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Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas

Raverot, Gerald ; Burman, Pia LU ; Abreu, Ana Paula ; Heaney, Anthony P. ; Van Hulsteijn, Leonie ; Lin, Andrew L. ; Marcus, Hani ; McCormack, Ann ; Minniti, Giuseppe and Petersenn, Stephan , et al. (2025) In European Journal of Endocrinology 192(6). p.45-78
Abstract

Pituitary tumours, originating from endocrine cells of the anterior pituitary, are quite common, and in most cases well-controlled by surgery or medical treatment. However, a small subset of pituitary tumours presents with multiple local recurrences or tumour progression despite combined surgical, medical or radiotherapeutic treatment. These are known as aggressive pituitary tumours (APT); also called aggressive pituitary neuroendocrine tumours (PitNETs); or, in the rare case of metastases, pituitary carcinomas (PC) or metastatic PitNETs. Early identification of APT is challenging but is of major clinical importance as they are associated with an increased morbidity and mortality even in the absence of metastases. Here, we provide a... (More)

Pituitary tumours, originating from endocrine cells of the anterior pituitary, are quite common, and in most cases well-controlled by surgery or medical treatment. However, a small subset of pituitary tumours presents with multiple local recurrences or tumour progression despite combined surgical, medical or radiotherapeutic treatment. These are known as aggressive pituitary tumours (APT); also called aggressive pituitary neuroendocrine tumours (PitNETs); or, in the rare case of metastases, pituitary carcinomas (PC) or metastatic PitNETs. Early identification of APT is challenging but is of major clinical importance as they are associated with an increased morbidity and mortality even in the absence of metastases. Here, we provide a revision of the first international, interdisciplinary European Society of Endocrinology (ESE) clinical practice guideline on APTs and PC (2018). Since publication of the 2018 guideline, results from the second ESE survey on APT and PC were published, and more data on APT treatment, including temozolomide, immune checkpoint inhibitors and bevacizumab, emerged. These data are reviewed in this guideline and translated into a practical algorithm to guide APT and PC management. Furthermore, standardized reporting of imaging and histopathological investigations of these tumours is proposed, and the role of molecular analysis is discussed. Last, a section is dedicated to special circumstances such as APT in pregnancy.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
aggressive pituitary tumour-Pituitary carcinoma, pituitary adenoma, prognosis, therapy
in
European Journal of Endocrinology
volume
192
issue
6
pages
45 - 78
publisher
Society of the European Journal of Endocrinology
external identifiers
  • scopus:105008391404
  • pmid:40506054
ISSN
0804-4643
DOI
10.1093/ejendo/lvaf100
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2025 The Author(s). Published by Oxford University Press on behalf of European Society of Endocrinology.
id
35e99304-7915-4ef4-8584-4bc46e39ac2b
date added to LUP
2025-12-29 13:18:25
date last changed
2025-12-29 13:19:07
@article{35e99304-7915-4ef4-8584-4bc46e39ac2b,
  abstract     = {{<p>Pituitary tumours, originating from endocrine cells of the anterior pituitary, are quite common, and in most cases well-controlled by surgery or medical treatment. However, a small subset of pituitary tumours presents with multiple local recurrences or tumour progression despite combined surgical, medical or radiotherapeutic treatment. These are known as aggressive pituitary tumours (APT); also called aggressive pituitary neuroendocrine tumours (PitNETs); or, in the rare case of metastases, pituitary carcinomas (PC) or metastatic PitNETs. Early identification of APT is challenging but is of major clinical importance as they are associated with an increased morbidity and mortality even in the absence of metastases. Here, we provide a revision of the first international, interdisciplinary European Society of Endocrinology (ESE) clinical practice guideline on APTs and PC (2018). Since publication of the 2018 guideline, results from the second ESE survey on APT and PC were published, and more data on APT treatment, including temozolomide, immune checkpoint inhibitors and bevacizumab, emerged. These data are reviewed in this guideline and translated into a practical algorithm to guide APT and PC management. Furthermore, standardized reporting of imaging and histopathological investigations of these tumours is proposed, and the role of molecular analysis is discussed. Last, a section is dedicated to special circumstances such as APT in pregnancy.</p>}},
  author       = {{Raverot, Gerald and Burman, Pia and Abreu, Ana Paula and Heaney, Anthony P. and Van Hulsteijn, Leonie and Lin, Andrew L. and Marcus, Hani and McCormack, Ann and Minniti, Giuseppe and Petersenn, Stephan and Popovic, Vera and Theodoropoulou, Marily and Trouillas, Jacqueline and Dekkers, Olaf M.}},
  issn         = {{0804-4643}},
  keywords     = {{aggressive pituitary tumour-Pituitary carcinoma; pituitary adenoma; prognosis; therapy}},
  language     = {{eng}},
  month        = {{06}},
  number       = {{6}},
  pages        = {{45--78}},
  publisher    = {{Society of the European Journal of Endocrinology}},
  series       = {{European Journal of Endocrinology}},
  title        = {{Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas}},
  url          = {{http://dx.doi.org/10.1093/ejendo/lvaf100}},
  doi          = {{10.1093/ejendo/lvaf100}},
  volume       = {{192}},
  year         = {{2025}},
}