Michaela Kuhlen, Marina Kunstreich, Desiree Dunstheimer, Niklas Aumann, Kerstin Lorenz, Markus Luster, Kurt W Schmid, Antje Redlich
{"title":"Autoimmune thyroiditis in paediatric thyroid cancer: clinical features and outcomes.","authors":"Michaela Kuhlen, Marina Kunstreich, Desiree Dunstheimer, Niklas Aumann, Kerstin Lorenz, Markus Luster, Kurt W Schmid, Antje Redlich","doi":"10.1093/ejendo/lvaf133","DOIUrl":"10.1093/ejendo/lvaf133","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate clinical characteristics and survival outcomes of paediatric differentiated thyroid carcinoma (paedDTC) in patients with and without autoimmune thyroiditis (AIT).</p><p><strong>Design: </strong>Retrospective cohort analysis of paedDTC patients with and without AIT, prospectively enrolled in the German Malignant Endocrine Tumour Registry between 1997 and 2024.</p><p><strong>Methods: </strong>We analysed data from paediatric patients (<18 years) with histopathologically confirmed DTC, stratified by AIT status. Variables included demographics, tumour characteristics, lymph node involvement, and metastasis. Survival outcomes were assessed using Kaplan-Meier estimates.</p><p><strong>Results: </strong>Among 401 evaluable patients, 16.0% had AIT. AIT patients were significantly older at diagnosis (P < .001) and showed a non-significant tendency toward higher female predominance (P = .098). Patients with AIT demonstrated significantly lower rates of distant metastasis (7.5% vs. 22.2%, P = .014) compared with those without AIT. No significant differences were observed in tumour size, capsule invasion, lymphatic or vascular invasion, soft tissue infiltration, or multifocality. A tendency toward improved event-free survival (EFS) was observed in AIT patients (87.5% vs. 72.1%, P = .255), while overall survival (OS) was comparable (91.8% vs. 86.0%, P = .167) during the study period. Among patients with known AIT history, paedDTC was significantly more frequently diagnosed through surveillance compared with non-AIT patients (44.2% vs. 28.3%, P = .022).</p><p><strong>Conclusions: </strong>AIT was present in 16% of paedDTC patients and was associated with reduced distant metastasis. A tendency toward improved EFS but similar OS during the follow-up period was observed. Earlier detection through surveillance in AIT patients may contribute to these findings, warranting further investigation into underlying biological mechanisms.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"48-55"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144511720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fabio Bioletto, Caroline Bogeat, Maxime Barat, Nesrine Benanteur, Laurence Guignat, Mirella Hage, Cyril Garcia, Valentin Calugaru, Julian Jacob, Jennifer Arrondeau, Lionel Groussin, Xavier Bertagna, Jérôme Bertherat, Chiara Villa, Anne Jouinot, Bertrand Baussart, Guillaume Assié
{"title":"Progression of potentially aggressive pituitary neuroendocrine tumors after radiotherapy: risk factors, management, and outcomes.","authors":"Fabio Bioletto, Caroline Bogeat, Maxime Barat, Nesrine Benanteur, Laurence Guignat, Mirella Hage, Cyril Garcia, Valentin Calugaru, Julian Jacob, Jennifer Arrondeau, Lionel Groussin, Xavier Bertagna, Jérôme Bertherat, Chiara Villa, Anne Jouinot, Bertrand Baussart, Guillaume Assié","doi":"10.1093/ejendo/lvaf136","DOIUrl":"10.1093/ejendo/lvaf136","url":null,"abstract":"<p><strong>Objective: </strong>Radiotherapy plays a relevant role in uncontrolled pituitary neuroendocrine tumors (PitNETs). Radiotherapy controls tumor progression in most cases, but not always. Prognostic factors for tumor progression after radiotherapy remain poorly defined. The aim was to evaluate tumor progression after radiotherapy, to identify risk factors, and to report management and outcomes in a cohort of PitNETs with uncontrolled progression.</p><p><strong>Design: </strong>This was a retrospective, single-center, observational study.</p><p><strong>Methods: </strong>In total, 123 consecutive patients who underwent radiotherapy for PitNETs and were followed at Cochin Hospital between 2000 and 2022 were included. Indication for radiotherapy was uncontrolled tumor progression (80%), adjuvant (9%), or uncontrolled secretion (11%). The median follow-up after radiotherapy was 10.0 years.