{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T11:10:17Z","timestamp":1756379417560,"version":"3.44.0"},"reference-count":7,"publisher":"SMC Media","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Pancreatic neuroendocrine neoplasms (PanNENs) rarely secrete serotonin, which is the main cause of carcinoid syndrome. One of its unusual manifestations is carcinoid heart disease or Hedinger\u2019s syndrome which is seldom accompanied by cardiac arrhythmias. We report the case of an 88-year-old woman who presented with recently experienced episodes of palpitations and a newly developed atrial flutter with a ventricular rate of 130 beats per minute. Echocardiography revealed thickened and tethered tricuspid and pulmonary valve leaflets causing severe valvular regurgitation and right ventricular dilatation. Episodes of intermittent diarrhoea over the previous 2 years were mentioned, making carcinoid syndrome our working diagnosis. The 5-hydroxyindoleacetic acid (5-HIAA) levels in a 24-hour urine collection specimen were elevated. Conventional imaging studies and a Ga-68 dodecane tetraacetic acid tyrosine-3-octreotate (DOTATATE) positron emission tomography\/computer tomography (PET\/CT) scan revealed the presence of a metastatic PanNEN arising from the pancreatic tail. The patient was managed with lanreotide and telotristat with remarkable improvement of her symptoms. To our knowledge, this is the first reported case of carcinoid syndrome presenting with atrial flutter as the initial symptom.<\/jats:p>","DOI":"10.12890\/2021_002216","type":"journal-article","created":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T09:52:37Z","timestamp":1621936357000},"source":"Crossref","is-referenced-by-count":0,"title":["Rare Presentation of a Metastatic Pancreatic Neuroendocrine Neoplasm Presenting with Atrial Flutter"],"prefix":"10.12890","volume":"8","author":[{"given":"Paraskevi C","family":"Fragkou","sequence":"additional","affiliation":[{"name":"4th Department of Internal Medicine, Attikon University Hospital, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ioannis A","family":"Papadopoulos","sequence":"additional","affiliation":[{"name":"5th Department of Internal Medicine, Hygeia General Hospital, Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonios","family":"Papadopoulos","sequence":"additional","affiliation":[{"name":"4th Department of Internal Medicine, Attikon University Hospital, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Evangelos-Achilleas","family":"Kontoveros","sequence":"additional","affiliation":[{"name":"Ultrasound Department, Hygeia General Hospital, Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory","family":"Kaltsas","sequence":"additional","affiliation":[{"name":"1st Propaedeutic Department of Internal Medicine, Laikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Foula","family":"Vassilara","sequence":"additional","affiliation":[{"name":"Hygeia General Hospital, Athens"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10397","published-online":{"date-parts":[[2021,1,20]]},"reference":[{"key":"10.12890\/2021_002216_ref1","unstructured":"<li>WHO Classification of Tumours Editorial Board. Digestive System Tumours, WHO Classification of Tumours. 5th ed. Lyon, France: IARC Press; 2019.<\/li>"},{"key":"10.12890\/2021_002216_ref2","doi-asserted-by":"crossref","unstructured":"<li>Ito T, Lee L, Jensenc RT. Carcinoid-syndrome: recent advances, current status and controversies. <em>Curr Opin Endocrinol Diabetes Obes<\/em> 2018;<strong>25<\/strong>:22\u201335. <\/li>","DOI":"10.1097\/MED.0000000000000376"},{"key":"10.12890\/2021_002216_ref3","doi-asserted-by":"crossref","unstructured":"<li>Langer C, Piper C, Vogt J, Heintze J, Butz T, Lindner O, et al. Atrial fibrillation in carcinoid heart disease: the role of serotonin. A review of the literature. <em>Clin Res Cardiol<\/em> 2007;<strong>96<\/strong>:114\u2013118. <\/li>","DOI":"10.1007\/s00392-006-0463-y"},{"key":"10.12890\/2021_002216_ref4","doi-asserted-by":"crossref","unstructured":"<li>Pavel M, Oberg K, Falconi M, Krenning EP, Sundin A, Perren A, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow up. <em>Ann Oncol<\/em> 2020;<strong>31<\/strong>(7):844\u2013860. <\/li>","DOI":"10.1016\/j.annonc.2020.03.304"},{"key":"10.12890\/2021_002216_ref5","doi-asserted-by":"crossref","unstructured":"<li>Davar J, Connolly HM, Caplin ME, Pavel M, Zacks J, Bhattacharyya S, et al. Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. <em>J Am Coll Cardiol<\/em> 2017;<strong>69<\/strong>:1288\u20131304. <\/li>","DOI":"10.1016\/j.jacc.2016.12.030"},{"key":"10.12890\/2021_002216_ref6","doi-asserted-by":"crossref","unstructured":"<li>Mota JM, Sousa LG, Riechelmann RP. Complications from carcinoid syndrome: review of the current evidence.<em> Ecancermedicalscience<\/em> 2016 Aug 8;10.662. https:\/\/doi.org\/10.3332\/ecancer.2016.662.","DOI":"10.3332\/ecancer.2016.662"},{"key":"10.12890\/2021_002216_ref7","doi-asserted-by":"crossref","unstructured":"<li>Tsoli M, Chatzellis E, Koumarianou A, Kolomodi D, Kaltsas G. Current best practice in the management of neuroendocrine tumors. <em>Ther Adv Endocrinol Metab<\/em> 2019;<strong>10<\/strong>:1\u201318. <\/li>","DOI":"10.1177\/2042018818804698"}],"container-title":["European Journal of Case Reports in Internal Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/2216\/2522","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/2216\/2523","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/2216\/2523","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T10:29:52Z","timestamp":1756376992000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/view\/2216"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,20]]},"references-count":7,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021]]}},"URL":"https:\/\/doi.org\/10.12890\/2021_002216","relation":{"has-manifestation":[{"id-type":"other","id":"1-66-2216-g2522","asserted-by":"subject"},{"id-type":"other","id":"1-66-2216-g2523","asserted-by":"subject"}],"is-part-of":[{"id-type":"other","id":"1-66","asserted-by":"subject"}]},"ISSN":["2284-2594"],"issn-type":[{"type":"electronic","value":"2284-2594"}],"subject":[],"published":{"date-parts":[[2021,1,20]]},"article-number":"13"}}