Vincent Amodru1, Pauline Romanet2, Ugo Scemama3, Marion Montava4, Nicolas Fakhry4, Frédéric Sebag5, Frédéric Castinetti1, Jean-Pierre Lavieille2, Anderson Loundou6, Arthur Varoquaux3, Anne Barlier2, Karel Pacak7, David Taïeb8,9. 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.
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.
Authors: B E Baysal; R E Ferrell; J E Willett-Brozick; E C Lawrence; D Myssiorek; A Bosch; A van der Mey; P E Taschner; W S Rubinstein; E N Myers; C W Richard; C J Cornelisse; P Devilee; B Devlin Journal: Science Date: 2000-02-04 Impact factor: 47.728
Authors: Henri J L M Timmers; Karel Pacak; Jérôme Bertherat; Jacques W M Lenders; Michèle Duet; Graeme Eisenhofer; Constantine A Stratakis; Patricia Niccoli-Sire; Ba Huy Patrice Tran; Nelly Burnichon; Anne-Paule Gimenez-Roqueplo Journal: Clin Endocrinol (Oxf) Date: 2007-10-31 Impact factor: 3.478
Authors: Aurélien Archier; Arthur Varoquaux; Philippe Garrigue; Marion Montava; Carole Guerin; Sophie Gabriel; Eva Beschmout; Isabelle Morange; Nicolas Fakhry; Frédéric Castinetti; Frédéric Sebag; Anne Barlier; Anderson Loundou; Benjamin Guillet; Karel Pacak; David Taïeb Journal: Eur J Nucl Med Mol Imaging Date: 2015-12-05 Impact factor: 9.236
Authors: Ingo Janssen; Clara C Chen; David Taieb; Nicholas J Patronas; Corina M Millo; Karen T Adams; Joan Nambuba; Peter Herscovitch; Samira M Sadowski; Antonio T Fojo; Inga Buchmann; Electron Kebebew; Karel Pacak Journal: J Nucl Med Date: 2015-11-12 Impact factor: 10.057