</p><p><strong>Results: </strong>Tumor progression after radiotherapy was observed in 28/123 (23%) patients. Higher risk of progression was associated with lactotroph and corticotroph tumor types (HR [95% CI] 12.0 [1.2-117.1] and 9.3 [1.3-69.6], respectively), male sex (3.7 [1.6-8.4]), and necrotic-hemorrhagic changes before radiotherapy on magnetic resonance imaging (3.1 [1.1-8.4]). Surgery, temozolomide, and re-irradiation were the most frequent treatments for the management of patients with tumor progression after radiotherapy, used in 18/28(64%), 16/28(57%), and 8/28(29%) cases, respectively. The most common complication of radiotherapy was the new onset of pituitary deficits, observed in 41% of cases; other complications, including radiation-induced neuroinflammation, cerebrovascular events, and second brain tumors, were rare. Three patients developed metastases, and 6 patients died because of tumor progression.</p><p><strong>Conclusions: </strong>Lactotroph and corticotroph PitNETs, in male patients, and/or with necrotic-hemorrhagic changes are at higher risk of progression after radiotherapy. Patients with progression after radiotherapy require additional heavy treatments with variable outcome.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"167-178"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Paula Casano-Sancho, Paula-Ximena Molina, Anna Valls, Hector Salvador
{"title":"Growth hormone excess in children with neurofibromatosis and optic pathway glioma, an underdiagnosed condition: experience with long-acting treatment.","authors":"Paula Casano-Sancho, Paula-Ximena Molina, Anna Valls, Hector Salvador","doi":"10.1093/ejendo/lvaf113","DOIUrl":"10.1093/ejendo/lvaf113","url":null,"abstract":"<p><strong>Background: </strong>Growth hormone excess (GHE) in children with neurofibromatosis Type 1 (NF1) has been reported in some cases. The prevalence of GHE in NF1 has not been described and treatment with long-acting somatostatin analogues (SSAs) has not been well characterized.</p><p><strong>Objective: </strong>To describe in children with NF1/optic pathway glioma (OPG) the prevalence of GHE and response to SSA.</p><p><strong>Results: </strong>From 379 children with NF1, 80 were diagnosed of OPG (21%). In a prospective follow-up, 8.7% were identified as having GHE; all were prepubertal, with a mean age of 4.4 ± 1.9 years. The mean height was +0.86 ± 0.76 SD above the mid-parental height and growth velocity +4.07 SD, mean insulin-like growth factor 1 (IGF-1) > 1 SD (457.8 ± 151.3 ng/mL). In 4 patients, the GHE was observed during tumour progression. The first 2 patients were initially treated with short-acting SSAs (1.5 μg/kg/day). After confirming tolerability, it was replaced by long-acting SSAs (10 mg/28 days). Four were initially treated with (10 mg/28 d). 6/7 patients showed a normalization of IGF-1 and growth velocity. Treatment could be withdrawn in all patients after 19.9 ± 4.6 months. The patients remained stable for 48 months (24-60). Except for mild diarrhoea, no other adverse events were observed.</p><p><strong>Conclusions: </strong>We should consider the risk GHE in patients with NF1-OPG, as this may be a cause for concern indicating tumour progression. Treatment with long-acting SSAs was effective and safe. After treatment, the patients' growth and analytical parameters remained within normal range, confirming the reversibility of GHE.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"129-134"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144224713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fidéline Bonnet-Serrano, Louis Thomeret, Nesrine Benanteur, Patricia Vaduva, Florian Violon, Lucas Bouys, Bruno Ragazzon, Annabel Berthon, Karine Perlemoine, Hortense Wilmot-Roussel, Corinne Zientek, Samir Nakib, Martin Gaillard, Mathilde Sibony, Christelle Laguillier-Morizot, Marie-Claude Menet, Laurence Guignat, Rossella Libe, Lionel Groussin, Jean Guibourdenche, Anne Jouinot, Guillaume Assié, Jérôme Bertherat
