{"title":"Letter to the editor: Long-term safety and efficacy of pegvisomant monotherapy for acromegaly: final data from the full ACROSTUDY cohort.","authors":"Neslihan Koray Fedai, Ilknur Ozturk Unsal, Erman Cakal","doi":"10.1007/s11102-026-01687-5","DOIUrl":"10.1007/s11102-026-01687-5","url":null,"abstract":"","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147841814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Decision-making and impulsivity in cabergoline-treated prolactinoma: findings from Iowa Gambling, Go/No-Go, and barratt scales.","authors":"Püren Gökbulut, Hüseyin Nergiz, Hatice Ceren Tırış, Işılay Taşkaldıran, Volkan Gökbulut, Şerife Mehlika Kuşkonmaz","doi":"10.1007/s11102-026-01662-0","DOIUrl":"10.1007/s11102-026-01662-0","url":null,"abstract":"<p><p>PURPOSE: To assess impulsivity, decision-making performance and inhibitory control in prolactinoma patients treated with dopamine agonists (DA), and to compare these parameters with healthy control(HC) subjects. METHODS: A total of 34 patients with prolactinoma, and 35 HCs were included in the study. All patients and controls were screened using demographic information questionnaire, Barratt Impulsiveness Scale–Short Form (BIS-11-SF), Iowa Gambling Task and Go/No-Go Task. Clinical data of all subjects were collected from their electronic medical records. RESULTS: Compared with healthy controls, patients with prolactinoma receiving dopamine agonist therapy showed a significantly higher frequency of gambling urges and sexual desire during the preceding four weeks (p = 0.003 for both). In the Go/No-Go Task, a significant between-group difference was observed in commission error rates (p = 0.041). Treatment duration was positively correlated with both the frequency of sexual desire over the past four weeks and the non-planning subscale of the BIS-11-SF (r = 0.364, p = 0.035; r = 0.429, p = 0.011, respectively). Cumulative dopamine agonist dose was positively correlated with the frequency of sexual desire, the frequency of sexual intercourse attempts over the past four weeks, and the non-planning subscale of the BIS-11-SF (r = 0.405, p = 0.018; r = 0.433, p = 0.011; and r = 0.418, p = 0.014, respectively). CONCLUSIONS: In patients with prolactinoma, dopamine agonist treatment is associated with selective alterations in decision-making–related impulsivity and inhibitory control, influenced by treatment duration and cumulative dopamine agonist exposure. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-29DOI: 10.1007/s11102-026-01679-5
Ryan Beerling Dolovac, Ann McCormack, Lachlan R Evans, James A King, Christopher Ovenden, Jeremy Kam, Tony Goldschlager, Mendel Castle-Kirszbaum
{"title":"Pituitary stalk biopsy - A systematic review of the safety and efficacy of stalk lesion biopsy.","authors":"Ryan Beerling Dolovac, Ann McCormack, Lachlan R Evans, James A King, Christopher Ovenden, Jeremy Kam, Tony Goldschlager, Mendel Castle-Kirszbaum","doi":"10.1007/s11102-026-01679-5","DOIUrl":"10.1007/s11102-026-01679-5","url":null,"abstract":"<p><p>INTRODUCTION: A myriad of pathological process may involve the pituitary stalk (infundibulum), and despite extensive investigations, biopsy is often required. Due to the rarity of the procedure, the risks and yield of stalk biopsy are not well known, with important implications for clinical decision making. METHODS: A systematic review was performed in accordance with the PRISMA statement. Studies that reported diagnostic yield and complication rates after stalk biopsy were included. Bias was assessed using ROBINS-V2. RESULTS: A total of 13 studies, including 832 patients and 406 biopsies were included. Mean time from diagnosis to biopsy was 11 months. Preoperative visual symptoms were seen in 29.1%, AVP-deficiency (diabetes insipidus) in 71.0%, and hypopituitarism in 66.3%. Biopsy was predominately performed through the endoscopic transsphenoidal approach (70.9%). Diagnostic yield was 95.8%, and complications included postoperative CSF leak (1.6%), visual worsening (1.6%), new cranial nerve palsy (1.2%), new permanent AVP-deficiency (9.0%), new hypopituitarism (15.7%), and meningitis (2.7%). CONCLUSION: Pituitary stalk biopsy is a high yield and safe diagnostic test, with low periprocedural morbidity. The rate of new endocrinopathy compares favourably to the natural history of progressive stalk disease. Stalk biopsy could be considered earlier in the workup of undifferentiated progressive or symptomatic stalk lesions to avoid unnecessary delays in diagnosis and treatment. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13124891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-29DOI: 10.1007/s11102-026-01676-8
