| Contributors: |
Burman, P.; Trouillas, J.; Losa, M.; Mccormack, A.; Petersenn, S.; Popovic, V.; Theodoropoulou, M.; Raverot, G.; Dekkers, O. M.; Guenego, A.; Micko, A.; Hubalewska-Dydejezky, A.; Troendle, A.; Rasmussen, Å. K.; Whitelaw, B.; Decoudier, B.; Ekman, B.; Engström, B. E.; Höybye, C.; Jublanc, C.; Rudelli, C. C.; Higham, C.; Garcia, C.; Bresson, D.; Henley, D.; Larrieu-Ciron, D.; Maiter, D.; Laws, E. R.; Christ, E.; Kuhn, E.; Ceccato, F.; Schillo, F.; Castinetti, F.; Mantovani, G.; Vila, G.; Lasolle, H.; Garay, I. B.; Kralievic, I.; Jorgensen, J. O. L.; Berinder, K.; Ritzel, K.; Bach, L.; Ortiz, L. D.; Criniere, L.; Syro, L.; Haissaguerre, M.; Zatelli, M. C.; Batisse-Lignier, M.; Jaffrain-Rea, M. -L.; Korbonits, M.; Ragonese, M.; Reincke, M.; Toth, M.; Bourcigaux, N.; Chevalier, N.; Ragnarsson, O.; Chanson, P.; Pekic, S.; Mallea-Gil, S.; Usui, T.; Deutschbein, T. L. M. T.; Dusek, T.; Feldt-Rasmussen, U.; Greenman, Y. |
| Description: |
ObjectiveTo describe clinical and pathological characteristics and treatment outcomes in a large cohort of aggressive pituitary tumours (APT)/pituitary carcinomas (PC). DesignElectronic survey August 2020-May 2021. Results96% 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. ConclusionAPT/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. |