{"title":"Unexpected discrepancies between steroid intra-tissular content in adrenal tumors and clinical diagnosis of steroid excess.","authors":"Fidéline Bonnet-Serrano, Louis Thomeret, Nesrine Benanteur, Patricia Vaduva, Florian Violon, Lucas Bouys, Bruno Ragazzon, Annabel Berthon, Karine Perlemoine, Hortense Wilmot-Roussel, Corinne Zientek, Samir Nakib, Martin Gaillard, Mathilde Sibony, Christelle Laguillier-Morizot, Marie-Claude Menet, Laurence Guignat, Rossella Libe, Lionel Groussin, Jean Guibourdenche, Anne Jouinot, Guillaume Assié, Jérôme Bertherat","doi":"10.1093/ejendo/lvaf129","DOIUrl":"10.1093/ejendo/lvaf129","url":null,"abstract":"<p><strong>Objective: </strong>Adrenocortical tumor (ACT) morbidity is associated with steroid secretion, depending on tumor type. Indeed, adrenal steroidogenesis is a finely regulated process, altered in ACT. These alterations are usually characterized by blood steroid assays, also depending on steroid gonadal production and metabolism. Our aim was to determine steroid content directly in ACT tissues and to compare it with clinical diagnosis of steroid excess.</p><p><strong>Methods: </strong>A profile of 13 steroids was analyzed in ultra-high-performance liquid chromatography coupled to tandem mass spectrometry (Thermo Fisher Scientific®) in frozen tissue samples from 75 ACT, 7 Cushing's disease, and 9 normal adrenals.</p><p><strong>Results: </strong>Steroid levels were 10-1000 times higher in tissue from normal adrenal than normal concentrations expected in blood. Concentration ratios between tissue from normal adrenal and blood reference values were lower for distal products than for steroid precursors. In adrenocortical cancers, intra-tissular steroid content was lower than in unilateral benign tumors despite clear clinical steroid excess. Unexpectedly, in overt-Cushing adenomas, intra-tissular cortisol levels were not higher and androstenedione levels were not lower than in non-functioning adenomas. Adrenal differentiation score based on transcriptome was well correlated with intra-tissular cortisol levels.</p><p><strong>Conclusion: </strong>Discrepancies observed between steroid levels measured in ACT tissue and clinical diagnosis of steroid excess suggest a dysregulation of steroid export depending on tumor type, opening new perspectives for diagnosis and treatment of steroid excess.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"106-116"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Linus Haberbosch, Christian J Strasburger, Lukas Maurer, Knut Mai
{"title":"Diagnostic strategies for central adrenal insufficiency in clinical practice: authors' response.","authors":"Linus Haberbosch, Christian J Strasburger, Lukas Maurer, Knut Mai","doi":"10.1093/ejendo/lvaf126","DOIUrl":"10.1093/ejendo/lvaf126","url":null,"abstract":"","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"L3-L4"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293565","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eu Jeong Ku, Won Sang Yoo, Yu Been Hwang, Subin Jang, Jooyoung Lee, Shinje Moon, Eun Kyung Lee, Hwa Young Ahn
{"title":"Osteoporotic fracture risk in hypoparathyroidism following total thyroidectomy: a retrospective nationwide cohort study.","authors":"Eu Jeong Ku, Won Sang Yoo, Yu Been Hwang, Subin Jang, Jooyoung Lee, Shinje Moon, Eun Kyung Lee, Hwa Young Ahn","doi":"10.1093/ejendo/lvaf128","DOIUrl":"10.1093/ejendo/lvaf128","url":null,"abstract":"<p><strong>Objective: </strong>Hypoparathyroidism is an uncommon but significant complication of total thyroidectomy. As the incidence of thyroid cancer increases, the prevalence of hypoparathyroidism also increases. However, its effect on the risk of osteoporotic fractures remains poorly understood. We aimed to evaluate the risk of osteoporotic fractures in patients with postoperative hypoparathyroidism compared with that in a matched control group.</p><p><strong>Design: </strong>This retrospective cohort study included 417 patients with hypoparathyroidism who had undergone total thyroidectomy for thyroid cancer (TC with hypoP) and 2085 matched controls from the Korean National Health Insurance Service-National Sample Cohort.</p><p><strong>Methods: </strong>The osteoporotic fracture rates and associated risk factors were analyzed in comparison after propensity score matching.</p><p><strong>Results: </strong>During a median follow-up period of 5.07 years (interquartile range 2.19-7.38), postoperative hypoparathyroidism was not associated with a significantly greater risk of osteoporotic fractures than that in the control group (hazard ratio [HR] 0.86, 95% confidence interval [CI], 0.59-1.24, P = .406). Age-specific analysis showed a trend toward higher fracture incidence in patients aged <50 years (HR 1.31, 95% CI, 0.53-3.22, P = .558) and a decreasing trend in those aged ≥50 years (HR 0.75, 95% CI, 0.51-1.12, P = .163), although the differences were not statistically significant. No significant differences in fracture risk were observed between the TC with and without hypoP groups across all fracture sites. The frequency of osteoporotic medication use was higher in the TC with hypoP group than in the control group, particularly among women aged <50 years.</p><p><strong>Conclusions: </strong>Hypoparathyroidism after total thyroidectomy does not appear to increase the risk of osteoporotic fractures compared with that in matched controls in clinical practice.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"39-47"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144505223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Charmaine Ter, Xuan Han Koh, Hieu Tran, Irina Bancos, Mohamed Bassiony, Marta Araujo-Castro, Miguel Paja, Marga González Boillos, Eleftheria Gkaniatsa, Martin Reincke, Christian Adolf, Thang Viet Tran, Michael Stowasser, Drishya Nayak, Marianne A Grytaas, Adina F Turcu, Joanna Matrozova, Norlela Sukor, Farhana Ismail, Tomaz Kocjan, Mirko Parasiliti-Caprino, Rene Baudrand, Thomas Uslar, Mika Tsuiki, Masanori Murakami, Jun Yang, Chrislyn Ng, Takuyuki Katabami, Mitsuhide Naruse, Matthieu St-Jean, Filippo Ceccato, Seyed Ehsan Saffari, Ada E D Teo, Troy H Puar
{"title":"A global real-world study assessing total time to adrenalectomy in primary aldosteronism.","authors":"Charmaine Ter, Xuan Han Koh, Hieu Tran, Irina Bancos, Mohamed Bassiony, Marta Araujo-Castro, Miguel Paja, Marga González Boillos, Eleftheria Gkaniatsa, Martin Reincke, Christian Adolf, Thang Viet Tran, Michael Stowasser, Drishya Nayak, Marianne A Grytaas, Adina F Turcu, Joanna Matrozova, Norlela Sukor, Farhana Ismail, Tomaz Kocjan, Mirko Parasiliti-Caprino, Rene Baudrand, Thomas Uslar, Mika Tsuiki, Masanori Murakami, Jun Yang, Chrislyn Ng, Takuyuki Katabami, Mitsuhide Naruse, Matthieu St-Jean, Filippo Ceccato, Seyed Ehsan Saffari, Ada E D Teo, Troy H Puar","doi":"10.1093/ejendo/lvaf124","DOIUrl":"10.1093/ejendo/lvaf124","url":null,"abstract":"<p><strong>Background: </strong>Primary aldosteronism (PA) is a common treatable cause of hypertension. When caused by unilateral adrenal disease, it is potentially curable by adrenalectomy. However, specialized tests and other factors may delay definitive treatment. We assessed the time to adrenalectomy (TTA) for patients worldwide.</p><p><strong>Methods: </strong>We conducted an international, multicentre retrospective study involving 39 centres from 15 countries to determine the total time taken from the first presentation to adrenalectomy and the intervals between each stage (screening, confirmatory, subtyping, and adrenalectomy). We included patients with PA who underwent adrenalectomy from January 1, 2018, to October 30, 2022. Post-adrenalectomy outcomes were evaluated using the Primary Aldosteronism Surgery Outcome criteria. We performed multivariable quantile and linear regression to identify characteristics associated with longer TTA.</p><p><strong>Results: </strong>We included 861 patients, mean age 49.3 ± 11.1 years, and 44.5% were women. Overall median TTA was 13.5 months, IQR: 6.6-24.5. Median intervals were 0.1 months (screening), 1.0 months (confirmatory), 4.1 months (subtyping), and 4.3 months (adrenalectomy). On multivariable analysis, median TTA was increased by 5.4 months for each additional adrenal vein sampling (AVS) procedure. Other factors associated with longer TTA included adrenalectomy post-COVID-19, younger age, and additional screening tests. Compared with countries with routine AVS, those without AVS had a shorter TTA (6.1 vs 15.1 months, P < .001), but greater likelihood of absent/partial biochemical success post-adrenalectomy (27.4% vs 12.4%, P < .001).