Fung Kuen Tam, Hiu Yan Chan, Ming Him Yuen, Hoi Kwan Calvin Mak, Mei Tik Stella Leung, Choi Yee Lau, Po Shan Judy Lai, Pak Lam Sammy Chen, Chi Chung Shek, Sze Wai Karen Lee, Yew Chuan David Chong, Chiu Ming Ng, Cheung Hei Choi
{"title":"COPeptin Evaluation in argininevasopressin Deficiency post transsphenoidal surgery (COPED study).","authors":"Fung Kuen Tam, Hiu Yan Chan, Ming Him Yuen, Hoi Kwan Calvin Mak, Mei Tik Stella Leung, Choi Yee Lau, Po Shan Judy Lai, Pak Lam Sammy Chen, Chi Chung Shek, Sze Wai Karen Lee, Yew Chuan David Chong, Chiu Ming Ng, Cheung Hei Choi","doi":"10.1007/s11102-026-01676-8","DOIUrl":"10.1007/s11102-026-01676-8","url":null,"abstract":"<p><p>PURPOSE: Copeptin is an emerging biomarker for polyuria-polydipsia syndrome. Postoperative copeptin levels may predict acquired arginine vasopressin deficiency (AVP-D). This prospective study evaluated the diagnostic performance of serum copeptin for predicting postoperative AVP-D after transsphenoidal surgery (TSS). METHODS: Copeptin was measured preoperatively, immediately after surgery (periextubation, T0), upon first ward arrival (T1), daily until discharge, and at week 2. Delta copeptin was calculated as the percentage change from T0 to T1. RESULTS: Of 53 patients, 23 (43.4%) developed AVP-D, including 4 (7.5%) permanent and 19 (35.8%) transient cases. The AVP-D group showed significantly lower delta copeptin (median -38% vs. +66%, p < 0.001), day 2 (median 3.1 vs. 4.0 pmol/L, p = 0.021), and week 2 levels (median 2.5 vs. 3.2 pmol/L, p = 0.021) compared with the non-AVP-D group. Delta copeptin (area under the curve 0.826, p = 0.001) demonstrated the best diagnostic performance for AVP-D with an optimal cutoff of < -10% (sensitivity 100%, specificity 76.2%). All AVP-D patients exhibited negative delta copeptin. Week 2 copeptin (area under the curve 0.891, p = 0.011) predicted permanent AVP-D best with an optimal cutoff of < 2.6 pmol/L (sensitivity 100%, specificity 66.7%). CONCLUSIONS: Delta copeptin—the change in copeptin levels from T0 (periextubation) to T1 (2–4 h post-surgery)—predicted AVP-D after TSS. We recommend measuring copeptin immediately after surgery and repeat at 2–4 h. Patients with positive delta copeptin were unlikely to develop AVP-D. A copeptin value > 2.6 pmol/L at week 2 ruled out permanent AVP-D. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-29DOI: 10.1007/s11102-026-01683-9
Kate Spuler, Roland White, Wu Tzen Lim, Jacob Deeb, James McNeil, George Bouras, Alistair Jukes, Stephen Santoreneos, Alkis Psaltis, Sandy Patel, Sunita M C De Sousa
{"title":"A Retrospective cohort study of Knosp grade reporting of cavernous sinus invasion in pituitary macroadenomas.","authors":"Kate Spuler, Roland White, Wu Tzen Lim, Jacob Deeb, James McNeil, George Bouras, Alistair Jukes, Stephen Santoreneos, Alkis Psaltis, Sandy Patel, Sunita M C De Sousa","doi":"10.1007/s11102-026-01683-9","DOIUrl":"10.1007/s11102-026-01683-9","url":null,"abstract":"<p><p>PURPOSE: The Knosp grading system classifies the extent of cavernous sinus invasion (CSI) in pituitary adenomas (PAs). Knosp grade is predictive of surgical remission rates and is used in decision-making for the management of these adenomas. This study evaluates the rate and accuracy of Knosp grade reporting of PAs by radiologists. METHODS: This is a retrospective observational study of 100 consecutive patients with pituitary macroadenomas who underwent pituitary MR imaging between March 2023 and March 2025. The rate of CSI reporting in radiologist reports of the scans was determined, and the reported grade was compared with the Knosp grade calculated by the study panel. RESULTS: Radiologist reports contained a specific comment regarding CSI, or its absence, in 73/100 (73%) of patients. Analysing left and right cavernous sinuses as separate observations, implicit phrasing reporting a specific Knosp grade occurred in 12/200 (6%), and explicit reporting occurred in 10/200 (5%). Analysis of implicit and explicit reporting revealed a statistically significant difference between the reported Knosp grades and study grades (p = 0.02). No significant correlation was found between CSI reporting rates and patients undergoing surgery (p = 0.179), radiotherapy (p = 0.427), or medical therapy (p = 0.127) before or at the time of the MRI. CONCLUSION: Despite the integral importance of CSI in pituitary adenoma management decisions as enshrined in international guidelines, the rate and accuracy of Knosp grade reporting are suboptimal. Knosp reporting in MRI reports should be standardised to improve the rate and quality of CSI reporting. