Pituitary最新文献

{"title":"Soluble alpha-klotho as an adjunct biomarker of disease activity in acromegaly: Added value beyond GH and IGF-1.","authors":"Vivek Jha, Sanja Medenica, Naresh Sachdeva, Vaishali Kaur, Pinaki Dutta","doi":"10.1007/s11102-026-01748-9","DOIUrl":"https://doi.org/10.1007/s11102-026-01748-9","url":null,"abstract":"<p><strong>Purpose: </strong>Assessment of disease activity in acromegaly is mainly based on growth hormone and insulin-like growth factor 1. However, discordance between these markers is common and may make follow-up difficult. This study evaluated soluble alpha-Klotho as an adjunct biomarker of biochemical disease activity in acromegaly.</p><p><strong>Methods: </strong>This case-control study included 80 patients with acromegaly and 80 controls. Patients were categorized as controlled, discordant, uncontrolled, or active disease. Serum soluble alpha-Klotho was measured by sandwich enzyme-linked immunosorbent assay and analyzed across groups, disease-status categories, and clinical-biochemical variables. Receiver operating characteristic analysis and nested models assessed its diagnostic and incremental value.</p><p><strong>Results: </strong>Alpha-Klotho concentrations were higher in acromegaly than controls [1551.9 (835.0-3158.3) vs. 759.7 (625.0-898.5) pg/mL; P < 0.001] and increased across disease-status categories: controlled treated acromegaly [722.0 (668.4-837.8)], discordant treated acromegaly [1426.9 (1119.0-1499.9)], uncontrolled treated acromegaly [2980.0 (2585.0-3650.0)], and newly diagnosed active acromegaly [3820.0 (2460.0-4620.0) pg/mL; overall P < 0.001]. Alpha-Klotho correlated with growth hormone and insulin-like growth factor 1 and identified active/uncontrolled disease with an area under the curve of 0.983. Its addition to growth hormone, insulin-like growth factor 1, age, sex, and body mass index improved discrimination from 0.947 to 0.986. Alpha-Klotho ≥ 1579.8 pg/mL was independently associated with active/uncontrolled disease.</p><p><strong>Conclusion: </strong>Soluble alpha-Klotho may serve as an adjunct marker of current biochemical activity in acromegaly, particularly for identifying uncontrolled treated or newly diagnosed active disease. It may aid selected cases with difficult biochemical interpretation, but should be used alongside GH, IGF-1, clinical assessment, and pituitary imaging, not as a replacement test.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 5","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797301","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":"Beyond biochemical control: factors associated with quality of life in patients with acromegaly.","authors":"Ayşe Merve Ok Kurt, Özge Telci Çaklılı, Fatih Bektaş, Gamze Bilik Oyman, Ümmü Mutlu, Hülya Hacişahinoğulları, Gülşah Yenidünya Yalın, Nurdan Gül, Ayşe Kubat Üzüm, Ferihan Aral, Sema Yarman, Özlem Soyluk Selçukbiricik","doi":"10.1007/s11102-026-01746-x","DOIUrl":"https://doi.org/10.1007/s11102-026-01746-x","url":null,"abstract":"<p><strong>Purpose: </strong>Quality of life (QoL) impairment may persist in acromegaly despite biochemical control. We evaluated disease-specific and generic QoL, psychological symptom burden, and associated factors.</p><p><strong>Methods: </strong>In this cross-sectional study, 90 patients completed the Acromegaly Quality of Life Questionnaire (AcroQoL), EQ-5D-3 L, EQ-VAS, Beck Depression Inventory (BDI), and Beck Anxiety Inventory (BAI). Demographic, clinical, biochemical, and treatment-related data were retrieved from records. Correlation analyses and hierarchical multivariable linear regression were used to assess factors associated with total AcroQoL.</p><p><strong>Results: </strong>The cohort included 46 female and 44 male patients; 86 were in biochemical remission. Median total AcroQoL, BDI, BAI, EQ-5D index, and EQ-VAS scores were 76.7, 10.5, 9.0, 0.7, and 80.0, respectively. Female patients had lower total AcroQoL scores and higher anxiety and depression scores than male patients. Higher educational status was associated with better AcroQoL and lower BDI scores. In Model 1, female sex was associated with lower total AcroQoL; however, this association became non-significant after moderate-to-severe depressive symptoms were added in Model 2. Depressive symptom status showed the largest independent association with lower total AcroQoL among model variables (B = - 17.56, 95% CI - 25.23 to - 9.89, p < 0.001), while higher educational status remained independently associated with better QoL. R^² increased from 0.206 to 0.365.