocrine Surgery, Conception University Hospital, Aix-Marseille University, Marseille, France.
6
Department of Public Health, EA3279 Self-perceived Health Assessment Research Unit, La Timone University, Aix-Marseille University, Marseille, France.
7
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health & Human Development, National Institutes of Health, Bethesda, Maryland.
8
Department of Nuclear Medicine, La Timone University Hospital, Aix-Marseille University, Marseille, France.
9
European Center for Research in Medical Imaging, Aix-Marseille University, Marseille, France.

Abstract

BACKGROUND:

18 F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the relationship between tumor characteristics and the SDHx-mutational status in a large series of patients with HNPGL evaluated by 18 F-FDOPA PET/CT.

METHODS:

A total of 104 patients with HNPGL (65 sporadic/39 SDHx-mutated) were included.

RESULTS:

In comparison to SDHB/SDC/SDHx-negative cases, patients with SDHD were younger at diagnosis and had a higher rate of multifocal, vagal, and carotid paraganglioma. In patients with SDHD, vagal paraganglia represented the primary site of tumor origin. Multicentric involvement of the vagus nerve alone or in association with other locations was found to be a typical feature of SDHD cases compared to other cases (odds ratio = 59.4).

CONCLUSION:

The present study shows that tumor multifocality within the vagus nerve is a phenotypic marker of SDHD mutation. This information is essential in the choice of the therapeutic strategy.

KEYWORDS:

diagnostic imaging; genetics; paragangliomas; vagus nerve

PMID:
30584686
DOI:
10.1002/hed.25604

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