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13124751/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779103","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-29DOI: 10.1007/s11102-026-01639-z
Andrea Giustina, Luigi di Filippo, Maria Fleseriu, Rosario Pivonello, Stephan Petersenn, John Wass, Susan L Samson, Alberto M Pereira, Raúl M Luque, Betina Biagetti, Maria Chiara Zatelli, Ken K Y Ho, Cesar L Boguszewski, Aart Jan van der Lely, Mark Gurnell, Nienke Biermasz, Katharina Schilbach, Diego Ferone, Monica R Gadelha, Adriana G Ioachimescu, Ezio Ghigo, Christian J Strasburger, Pinar Kadioglu, Pietro Maffei, Niki Karavitaki, Mónica Marazuela, Michael Buchfelder, Sabrina Chiloiro, Anton Luger, Yona Greenman, Elena Valassi, Ignacio Bernabeu, Stefano Frara, Philippe Chanson, Thierry Brue, John Ayuk, Felipe F Casanueva, Annamaria Colao, Pietro Mortini, Sebastian Neggers, Manel Puig-Domingo, Meliha Melin Uygur, Shlomo Melmed
{"title":"Consensus on acromegaly complications: an update.","authors":"Andrea Giustina, Luigi di Filippo, Maria Fleseriu, Rosario Pivonello, Stephan Petersenn, John Wass, Susan L Samson, Alberto M Pereira, Raúl M Luque, Betina Biagetti, Maria Chiara Zatelli, Ken K Y Ho, Cesar L Boguszewski, Aart Jan van der Lely, Mark Gurnell, Nienke Biermasz, Katharina Schilbach, Diego Ferone, Monica R Gadelha, Adriana G Ioachimescu, Ezio Ghigo, Christian J Strasburger, Pinar Kadioglu, Pietro Maffei, Niki Karavitaki, Mónica Marazuela, Michael Buchfelder, Sabrina Chiloiro, Anton Luger, Yona Greenman, Elena Valassi, Ignacio Bernabeu, Stefano Frara, Philippe Chanson, Thierry Brue, John Ayuk, Felipe F Casanueva, Annamaria Colao, Pietro Mortini, Sebastian Neggers, Manel Puig-Domingo, Meliha Melin Uygur, Shlomo Melmed","doi":"10.1007/s11102-026-01639-z","DOIUrl":"10.1007/s11102-026-01639-z","url":null,"abstract":"<p><p>The 16th Acromegaly Consensus Conference in September 2024 updated recommendations on diagnosis and treatment of acromegaly comorbidities. Since the 2020 acromegaly comorbidity management guideline was published, new evidence has emerged on novel and known comorbidities and new treatment approaches. Forty-three experts in the management of acromegaly reviewed the current literature and assessed changes in clinical practice standards and management. Current outcome goals were considered and updated, with a focus on the impact of current and emerging treatments of these comorbidities. Participants assessed factors that determine pharmacological choices, as well as use of specific agents in the management of the most relevant acromegaly comorbidities. We present consensus recommendations highlighting optimization of evidence-based acromegaly comorbidities management.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13124806/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-20DOI: 10.1007/s11102-026-01682-w
Ozan Baskurt, Nurperi Gazioglu
{"title":"External validation of the PANOMEN-3 classification for predicting 1-year surgical remission in pituitary adenoma.","authors":"Ozan Baskurt, Nurperi Gazioglu","doi":"10.1007/s11102-026-01682-w","DOIUrl":"10.1007/s11102-026-01682-w","url":null,"abstract":"<p><p>PURPOSE: The PANOMEN-3 classification was developed as a multidimensional framework to improve prognostic stratification in pituitary adenomas. While prior validation studies have primarily focused on long-term outcomes, its value in predicting early postoperative remission remains less well defined. We aimed to externally validate PANOMEN-3 for predicting 1-year remission in a single-center surgical cohort. METHODS: This retrospective study included 77 consecutive patients who underwent transsphenoidal surgery for pituitary adenoma between June 2023 and January 2025. Tumors were classified according to the WHO 2022 criteria and graded using the PANOMEN-3 system. The primary endpoint was remission at 1 year, defined biochemically for functioning tumors