</p><p><strong>Conclusion: </strong>In this predominantly biochemically controlled cohort, disease-specific QoL was more closely associated with depressive symptom burden and educational status than with the clinical variables examined. These findings support routine assessment of psychological symptoms and patient-reported outcomes during long-term care.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 5","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797214","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":"Natural history of conservatively managed nonfunctioning pituitary macroadenomas in the elderly: A study from the Swedish Pituitary Registry.","authors":"Camilla Jerning, Charlotte Höybye, Sophie Bensing, Britt Edén Engström, Nasrin Al-Shamkhi, Bertil Ekman, Lorenza Bonelli, Henrik Borg, Pia Burman, Per Dahlqvist, Victor Hantelius, Oskar Ragnarsson, Anna Svensson, Jeanette Wahlberg, Anna-Karin Åkerman, Katarina Berinder, Maria Petersson","doi":"10.1007/s11102-026-01752-z","DOIUrl":"https://doi.org/10.1007/s11102-026-01752-z","url":null,"abstract":"<p><strong>Purpose: </strong>To investigate adenoma progression, pituitary function, visual impairment, and need for later surgery in elderly patients with conservatively managed nonfunctioning pituitary macroadenomas (macro-NFPAs).</p><p><strong>Methods: </strong>Patients ≥ 65 years in the Swedish Pituitary Registry diagnosed 1991-2023 with macro-NFPA and a decision for conservative management were included (n = 673, 38% females). Data at diagnosis and at 1- and 5-year follow-up, defined as assessments closest to 1 year (0.5-<2.5) and 5 years (2.5-<7.5), were retrieved. Surgery was recorded up to 7.5 years. Variables included adenoma size, pituitary function, visual impairment, and surgery. Patients were stratified into age groups 65-72, 73-80, and ≥ 81 years.</p><p><strong>Results: </strong>At diagnosis, mean age was 76 years, females were older (p < 0.001). Mean adenoma diameter and volume were 20 mm and 3067 mm³. Visual field defects and reduced visual acuity were seen in 20% and 12%, with no sex differences. Males had more hormone deficiencies (45% vs. 33%, p < 0.01). Hormone deficiencies and visual impairments increased with age (p < 0.001). The oldest subgroup had the largest adenomas (p < 0.01), with females having larger tumours (p < 0.05) and males more deficiencies (p < 0.05). Adenoma size, hormone deficiencies, and visual impairments remained stable over time within each age group. 6.7% underwent surgery during follow-up. In regression analyses, later surgery was associated with visual field defects at diagnosis (p < 0.05) but not with adenoma size.</p><p><strong>Conclusion: </strong>Pituitary surgery was uncommon in conservatively managed elderly patients with macro-NFPAs. Baseline visual field defects, but not tumour size, were associated with subsequent surgery, supporting regular individualised follow-up in these patients.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 5","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13493488/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797285","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}

{"title":"Clinical presentation, investigation findings, and outcomes of hypophysitis in sarcoidosis: a systematic review.","authors":"Julia F B Cavalcanti, Sara Caixeta de Souza, Leandro Tavares Lucato, Andrea Glezer, Guilherme Diogo Silva","doi":"10.1007/s11102-026-01753-y","DOIUrl":"10.1007/s11102-026-01753-y","url":null,"abstract":"<p><strong>Purpose: </strong>Hypophysitis in sarcoidosis is a rare manifestation of neurosarcoidosis that may cause permanent endocrine dysfunction, visual impairment, and neurological morbidity. We performed a systematic review to characterize its clinical presentation, diagnostic findings, treatment, and outcomes.</p><p><strong>Methods: </strong>PubMed, Embase, and Scopus were searched from inception to January 22, 2025, for reports of adult patients with sarcoidosis-related hypophysitis. Cases were reassessed according to the 2018 Neurosarcoidosis Consortium Consensus Group criteria. Individual patient data were extracted regarding clinical manifestations, hormonal abnormalities, imaging findings, systemic involvement, treatment, recurrence, and long-term outcomes. Sensitivity analyses restricted the cohort to patients meeting definite or probable criteria and stratified cases by publication era (1954-1999, 2000-2017, 2018-2024) to assess the robustness of findings and the impact of temporal heterogeneity.