</p><p><strong>Conclusion: </strong>Primary aldosteronism management is time-consuming worldwide, especially for subtyping tests and adrenalectomy. While omitting AVS reduces overall time, patients are less likely to achieve biochemical cure post-adrenalectomy.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":" ","pages":"65-75"},"PeriodicalIF":5.3,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12224190/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144505220","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jinting Zhou, Menglin Fan, Aaron M Lett, Geling Jin, Qiqi You, Jingjing Zeng, Bo Chen, Yucen Wu, Hui Xing, Shaoyong Xu
{"title":"Association between premature ovarian insufficiency and biological aging.","authors":"Jinting Zhou, Menglin Fan, Aaron M Lett, Geling Jin, Qiqi You, Jingjing Zeng, Bo Chen, Yucen Wu, Hui Xing, Shaoyong Xu","doi":"10.1093/ejendo/lvaf102","DOIUrl":"https://doi.org/10.1093/ejendo/lvaf102","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to analyze whether premature ovarian insufficiency (POI) is associated with accelerated biological aging, whether the degree of biological aging is exacerbated by an earlier age at menopause, and whether menopausal hormone therapy (MHT) in the POI population is associated with reduced biological aging.</p><p><strong>Design: </strong>This is a cross-sectional study. A total of 229 779 participants aged 40 years and older in the UK Biobank (2006-2010) and NHANES (1999-2018) were included in the study.</p><p><strong>Methods: </strong>Menopause information was collected through questionnaires. Biological age acceleration was defined by the Klemera-Doubal method, which is calculated through biomarkers, in reference to chronological age. Biological age acceleration > 0 was defined as biological aging. Association between POI and biological aging analyzed using multivariate linear regression and logistic regression models.</p><p><strong>Results: </strong>The results showed that participants with POI had an increased risk of biological aging (UK Biobank: OR = 1.50 [95% CI: 1.24-1.82]; NHANES: OR = 1.20 [95% CI: 1.07-1.34]) and decrease in leukocyte telomere length compared with those without POI (UK Biobank: 0.0109 [95% CI: 0.0079-0.0109]). Participants with POI who underwent MHT had reduced risk of aging compared with those who did not (UK Biobank: OR = 0.63 [95% CI: 0.43-0.92]; NHANES: OR = 0.75 [95% CI: 0.61-0.92]).</p><p><strong>Conclusion: </strong>This study showed that participants with POI had a significantly increased risk of biological aging compared with those without POI. Participants with POI who received MHT had a reduced risk of aging compared with those who did not.</p>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 6","pages":"744-753"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144247071","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gerald Raverot, Pia Burman, Ana Paula Abreu, Anthony P Heaney, Leonie van Hulsteijn, Andrew L Lin, Hani Marcus, Ann McCormack, Giuseppe Minniti, Stephan Petersenn, Vera Popovic, Marily Theodoropoulou, Jacqueline Trouillas, Olaf M Dekkers
{"title":"Revised European Society of Endocrinology Clinical Practice Guideline for the management of aggressive pituitary tumours and pituitary carcinomas.","authors":"Gerald Raverot, Pia Burman, Ana Paula Abreu, Anthony P Heaney, Leonie van Hulsteijn, Andrew L Lin, Hani Marcus, Ann McCormack, Giuseppe Minniti, Stephan Petersenn, Vera Popovic, Marily Theodoropoulou, Jacqueline Trouillas, Olaf M Dekkers","doi":"10.1093/ejendo/lvaf100","DOIUrl":"10.1093/ejendo/lvaf100","url":null,"abstract":"<p><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>","PeriodicalId":11884,"journal":{"name":"European Journal of Endocrinology","volume":"192 6","pages":"R45-R78"},"PeriodicalIF":5.3,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144283043","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}