title: Magnetic resonance spectroscopy of paragangliomas: new insights into in vivo metabolomics.
abstract: Paragangliomas (PGLs) can be associated with mutations in genes of the tricarboxylic acid (TCA) cycle. Succinate dehydrogenase (SDHx) mutations are the prime examples of genetically determined TCA cycle defects with accumulation of succinate. Succinate, which acts as an oncometabolite, can be detected by ex vivo metabolomics approaches. The aim of this study was to evaluate the potential role of proton magnetic resonance (MR) spectroscopy ((1)H-MRS) for identifying SDHx-related PGLs in vivo and noninvasively. Eight patients were prospectively evaluated with single voxel (1)H-MRS. MR spectra from eight tumors (four SDHx-related PGLs, two sporadic PGLs, one cervical schwannoma, and one cervical neurofibroma) were acquired and interpreted qualitatively. Compared to other tumors, a succinate resonance peak was detected only in SDHx-related tumor patients. Spectra quality was considered good in three cases, medium in two cases, poor in two cases, and uninterpretable in the latter case. Smaller lesions had lower spectra quality compared to larger lesions. Jugular PGLs also exhibited a poorer spectra quality compared to other locations. (1)H-MRS has always been challenging in terms of its technical requisites. This is even more true for the evaluation of head and neck tumors. However, (1)H-MRS might be added to the classical MR sequences for metabolomic characterization of PGLs. In vivo detection of succinate might guide genetic testing, characterize SDHx variants of unknown significance (in the absence of available tumor sample), and even optimize a selection of appropriate therapies.
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France david.taieb@ap-hm.fr DamienArthur.VAROQUAUX@ap-hm.fr.
authors.forename: Arthur
authors.initials: A
authors.lastname: Varoquaux
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France.
authors.forename: Yann
authors.initials: Y
authors.lastname: le Fur
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes o
authors.forename: Alessio
authors.initials: A
authors.lastname: Imperiale
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France.
authors.forename: Antony
authors.initials: A
authors.lastname: Reyre
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France.
authors.forename: Marion
authors.initials: M
authors.lastname: Montava
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Fakhry
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes o
authors.forename: Izzie-Jacques
authors.initials: IJ
authors.lastname: Namer
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France.
authors.forename: Guy
authors.initials: G
authors.lastname: Moulin
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France.
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes o
authors.forename: Maxime
authors.initials: M
authors.lastname: Guye
authors.affiliation: Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland 20892, USADepartment of Nuclear MedicineLa Timone University Hospital, Aix-Marseille University, Marseille, FranceBiophysics and Nuclear MedicineEuropean Center for Research in Medical Imaging, La Timone University Hospital, Aix-Marseille University, 264, Rue Saint-Pierre, 13385 Marseille, FranceINSERMUMR1068, Institut Paoli-Calmettes, Marseille Cancerology Research Center, Marseille, France Department of Medical ImagingLa Timone University Hospital, Aix-Marseille University, Marseille, FranceCNRSCRMBM UMR 7339, Aix-Marseille University, 13385 Marseille, FranceDepartment of Biophysics and Nuclear MedicineUniversity Hospitals of Strasbourg, Strasbourg, FranceICubeUMR 7357, Faculty of Medicine, University of Strasbourg/CNRS and FMTS, Strasbourg, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryNorth Hospital, Aix-Marseille University, Marseille, FranceDepartment of Otorhinolaryngology-Head and Neck SurgeryLa Timone University Hospital, Aix-Marseille University, Marseille, FranceProgram in Reproductive and Adult Endocrinology<ce:italic>Eunice Kennedy Shriver</ce:italic> National Institute of Child Health and Human Development (NICHD), National Institutes o
authors.forename: David
authors.initials: D
authors.lastname: Taïeb
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D055432:Metabolomics; D008875:Middle Aged; D010235:Paraganglioma / Q000175:diagnosis* / Q000201:enzymology* / Q000473:pathology; D066244:Proton Magnetic Resonance Spectroscopy; D013385:Succinate Dehydrogenase / Q000737:chemistry*; D047368:Tumor Burden
chemical_list: D013385:Succinate Dehydrogenase
keywords: MR-spectroscopy; metabolomics; paraganglioma; positron-emission tomography