{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T20:08:20Z","timestamp":1791403700768,"version":"4.3.3"},"reference-count":12,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,10,26]],"date-time":"2023-10-26T00:00:00Z","timestamp":1698278400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Nucl. Med."],"abstract":"<jats:p>TEMPI syndrome (TEMPI) compounds telangiectasias and polycythemia with elevated erythropoietin levels, monoclonal gammopathy, perirenal fluid collections, and intrapulmonary shunt. Although the pathophysiology of this syndrome remains unclarified, prior research has been established that it is a plasma cell neoplasm, often containing less than 10% bone marrow plasma cells. <jats:sup>18<\/jats:sup>F-FDG PET serves as a valuable instrument for initial staging and treatment monitoring in multiple myeloma management. Thus, <jats:sup>18<\/jats:sup>F-FDG PET can be legitimately applied for TEMPI assessment. Here, we present the first <jats:sup>18<\/jats:sup>F-FDG PET images for the initial evaluation and treatment monitoring of TEMPI in a 51-year-old woman, who exhibited polycythemia (EPO:5,448 mIU\/ml) without JAK2 mutation, telangiectasias, monoclonal IgG lambda gammopathy (13.9) g\/L and 7% dysmorphic plasma cells (CD38\u2009+\u2009CD138+), occasionally clustered, in favor of tumoral plasmacytomas. The first PET scan exhibited hypermetabolic diffuse bone marrow, potentially related to polycythemia, accompanied by non-lytic bone hypermetabolic lesions in the femoral and humeral diaphysis, and ametabolic peri-renal fluid collections, brown fat, and pleural talcoma. Post-treatment <jats:sup>18<\/jats:sup>F-FDG PET (Daratumumab Bortezomib Thalidomide Dexamethasone) revealed a completely reduced signal of bone lesions, suggesting a complete response, which was substantiated both clinically and biologically, with the concurrent disappearance of telangiectasia and the monoclonal component, and the normalization of the EPO level. In future, additional data will be required to confirm the added value of <jats:sup>18<\/jats:sup>F-FDG PET with TEMPI. Nevertheless, <jats:sup>18<\/jats:sup>F-FDG PET can be a preferred tool for the extension workup and therapeutic evaluation of TEMPI syndrome.<\/jats:p>","DOI":"10.3389\/fnume.2023.1273967","type":"journal-article","created":{"date-parts":[[2023,10,27]],"date-time":"2023-10-27T03:18:04Z","timestamp":1698376684000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["First use of 18F-FDG PET in TEMPI syndrome: can it be used for treatment assessment? A case report"],"prefix":"10.3389","volume":"3","author":[{"given":"Henri","family":"Pasquesoone","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lien","family":"Callaud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thibaut","family":"Carsuzaa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Chalopin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria-Joao","family":"Santiago-Ribeiro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,10,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"949647","DOI":"10.3389\/fonc.2022.949647","article-title":"Case report: TEMPI syndrome: report of three cases and treatment follow-up","volume":"12","author":"Xu","year":"2022","journal-title":"Front Oncol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1056\/NEJMc1106670","article-title":"The TEMPI syndrome\u2013a novel multisystem disease","volume":"365","author":"Sykes","year":"2011","journal-title":"N Engl J Med"},{"key":"B3","doi-asserted-by":"publisher","first-page":"1199","DOI":"10.1182\/blood.2019004216","article-title":"The TEMPI syndrome","volume":"135","author":"Sykes","year":"2020","journal-title":"Blood"},{"key":"B4","doi-asserted-by":"publisher","first-page":"886961","DOI":"10.3389\/fendo.2022.886961","article-title":"TEMPI syndrome: update on clinical features, management, and pathogenesis","volume":"13","author":"Xu","year":"2022","journal-title":"Front Endocrinol (Lausanne)"},{"key":"B5","doi-asserted-by":"publisher","first-page":"724","DOI":"10.1016\/j.clml.2018.07.284","article-title":"TEMPI syndrome: erythrocytosis in plasma cell dyscrasia","volume":"18","author":"Zhang","year":"2018","journal-title":"Clin Lymphoma Myeloma Leuk"},{"key":"B6","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1053\/j.semnuclmed.2017.02.004","article-title":"Benign bone conditions that may be FDG-avid and mimic malignancy","volume":"47","author":"Kwee","year":"2017","journal-title":"Semin Nucl Med"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1334","DOI":"10.2967\/jnumed.115.160507","article-title":"Characterizing POEMS syndrome with 18F-FDG PET\/CT","volume":"56","author":"Pan","year":"2015","journal-title":"J Nucl Med"},{"key":"B8","doi-asserted-by":"publisher","first-page":"1231","DOI":"10.1007\/s00259-015-3282-4","article-title":"Malignancy rate of biopsied suspicious bone lesions identified on FDG PET\/CT","volume":"43","author":"Adams","year":"2016","journal-title":"Eur J Nucl Med Mol Imaging"},{"key":"B9","doi-asserted-by":"publisher","first-page":"1843","DOI":"10.1056\/NEJMc1202649","article-title":"Bortezomib to treat the TEMPI syndrome","volume":"366","author":"Kwok","year":"2012","journal-title":"N Engl J Med"},{"key":"B10","doi-asserted-by":"publisher","first-page":"553","DOI":"10.1159\/000525056","article-title":"Successful treatment of the TEMPI syndrome with pomalidomide plus dexamethasone followed by autologous stem cell transplantation","volume":"145","author":"Kawamura","year":"2022","journal-title":"Acta Haematol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"702","DOI":"10.1016\/j.clml.2022.04.018","article-title":"Long-term complete clinical and hematological response with bortezomib: the report of a case with TEM(P)I syndrome and a review of the literature","volume":"22","author":"Undar","year":"2022","journal-title":"Clin Lymphoma Myeloma Leuk"},{"key":"B12","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1038\/modpathol.2014.117","article-title":"Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist","volume":"28","author":"Rosado","year":"2015","journal-title":"Mod Pathol"}],"container-title":["Frontiers in Nuclear Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fnume.2023.1273967\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,27]],"date-time":"2023-10-27T03:18:06Z","timestamp":1698376686000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fnume.2023.1273967\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,26]]},"references-count":12,"alternative-id":["10.3389\/fnume.2023.1273967"],"URL":"https:\/\/doi.org\/10.3389\/fnume.2023.1273967","relation":{},"ISSN":["2673-8880"],"issn-type":[{"value":"2673-8880","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,26]]},"article-number":"1273967"}}