</p><p><strong>Results: </strong>A total of 166 studies comprising 274 patients were included. Diagnostic certainty was definite in 26.3%, probable in 47.8%, and possible in 25.9% of cases. Median age was 37 years (IQR 28-48), and 53.5% were male. AVP deficiency was the most frequent manifestation (53.6%), followed by gonadal dysfunction (38.3%), visual loss (36.1%), headache (32.2%), and fatigue (30.5%). Central hypogonadism was the most common anterior pituitary abnormality (74.1%), followed by central hypothyroidism (63.6%) and hyperprolactinemia (58.1%). MRI most commonly demonstrated pituitary stalk thickening (43.1%) and sellar/suprasellar masses (36.4%). CSF analysis frequently showed elevated protein (68.9%) and pleocytosis (59.5%). Systemic involvement occurred in 80.3% of patients, predominantly affecting lymph nodes and lungs. Although corticosteroids were the mainstay of treatment, recurrence occurred in 43.1% of patients, chronic hormone replacement was required in 36.2%, and long-term desmopressin dependence occurred in 30.5%. Findings were materially unchanged when restricted to the definite/probable cohort (n = 203) and across publication eras, despite increased MRI use and greater adoption of steroid-sparing and biologic therapies over time.</p><p><strong>Conclusions: </strong>Hypophysitis in sarcoidosis should be suspected in patients presenting with stalk thickening, sellar lesions, AVP deficiency, or unexplained hypogonadism. Because serum and CSF biomarkers showed limited sensitivity, systemic investigation and extracranial biopsy are essential. Despite treatment, recurrence and persistent endocrine dysfunction remain common, highlighting the need for long-term multidisciplinary follow-up.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 5","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13493403/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797204","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}

{"title":"Correction to: \"The effect of hypoprolactinemia on the cardiometabolic effects of rosuvastatin in men: A matched Cohort Pilot Study\".","authors":"Robert Krysiak, Karolina Kowalcze, Witold Szkróbka, Johannes Ott, Simona Zaami, Bogusław Okopień","doi":"10.1007/s11102-026-01744-z","DOIUrl":"https://doi.org/10.1007/s11102-026-01744-z","url":null,"abstract":"","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 5","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797229","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":"Cardiometabolic effects of sequential prolactin states in dopamine agonist-treated prolactinoma patients: a retrospective longitudinal study.","authors":"Pedro Iglesias, María Dolores Moure Rodríguez, Fernando Guerrero-Pérez, Andreu Simó-Servat, Laura González Fernández, Eva Fernández-Rodríguez, Patricia Pérez Castro, Rocío Villar-Taibo, Betina Biagetti, Aida Orois, Sara Donato, Victoria Alcázar Lázaro, Noel Roig-Marín, Guillermo Serra, Soralla Civantos, Gonzalo Rivero, Carmen María Fernandez de Araoz, Isabel Pavón de Paz, Elena Outeiriño-Blanco, Fernando Cordido, Rogelio García Centeno, Marta Araujo-Castro, Cristina Lamas, Miguel Paja, Felicia Alexandra Hanzu, Juan J Díez","doi":"10.1007/s11102-026-01740-3","DOIUrl":"10.1007/s11102-026-01740-3","url":null,"abstract":"<p><strong>Purpose: </strong>Hyperprolactinemia has been associated with adverse cardiometabolic alterations, whereas the metabolic consequences of treatment-induced hypoprolactinemia remain uncertain. We aimed to evaluate longitudinal cardiometabolic changes across sequential prolactin states in dopamine agonist-treated prolactinoma patients.</p><p><strong>Methods: </strong>We conducted a retrospective multicentre longitudinal cohort study including 47 prolactinoma patients from 19 tertiary hospitals in Spain. All patients sequentially transitioned from hyperprolactinemia (HyperPRL) to normoprolactinemia (NormoPRL) and subsequently to hypoprolactinemia (HypoPRL) during cabergoline therapy. Longitudinal changes in anthropometric, hemodynamic, and metabolic parameters were assessed using repeated-measures analyses and generalized estimating equation (GEE) models. Correlation and multivariable regression analyses were performed to evaluate associations between prolactin reduction and metabolic changes.