Naslov
Aggressive pituitary tumours and carcinomas, characteristics and management of 171 patients

Autori
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

Izvornik
European Journal of Endocrinology (0804-4643) 187 (2022), 4; 593-605

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
Aggressive pituitary tumours

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA