An important debate is simmering within the pituitary field – a proposed name change from pituitary adenoma to pituitary neuroendocrine tumour (PitNET). The primary controversy relates to use of the term “tumour” and concern about invoking undue anxiety within patients given that the majority of “tumours” are slow-growing and do not metastasise. Pituitary carcinomas (PC) are exceedingly rare, accounting for 0.2% of all pituitary tumours. However, 5-10% do behave in an aggressive manner, causing significant morbidity and mortality rates similar to PC, such that terms like “tumours with malignant potential” or even “carcinoma in situ” have been proposed for aggressive pituitary tumours (APT). The diagnosis of APT is a clinical one and defined in the 2018 European Society of Endocrinology Clinical Practice Guidelines as an invasive tumour with an unusually rapid tumour growth rate or clinically relevant growth despite optimal standard therapies1. This talk will outline important clinical, radiological and pathological features of APT and PC. Over the last decade significant advances in the management of these tumours has emerged. Temozolomide remains the first-line chemotherapy with second line therapy options including immune checkpoint inhibitors, anti-VEGF and other targeted therapies as well as peptide receptor radionuclide therapy. Timing of radiotherapy with oncological therapies is increasingly important. Many challenges remain such as patient selection, duration of therapy and predicting response to therapeutic options. Where available, tumour molecular testing can help guide management. All patients with APT and PC should be managed within an experienced pituitary multidisciplinary team.
1 Raverot G, Burman P, McCormack A, Heaney A, Petersenn S, Popovic V et al. European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas. Eur J Endocrinol. 2018;178(3):265-76.