{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:06:04Z","timestamp":1790193964435,"version":"4.1.0"},"reference-count":41,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,8,26]],"date-time":"2021-08-26T00:00:00Z","timestamp":1629936000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Endocrinology"],"published-print":{"date-parts":[[2021,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>Neuroendocrine neoplasia (NEN) of the thymus is a very rare entity with a poor prognosis. None of the treatments was proofed by studies. Usually, therapy protocols for bronchopulmonary carcinoids are used. So far no data exist on the effect of mammalian target of rapamycin (mTOR) inhibitors. We describe our long\u2010term experience with everolimus and give a thorough review of the therapeutic strategies used so far.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p>Four patients (mean age 46 years, range 37\u201355) with progressing thymic NEN (t\u2010NEN) (two well\u2010differentiated atypical carcinoids and two atypical carcinoids with large cell characteristics) were treated with everolimus 10\u2009mg\/day after the failure of at least one previous medical therapy. Everolimus was applied after a mean interval of 32.4 months (range 5\u201356) after the first diagnosis. The follow\u2010up included clinical examination, imaging and chromogranin A testing in 3 or 6 monthly intervals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We observed stable disease for a mean of 20.8 months. Both patients with large cell characteristics t\u2010NEN (Ki\u201067 of 20%) had rapid progress after 7 and 10 months and had more previous therapies (three and six) than the patients with well\u2010differentiated t\u2010NEN (Ki\u201067 5% and 10%, progress after 24 and 42 months, one and two previous therapies). No severe side effects occurred. In three of four patients, everolimus led to stable disease for the longest\u00a0compared to the other nonsurgical therapies used.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Comparing the sparse data available everolimus is a promising treatment for t\u2010NEN at least in second\u2010line therapy. A low Ki\u201067 index was associated with a better outcome.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cen.14572","type":"journal-article","created":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T12:01:53Z","timestamp":1627560113000},"page":"744-751","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Long\u2010term effect of everolimus in recurrent thymic neuroendocrine neoplasia"],"prefix":"10.1111","volume":"95","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3585-4287","authenticated-orcid":false,"given":"Matthias","family":"Lang","sequence":"first","affiliation":[{"name":"Department of General, Visceral and Transplant Surgery University Hospital Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thilo","family":"Hackert","sequence":"additional","affiliation":[{"name":"Department of General, Visceral and Transplant Surgery University Hospital Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chrysanthi","family":"Anamaterou","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine University Hospital Heidelberg  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,8,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(197204)29:4<1061::AID-CNCR2820290456>3.0.CO;2-3"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.lungcan.2012.12.001"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e3181dd4ec4"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2014.08.061"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.13189\/cor.2013.010103"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2004-1155"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-017-3685-5"},{"key":"e_1_2_8_9_1","volume-title":"Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart","author":"Travis WD","year":"2004"},{"issue":"9","key":"e_1_2_8_10_1","first-page":"1445","article-title":"A case of primary thymic carcinoid treated with everolimus and octreotide","volume":"46","author":"Katagiri Y","year":"2019","journal-title":"Gan To Kagaku Ryoho"},{"key":"e_1_2_8_11_1","doi-asserted-by":"crossref","unstructured":"BaudinE CaplinM Garcia\u2010CarboneroRet al. on behalf of the ESMO Guidelines Committee. 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