{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T11:14:49Z","timestamp":1793272489619,"version":"4.5.0"},"reference-count":57,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2023,1,8]],"date-time":"2023-01-08T00:00:00Z","timestamp":1673136000000},"content-version":"vor","delay-in-days":111,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2022,9,19]],"date-time":"2022-09-19T00:00:00Z","timestamp":1663545600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To describe clinical and pathological characteristics and treatment outcomes in a large cohort of aggressive pituitary tumours (APT)\/pituitary carcinomas (PC).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design<\/jats:title>\n                  <jats:p>Electronic survey August 2020\u2013May 2021.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>96% of 171 (121 APT, 50 PC), initially presented as macro\/giant tumours, 6 were microadenomas (5 corticotroph). Ninety-seven tumours, initially considered clinically benign, demonstrated aggressive behaviour after 5.5 years (IQR: 2.8\u201312). Of the patients, 63% were men. Adrenocorticotrophic hormone (ACTH)-secreting tumours constituted 30% of the APT\/PC, and the gonadotroph subtypes were under-represented. Five out of 13 silent corticotroph tumours and 2\/6 silent somatotroph tumours became secreting. Metastases were observed after median 6.3 years (IQR 3.7\u201312.1) from diagnosis. At the first surgery, the Ki67 index was \u22653% in 74\/93 (80%) and \u226510% in 38\/93 (41%) tumours. An absolute increase of Ki67 \u2265 10% after median of 6 years from the first surgery occurred in 18\/49 examined tumours. Tumours with an aggressive course from outset had higher Ki67, mitotic counts, and p53. Temozolomide treatment in 156\/171 patients resulted in complete response in 9.6%, partial response in 30.1%, stable disease in 28.1%, and progressive disease in 32.2% of the patients. Treatment with bevacizumab, immune checkpoint inhibitors, and peptide receptor radionuclide therapy resulted in partial regression in 1\/10, 1\/6, and 3\/11, respectively. Median survival in APT and PC was 17.2 and 11.3 years, respectively. Tumours with Ki67 \u2265 10% and ACTH-secretion were associated with worse prognosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>APT\/PCs exhibit a wide and challenging spectrum of behaviour. Temozolomide is the first-line chemotherapy, and other oncological therapies are emerging. Treatment response continues to be difficult to predict with currently studied biomarkers.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1530\/eje-22-0440","type":"journal-article","created":{"date-parts":[[2022,8,26]],"date-time":"2022-08-26T16:43:26Z","timestamp":1661532206000},"page":"593-605","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":112,"title":["Aggressive pituitary tumours and carcinomas, characteristics and management of 171 patients"],"prefix":"10.1093","volume":"187","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4844-8336","authenticated-orcid":false,"given":"Pia","family":"Burman","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Sk\u00e5ne University Hospital Malm\u00f6, University of Lund , Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Trouillas","sequence":"additional","affiliation":[{"name":"Faculty of Medicine Lyon-Est, 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