Aggressive pituitary tumors and pituitary carcinomas: from pathology to treatment

Pia Burman, Olivera Casar-Borota, Luis Gustavo Perez-Rivas, Olaf M Dekkers

Research output: Contribution to journalArticlepeer-review


Aggressive pituitary tumors (APT) and pituitary carcinomas (PC) are heterogeneous with regard to clinical presentation, proliferative markers, clinical course and response to therapy. Half of them show an aggressive course only many years after the first apparently benign presentation. APT and PC share several properties, but Ki67 index ≥10% and extensive p53 expression are more prevalent in PCs. Mutations in TP53 and ATRX are the most common genetic alterations, their detection might be of value for early identification of aggressiveness. Treatment requires a multimodal approach including surgery, radiotherapy, and drugs. Temozolomide (TMZ) is the recommended first line chemotherapy, with response rates of about 40%. Immune checkpoint inhibitors have emerged as second line treatment in PCs, with currently no evidence for a superior effect of dual therapy compared to monotherapy with PD-1 blockers. Bevacizumab has resulted in partial response (PR) in few patients, tyrosine kinase inhibitors and everolimus have generally not been useful. The effect of peptide receptor radionuclide therapy is limited as well. Management of APT/PC is challenging and should be discussed within an expert-team with consideration of clinical and pathological findings, age and general condition of the patient. Considering that APT/PCs are rare, new therapies should preferably be evaluated in shared standardized protocols. Prognostic and predictive markers to guide treatment decisions are needed and are scope of ongoing research.

Original languageEnglish
Pages (from-to)1585-1601
JournalThe Journal of clinical endocrinology and metabolism
Issue number7
Early online date2023 Feb 28
Publication statusPublished - 2023

Subject classification (UKÄ)

  • Clinical Laboratory Medicine


Dive into the research topics of 'Aggressive pituitary tumors and pituitary carcinomas: from pathology to treatment'. Together they form a unique fingerprint.

Cite this