Treatment of aggressive pituitary tumours and carcinomas: results of a European Society of Endocrinology (ESE) survey 2016

Ann McCormack(Garvan Institute of Medical Research), Olaf M Dekkers(Leiden University), Stephan Petersenn, Vera Popović(University of Belgrade), Jacqueline Trouillas(Hospices Civils de Lyon), Gérald Raverot(Université Claude Bernard Lyon 1), Pia Burman(Lund University), _ _, _ _, Alicia Hubalewska-Dydejezky(University of Padua), Guillaume Assié(University of Padua), Leon A. Bach(University of Padua), Marie Batisse‐Lignier(University of Padua), Katarina Berinder(University of Padua), Ismene Bilbao(University of Padua), Fabrice Bonnet(University of Padua), Damien Bresson(University of Padua), Oscar D. Bruno(University of Padua), Mariana Campderá(University of Padua), Philippe Caron(University of Padua), Frédéric Castinetti(University of Padua), Filippo Ceccato(University of Padua), Olivier Chabre(University of Padua), Philippe Chanson(University of Padua), Emanuel Christ(University of Padua), Lucie Cloix(University of Padua), Christine Cortet(University of Padua), Lise Crinière(University of Padua), Guillem Cuatrecasas(University of Padua), Miguel Debono(University of Padua), Brigitte Delemer(University of Padua), Rachel Desailloud(University of Padua), Timo Deutschbein(University of Padua), Tina Dušek(University of Padua), Britt Edén Engström(University of Padua), Marco Faustini‐Fustini(University of Padua), Schillo Franck(University of Padua), C. Garcia(University of Padua), Yona Greenman(University of Padua), Susana Mallea Gil(University of Padua), Giovanna Mantovani(University of Padua), Mark Gurnell(University of Padua), Anthony P. Heaney(University of Padua), David Henley(University of Padua), Claire Higham(University of Padua), E. W. Hoving(University of Padua), Charlotte Höybye(University of Padua), Atsuhiro Ichihara(University of Padua), Marie‐Lise Jaffrain‐Rea(University of Padua), Gudmundur Johannsson(University of Padua), Jens Otto Lunde Jørgensen(University of Padua), Christel Jublanc(University of Padua), Jan Komor(University of Padua), Márta Korbonits(University of Padua), Ivana Kralievic(University of Padua), Delphine Larrieu‐Ciron(University of Padua), Hélène Lasolle(University of Padua), Edward R. Laws(University of Padua), Marco Losa(University of Padua), Dominique Maiter(University of Padua), Claudio Marcocci(University of Padua), Olinda Marques(University of Padua), Tânia Longo Mazzuco(University of Padua), Alexander Micko(University of Padua), Nathalie Bourcigaux(University of Padua), Sebastian Neggers(University of Padua), John Newell‐Price(University of Padua), Belén Perez-Berida(University of Padua), León Darío Ortiz(University of Padua), Óskar Ragnarsson(University of Padua), Marta Ragonese(University of Padua), Martín Reincke(University of Padua), Jean‐Louis Sadoul(University of Padua), Akira Shimatsu(University of Padua), Luis V. Syro(University of Padua), Luc Taillandier(University of Padua), Miklós Tóth(University of Padua), Takeshi Usui(University of Padua), Zauzsanna Valkusz(University of Padua), Greisa Vila(University of Padua), Ben Whitelaw(University of Padua), Maria Chiara Zatelli(University of Padua)
European Journal of Endocrinology
January 12, 2018
10.1530/eje-17-0933
Cited by 277Open Access
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Abstract

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.

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