Aggressive pituitary tumours and carcinomas, characteristics and management of 171 patients. Issue 4 (19th September 2022)

• Record Type: Journal Article
• Authors: Burman, Pia; Trouillas, Jacqueline; Losa, Marco; McCormack, Ann; Petersenn, Stephan; Popovic, Vera; Theodoropoulou, Marily; Raverot, Gerald; Dekkers, Olaf M
• Other Names: author non-byline.; Guenego Agathe author non-byline.; Micko Alexander author non-byline.; Hubalewska-Dydejezky Alicia author non-byline.; Troendle Amineh author non-byline.; McCormack Ann author non-byline.; Krogh Rasmussen Åse author non-byline.; Whitelaw Ben author non-byline.; Decoudier Benedicte author non-byline.; Ekman Bertil author non-byline.; Engström Britt Eden author non-byline.; Höybye Charlotte author non-byline.; Jublanc Christel author non-byline.; Cortet Rudelli Christine author non-byline.; Higham Claire author non-byline.; Garcia Cyril author non-byline.; Bresson Damien author non-byline.; Henley David author non-byline.; Larrieu-Ciron Delphine author non-byline.; Maiter Dominique author non-byline.; Laws Edward R. author non-byline.; Christ Emanuel author non-byline.; Kuhn Emmanuelle author non-byline.; Ceccato Filippo author non-byline.; Schillo Franck author non-byline.; Castinetti Frederic author non-byline.; Raverot Gerald author non-byline.; Mantovani Giovanna author non-byline.; Vila Greisa author non-byline.; Lasolle Helene author non-byline.; Garay Ismene Bilbao author non-byline.; Kralievic Ivana author non-byline.; Otto Lunde Jorgensen Jens author non-byline.; Berinder Katarina author non-byline.; Ritzel Katrin author non-byline.; Bach Leon author non-byline.; Ortiz Leon D author non-byline.; Criniere Lise author non-byline.; Syro Luis author non-byline.; Haissaguerre Magalie author non-byline.; Losa Marco author non-byline.; Zatelli Maria Chiara author non-byline.; Batisse-Lignier Marie author non-byline.; Jaffrain-Rea Marie-Lise author non-byline.; Korbonits Marta author non-byline.; Ragonese Marta author non-byline.; Reincke Martin author non-byline.; Toth Miklos author non-byline.; Bourcigaux Nathalie author non-byline.; Chevalier Nicolas author non-byline.; Ragnarsson Oskar author non-byline.; Chanson Philippe author non-byline.; Burman Pia author non-byline.; Pekic Sandra author non-byline.; Petersenn Stephan author non-byline.; Mallea-Gil Susana author non-byline.; Usui Takeshi author non-byline.; Deutschbein Timo author non-byline.; Mazzuco Tania Longo author non-byline.; Dusek Tina author non-byline.; Feldt-Rasmussen Ulla author non-byline.; Popovic Vera author non-byline.; Greenman Yona author non-byline.;
• Is Part Of: European journal of endocrinology. Volume 187:Issue 4(2022)
• Page Start: 593
• Page End: 605
• Publication Date: 2022-09-19
• Subjects: Endocrinology -- Periodicals; 616.4005
• DOI: 10.1530/EJE-22-0440
• Languages: English
• ISSNs: 0804-4643

Abstract

Abstract : Objective: To describe clinical and pathological characteristics and treatment outcomes in a large cohort of aggressive pituitary tumours (APT)/pituitary carcinomas (PC). Design: Electronic survey August 2020–May 2021. Results: 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: 2.8–12). 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 3.7–12.1) from diagnosis. At the first surgery, the Ki67 index was ≥3% in 74/93 (80%) and ≥10% in 38/93 (41%) tumours. An absolute increase of Ki67 ≥ 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 ≥ 10% and ACTH-secretion were associated with worse prognosis. Conclusion: 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.