and radiologically for non-functioning tumors. Logistic regression analysis was performed to evaluate the association between PANOMEN grade and remission. Receiver operating characteristic (ROC) analysis was used to assess discriminative performance. RESULTS: At 1-year follow-up, remission was achieved in 52 of 77 patients (67.5%). Remission rates declined with increasing PANOMEN grade (Grade I: 87.5%; Grade II: 59.6%; Grade III: 50.0%; p = 0.0376). Logistic regression analysis demonstrated that PANOMEN-3 grade ≥ 2 was significantly associated with failure to achieve remission (OR 4.54, 95% CI 1.12–18.48, p = 0.034). ROC analysis demonstrated moderate discriminative performance (AUC 0.69). Ki-67 showed a moderate correlation with PANOMEN grade but was not significantly associated with remission. CONCLUSIONS: The PANOMEN-3 classification is associated with early postoperative remission and may provide clinically relevant information for risk stratification in pituitary adenomas. These findings complement previous studies focused on long-term outcomes and suggest that PANOMEN-3 may also have value in the early postoperative setting. Further studies are needed to determine whether early remission predicts long-term disease control. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147729581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-20DOI: 10.1007/s11102-026-01675-9
Isabella Nasi-Kordhishti, Mirko Hladik, Felix Behling, Jens Schittenhelm, Jürgen Honegger
{"title":"Impact of granulation pattern on surgical and long-term outcomes in Cushing's disease: a comparative study of DGCT and SGCT.","authors":"Isabella Nasi-Kordhishti, Mirko Hladik, Felix Behling, Jens Schittenhelm, Jürgen Honegger","doi":"10.1007/s11102-026-01675-9","DOIUrl":"10.1007/s11102-026-01675-9","url":null,"abstract":"<p><p>BACKGROUND: Functioning corticotroph adenomas/PitNETs can be subclassified into Densely and Sparsely Granulated Corticotroph Tumors (DGCT and SGCT). Previous studies suggest that DGCTs are associated with favourable outcomes, while SGCTs may be more aggressive. However, data comparing these subtypes in large cohorts remain limited. MATERIALS & METHODS: We performed a single-center retrospective analysis of 208 patients with CD who underwent TSS between 2004 and 2024. Of these, 175 had DGCT and 33 had SGCT. Data were collected on preoperative diagnostics and imaging, intraoperative findings and strategies, histopathology, and surgical early and long-term outcomes. RESULTS: We found no significant differences in preoperative tumor characteristics or surgical approaches between DGCT and SGCT. However, SGCT patients presented with a shorter interval between symptom onset and surgery. DGCT patients exhibited higher early remission rates (96.9% vs. 87.1%) and lower long-term recurrence rates (9.3% vs. 12.9%). Histopathological analysis revealed higher MIB-1 indices and p53 expression in SGCT, indicating a more aggressive tumor phenotype. CONCLUSION: Despite similar preoperative and intraoperative characteristics, SGCT was associated with a higher recurrence rate and more aggressive biological behavior. These findings emphasize the need for intensified follow-up and consideration of early adjuvant therapies for SGCT patients. Our study provides valuable insights into the management of DGCT and SGCT, suggesting that histological subtype plays a critical role in long-term prognosis and underscores the importance of tailored therapeutic strategies for these subtypes in CD. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147723572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-20DOI: 10.1007/s11102-026-01672-y
Valentino Marino Picciola, Anna Crociara, Serena Piacentini, Lucrezia Rossi, Maria Rosaria Ambrosio, Marco Gessi, Antonio d'Amati, Michele Rubini, Maria Chiara Zatelli
