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; Guenego, Agathe; Micko, Alexander; Hubalewska-Dydejezky, Alicia; Troendle, Amineh; McCormack, Ann; Krogh Rasmussen, Åse; Whitelaw, Ben; Decoudier, Benedicte; Ekman, Bertil; Engström, Britt Eden; Höybye, Charlotte; Jublanc, Christel; Cortet Rudelli, Christine; Higham, Claire; Garcia, Cyril; Bresson, Damien; Henley, David; Larrieu-Ciron, Delphine; Maiter, Dominique; Laws, Edward R; Christ, Emanuel; Kuhn, Emmanuelle; Ceccato, Filippo; Schillo, Franck; Castinetti, Frederic; Raverot, Gerald; Mantovani, Giovanna; Vila, Greisa; Lasolle, Helene; Garay, Ismene Bilbao; Kralievic, Ivana; Otto Lunde Jorgensen, Jens; Berinder, Katarina; Ritzel, Katrin; Bach, Leon; Ortiz, Leon D; Criniere, Lise; Syro, Luis; Haissaguerre, Magalie; Losa, Marco; Zatelli, Maria Chiara; Batisse-Lignier, Marie; Jaffrain-Rea, Marie-Lise; Korbonits, Marta; Ragonese, Marta; Reincke, Martin; Toth, Miklos; Bourcigaux, Nathalie; Chevalier, Nicolas; Ragnarsson, Oskar; Chanson, Philippe; Burman, Pia; Pekic, Sandra; Petersenn, Stephan; Mallea-Gil, Susana; Usui, Takeshi; Deutschbein, Timo; Mazzuco, Tania Longo; Dusek, Tina; Feldt-Rasmussen, Ulla; Popovic, Vera; Greenman, Yona;
• 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.