{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:20:23Z","timestamp":1784557223987,"version":"3.55.0"},"reference-count":54,"publisher":"Bioscientifica","issue":"2","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,2,1]]},"abstract":"<jats:p>\n                    Germline mutations in genes encoding succinate dehydrogenase (SDH) are frequently involved in pheochromocytoma\/paraganglioma (PPGL) development and were implicated in patients with the \u20183PAs\u2019 syndrome (associating pituitary adenoma (PA) and PPGL) or isolated PA. However, the causality link between\n                    <jats:italic>SDHx<\/jats:italic>\n                    mutation and PA remains difficult to establish, and\n                    <jats:italic>in vivo<\/jats:italic>\n                    tools for detecting hallmarks of SDH deficiency are scarce. Proton magnetic resonance spectroscopy (\n                    <jats:sup>1<\/jats:sup>\n                    H-MRS) can detect succinate\n                    <jats:italic>in vivo<\/jats:italic>\n                    as a biomarker of\n                    <jats:italic>SDHx<\/jats:italic>\n                    mutations in PGL. The objective of this study was to demonstrate the causality link between PA and SDH deficiency\n                    <jats:italic>in vivo<\/jats:italic>\n                    using\n                    <jats:sup>1<\/jats:sup>\n                    H-MRS as a novel noninvasive tool for succinate detection in PA. Three\n                    <jats:italic>SDHx<\/jats:italic>\n                    -mutated patients suffering from a PPGL and a macroprolactinoma and one patient with an apparently sporadic non-functioning pituitary macroadenoma underwent MRI examination at 3 T. An optimized\n                    <jats:sup>1<\/jats:sup>\n                    H-MRS semi-LASER sequence (TR\u2009=\u20092500 ms, TE\u2009=\u2009144 ms) was employed for the detection of succinate\n                    <jats:italic>in vivo.<\/jats:italic>\n                    Succinate and choline-containing compounds were identified in the MR spectra as single resonances at 2.44 and 3.2 ppm, respectively. Choline compounds were detected in all the tumors (three PGL and four PAs), while a succinate peak was only observed in the three macroprolactinomas and the three PGL of\n                    <jats:italic>SDHx<\/jats:italic>\n                    -mutated patients, demonstrating SDH deficiency in these tumors. In conclusion, the detection of succinate by\n                    <jats:sup>1<\/jats:sup>\n                    H-MRS as a hallmark of SDH deficiency\n                    <jats:italic>in vivo<\/jats:italic>\n                    is feasible in PA, laying the groundwork for a better understanding of the biological link between\n                    <jats:italic>SDHx<\/jats:italic>\n                    mutations and the development of these tumors.\n                  <\/jats:p>","DOI":"10.1530\/erc-22-0198","type":"journal-article","created":{"date-parts":[[2022,11,30]],"date-time":"2022-11-30T13:45:50Z","timestamp":1669815950000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":7,"title":["SDHx mutation and pituitary adenoma: can in vivo 1H-MR spectroscopy unravel the link?"],"prefix":"10.1530","volume":"30","author":[{"given":"Francesca","family":"Branzoli","sequence":"first","affiliation":[{"name":"Paris Brain Institute - Institut du Cerveau (ICM), Center for Neuroimaging Research (CENIR), Paris, France"},{"name":"Sorbonne University, UMR S 1127, Inserm U 1127, CNRS UMR 7225, ICM, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0889-0226","authenticated-orcid":true,"given":"Betty","family":"Salgues","sequence":"additional","affiliation":[{"name":"Sorbonne University, nuclear medicine department, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Assistance -Publique H\u00f4pitaux de Paris, Paris, France"},{"name":"Paris Cardiovascular Research Center (PARCC), Inserm, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4727-2447","authenticated-orcid":true,"given":"Ma\u0142gorzata","family":"Marja\u0144ska","sequence":"additional","affiliation":[{"name":"Center for Magnetic Resonance Research, Department of Radiology, University of Minnesota, Minneapolis, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Laloi-Michelin","sequence":"additional","affiliation":[{"name":"Endocrinology department, Lariboisi\u00e8re Hospital, Assistance -Publique H\u00f4pitaux de Paris, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Herman","sequence":"additional","affiliation":[{"name":"ENT unit, Lariboisi\u00e8re Hospital, Assistance -Publique H\u00f4pitaux de Paris, Paris-Cit\u00e9 University, INSERM U1141, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauriane","family":"Le Collen","sequence":"additional","affiliation":[{"name":"Inserm\/CNRS UMR 1283\/8199, Pasteur Institute of Lille, EGID, University of Lille, Lille, France"},{"name":"Department of Endocrinology Diabetology, University Hospital Center of Reims, Reims, France"},{"name":"Department of Genetic, University Hospital Center of Reims, Reims, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Delemer","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Diabetology, University Hospital Center of Reims, Reims, France"},{"name":"CRESTIC EA 3804, University of Reims Champagne Ardenne, UFR Sciences Exactes et Naturelles, Moulin de La Housse, BP 1039, Reims, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3002-1780","authenticated-orcid":true,"given":"Julien","family":"Riancho","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, Hypertension Unit, and Reference centre for rare adrenal diseases, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Kuhn","sequence":"additional","affiliation":[{"name":"Pituitary Unit, Piti\u00e9-Salp\u00eatri\u00e8re Hospital APHP, Sorbonne University, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christel","family":"Jublanc","sequence":"additional","affiliation":[{"name":"Pituitary Unit, Piti\u00e9-Salp\u00eatri\u00e8re Hospital APHP, Sorbonne University, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nelly","family":"Burnichon","sequence":"additional","affiliation":[{"name":"D\u00e9partement de m\u00e9decine g\u00e9nomique des tumeurs et des cancers, AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, Paris, France"},{"name":"Universit\u00e9 Paris Cit\u00e9, Inserm, PARCC, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence","family":"Amar","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, Hypertension Unit, and Reference centre for rare adrenal diseases, Paris, France"},{"name":"Universit\u00e9 Paris Cit\u00e9, Inserm, PARCC, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8190-5853","authenticated-orcid":true,"given":"Judith","family":"Favier","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris Cit\u00e9, Inserm, PARCC, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Paule","family":"Gimenez-Roqueplo","sequence":"additional","affiliation":[{"name":"D\u00e9partement de m\u00e9decine g\u00e9nomique des tumeurs et des cancers, AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, Paris, France"},{"name":"Universit\u00e9 Paris Cit\u00e9, Inserm, PARCC, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre","family":"Buffet","sequence":"additional","affiliation":[{"name":"D\u00e9partement de m\u00e9decine g\u00e9nomique des tumeurs et des cancers, AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, Paris, France"},{"name":"Universit\u00e9 Paris Cit\u00e9, Inserm, PARCC, 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