{"title":"A novel pathogenic AIP variant associated with familial isolated pituitary adenoma.","authors":"Valentino Marino Picciola, Anna Crociara, Serena Piacentini, Lucrezia Rossi, Maria Rosaria Ambrosio, Marco Gessi, Antonio d'Amati, Michele Rubini, Maria Chiara Zatelli","doi":"10.1007/s11102-026-01672-y","DOIUrl":"10.1007/s11102-026-01672-y","url":null,"abstract":"<p><p>PURPOSE: Familial Isolated Pituitary Adenoma (FIPA) is a rare autosomal dominant condition associated with germline mutations in the Aryl Hydrocarbon Receptor-Interacting Protein (AIP) gene in ~ 20% of the cases. This study aims to characterize a novel AIP variant in a Sardinian family through genetic, clinical and immunohistochemical analyses and evaluate its pathogenicity. METHODS: The proband, a 17-year-old girl with an invasive prolactin (PRL)-secreting Pituitary Neuroedocrine Tumor (Pit-NET), also known as a pituitary adenoma, underwent germline AIP testing, extended to family members. All variant carriers underwent clinical and radiologic evaluation. Immunohistochemistry for AIP expression was performed on Pituitary adenoma/PitNet tissue from an affected relative. Variant pathogenicity was classified according to guidelines. RESULTS: A novel heterozygous AIP germline variant, c.479del (p.Pro160LeufsTer11 ), was identified in the proband and six relatives. Cabergoline treatment led to PRL levels normalization and tumor shrinkage in the proband. An affected cousin had an aggressive GH-secreting Pituitary adenoma/PitNet requiring multiple treatments. All other carriers had no clinical findings, and their imaging was unremarkable. The variant causes a premature stop codon leading to a truncated protein, lacking the three C-terminal tetratricopeptide repeat domains, essential for AIP protein-protein interactions and tumor suppressor function. Immunohistochemistry showed reduced AIP expression. The variant is classified as pathogenic. CONCLUSION: This report expands the variants spectrum of AIP-related FIPA, confirming intra-familial phenotypic variability, from asymptomatic carriers to aggressive Pituitary adenoma/PitNet. Early genetic screening and surveillance are essential for timely diagnosis and personalized management. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13095975/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147729584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
PituitaryPub Date : 2026-04-15DOI: 10.1007/s11102-026-01671-z
Gerdi Tuli, Jessica Munarin, Alessandra Ferocino, Luisa De Sanctis
{"title":"\"Clinical and biochemical distinctions between organic and idiopathic arginine vasopressin deficiency (AVP-D) in children\".","authors":"Gerdi Tuli, Jessica Munarin, Alessandra Ferocino, Luisa De Sanctis","doi":"10.1007/s11102-026-01671-z","DOIUrl":"10.1007/s11102-026-01671-z","url":null,"abstract":"<p><p>PURPOSE: Arginine Vasopressin Deficiency (AVP-D) is a heterogeneous disorder in childhood, characterized by variable clinical severity and a wide range of underlying etiologies. Early identification of organic forms is crucial, as they are frequently associated with structural hypothalamic–pituitary disease and additional endocrine deficits. METHODS: We retrospectively analyzed 105 children diagnosed with AVP-D over a 25-yearperiod. Clinical features, etiology, baseline biochemical parameters, and results of dynamictesting were collected. Diagnostic evaluation included the water deprivation test and/orarginine-stimulated copeptin assessment. Copeptin concentrations were measured at baselineand after stimulation. RESULTS: Of the 105 patients (53 males; mean age 7.9 ± 0.5 years), 67 (63.8%) had an organic etiology and 38 (36.2%) an idiopathic form. Children with idiopathic AVP-D were significantly younger at diagnosis (6.3 ± 0.8 vs 8.8 ± 0.6 years; p = 0.01). Organic AVP-D was most commonly caused by craniopharyngioma, germ cell tumors, and congenital hypopituitarism. Associated anterior pituitary hormone deficiencies were present in 92.5% of organic cases versus 23.7% of idiopathic cases (p < 0.0001). Patients with organic AVP-D exhibited significantly higher serum sodium and plasma osmolality at diagnosis, together with markedly lower baseline and stimulated copeptin levels. Diagnosis based solely on baseline biochemical parameters was achieved in 70% of organic cases, compared with 15.8% of idiopathic cases (p< 0.0001). CONCLUSION: Organic AVP-D in children is characterized by a distinct and more severe biochemical phenotype, with profound copeptin suppression and frequent anterior pituitary involvement. Baseline biochemical assessment, complemented by stimulated copeptin testing when needed, allows accurate etiological stratification and supports a tailored diagnostic approach in pediatric AVP-D. </p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 3","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147691797","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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