Treatment of aggressive pituitary tumours and carcinomas: results of a European Society of Endocrinology (ESE) survey 2016
Authors: McCormack Ann , Dekkers Olaf M , Petersenn Stephan , Popovic Vera , Trouillas Jacqueline , Raverot Gerald , Burman Pia , _ _ , _ _ , Hubalewska-Dydejezky Alicia , Assie Guillaume , Bach Leon , Batisse-Lignier Marie , Berinder Katarina , Bilbao Ismene , Bonnet Fabrice , Bresson Damien , Bruno Oscar , Campdera Mariana , Caron Philippe , Castinetti Frederic , Ceccato Filippo , Chabre Olivier , Chanson Philippe , Christ Emanuel , Cloix Lucie , Cortet Christine , Criniere Lise , Cuatrecasas Guillem , Debono Miguel , Delemer Brigitte , Desailloud Rachel , Deutschbein Timo , Dusek Tina , Engström Britt Eden , Faustini-Fustini Marco , Franck Schillo , Garcia Cyril , Greenman Yona , Gil Susana Mallea , Mantovani Giovanna , Gurnell Mark , Heaney Anthony , Henley David , Higham Claire , Hoving EW , Höybye Charlotte , Ichihara Atsuhiro , Jaffrain-Rea Marie-Lise , Johannsson Gudmundur , Jorgensen Jens Otto Lunde , Jublanc Christel , Komor Jan , Korbonits Marta , Kralievic Ivana , Larrieu-Ciron Delphine , Lasolle Helene , Laws Edward , Losa Marco , Maiter Dominique , Marcocci Claudio , Marques Olinda Castro , Mazzuco Tania Longo , Micko Alexander , Bourcigaux Nathalie , Neggers Sebastian , Newell-Price John , Perez-Berida Belén , Ortiz Leon D , Ragnarsson Oskar , Ragonese Marta , Reincke Martin , Sadoul Jean-Louis , Shimatsu Akira , Syro Luis V , Taillandier Luc , Toth Miklos , Usui Takeshi , Valkusz Zauzsanna , Vila Greisa , Whitelaw Ben , Zatelli Maria Chiara 
Published: January 2018

Objective
To collect outcome data in a large cohort of patients with aggressive pituitary tumours (APT)/carcinomas (PC) and specifically report effects of temozolomide (TMZ) treatment.

Design
Electronic survey to ESE members Dec 2015–Nov 2016.

Results
Reports on 166 patients (40 PC, 125 APT, 1 unclassified) were obtained. Median age at diagnosis was 43 (range 4–79) years. 69% of the tumours were clinically functioning, and the most frequent immunohistochemical subtype were corticotroph tumours (45%). Ki-67 index did not distinguish APT from PC, median 7% and 10% respectively. TMZ was first-line chemotherapy in 157 patients. At the end of the treatment (median 9 cycles), radiological evaluation showed complete response (CR) in 6%, partial response (PR) in 31%, stable disease (SD) in 33% and progressive disease in 30%. Response was more frequent in patients receiving concomitant radiotherapy and TMZ. CR was seen only in patients with low MGMT expression. Clinically functioning tumours were more likely to respond than non-functioning tumours, independent of MGMT status. Of patients with CR, PR and SD, 25, 40 and 48% respectively progressed after a median of 12-month follow-up. Other oncological drugs given as primary treatment and to TMZ failures resulted in PR in 20%.

Conclusion
This survey confirms that TMZ is established as first-line chemotherapeutic treatment of APT/PC. Clinically functioning tumours, low MGMT and concurrent radiotherapy were associated with a better response. The limited long-term effect of TMZ and the poor efficacy of other drugs highlight the need to identify additional effective therapies.