Aggressive pituitary tumors and pituitary carcinomas : from pathology to treatment
(2023) In The Journal of clinical endocrinology and metabolism 108(7). p.1585-1601- Abstract
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... (More)
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.
(Less)
- author
- Burman, Pia LU ; Casar-Borota, Olivera ; Perez-Rivas, Luis Gustavo and Dekkers, Olaf M
- organization
- publishing date
- 2023-02-28
- type
- Contribution to journal
- publication status
- published
- subject
- in
- The Journal of clinical endocrinology and metabolism
- volume
- 108
- issue
- 7
- pages
- 1585 - 1601
- publisher
- Oxford University Press
- external identifiers
-
- scopus:85165496907
- pmid:36856733
- ISSN
- 1945-7197
- DOI
- 10.1210/clinem/dgad098
- language
- English
- LU publication?
- yes
- additional info
- © The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.
- id
- a9cef62e-4f64-4214-9aa8-9c770be3e248
- date added to LUP
- 2023-03-02 09:59:52
- date last changed
- 2024-06-14 05:48:42
@article{a9cef62e-4f64-4214-9aa8-9c770be3e248,
abstract = {{<p>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.</p>}},
author = {{Burman, Pia and Casar-Borota, Olivera and Perez-Rivas, Luis Gustavo and Dekkers, Olaf M}},
issn = {{1945-7197}},
language = {{eng}},
month = {{02}},
number = {{7}},
pages = {{1585--1601}},
publisher = {{Oxford University Press}},
series = {{The Journal of clinical endocrinology and metabolism}},
title = {{Aggressive pituitary tumors and pituitary carcinomas : from pathology to treatment}},
url = {{http://dx.doi.org/10.1210/clinem/dgad098}},
doi = {{10.1210/clinem/dgad098}},
volume = {{108}},
year = {{2023}},
}