</p><p><strong>Results: </strong>Transition from HyperPRL to NormoPRL was associated with significant reductions in body weight (80.4 ± 23.6 vs. 78.7 ± 22.8 kg; p = 0.009), body mass index (29.3 ± 7.4 vs. 28.4 ± 7.3 kg/m²; p = 0.034), and total cholesterol (194.3 ± 36.5 vs. 178.7 ± 34.8 mg/dL; p = 0.006). Triglyceride concentrations showed a trend toward reduction across prolactin states, with a significant decrease observed between HyperPRL and NormoPRL. No additional cardiometabolic improvements were detected after transition from NormoPRL to HypoPRL. GEE analyses confirmed significant longitudinal reductions in body weight and total cholesterol after adjustment for age, sex, and initial cabergoline dose, and results remained unchanged in sensitivity analyses additionally accounting for treatment duration. Although percentage prolactin reduction showed a weak inverse association with body weight change in exploratory analyses, no independent associations were identified after multivariable adjustment.</p><p><strong>Conclusions: </strong>Normalization of prolactin concentrations was associated with significant improvements in body weight, BMI, and total cholesterol concentrations. In contrast, no significant cardiometabolic improvements were detected following the subsequent development of treatment-induced hypoprolactinemia. Furthermore, the magnitude of prolactin reduction was not independently associated with changes in anthropometric or metabolic parameters after multivariable adjustment. These findings suggest that the main cardiometabolic changes occur during the transition from hyperprolactinemia to normoprolactinemia. However, the specific cardiometabolic effects of treatment-induced hypoprolactinemia require confirmation in larger prospective studies.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 5","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148797138","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":"Hypothalamic syndrome and acquired hypothalamic obesity: from fragmented care to integrated multidisciplinary management.","authors":"Betina Biagetti, Marta Ramon-Krauel, Juan José López Gómez, Irene Breton, Cristina Lamas, Assumpta Caixàs, Francisco PitaGutiérrez, María Dolores Ollero García, Albert Lecube, M Cristina Azcona-Sanjulian, Anna Aulinas, Esteban Cordero Asanza, Patricia Monsalve Martín, Marina Diaz Marsa, María D Ballesteros-Pomar","doi":"10.1007/s11102-026-01743-0","DOIUrl":"10.1007/s11102-026-01743-0","url":null,"abstract":"<p><p>Acquired hypothalamic obesity (AHO) is a complex neuroendocrine disorder characterized by rapid weight gain and multisystem dysfunction following hypothalamic damage. AHO represents the weight-related manifestation of the broader hypothalamic syndrome (HS), a heterogeneous spectrum of dysfunctions that can also encompass disturbances in pituitary function, energy expenditure, sleep, behavior, and thermoregulation, with obesity itself absent in some affected individuals. This narrative review provides an updated and integrative overview of AHO pathophysiology, clinical characterization, and current and emerging approaches to diagnosis, prevention, and treatment, with the aim of proposing a multidimensional framework to improve patient care. The literature, including seminal studies, recent systematic reviews, clinical trials, and expert consensus statements, was critically appraised with emphasis on clinical applicability and existing knowledge gaps. Current evidence highlights the limitations of BMI-based assessment and supports a multidomain clinical approach. In this context, a three-layer diagnostic model is proposed, integrating etiological factors, longitudinal anthropometric and metabolic assessment, and domain-specific clinical evaluation. Preventive strategies remain underexplored, although hypothalamus-sparing interventions, early monitoring of weight trajectory, and management in specialized Pituitary Tumor Centers of Excellence in surgical cases appear critical. Therapeutic options are limited, with modest evidence for conventional treatments; however, emerging therapies targeting the melanocortin pathway, including MC4R agonists, showed promising results. Overall, AHO requires a paradigm shift toward early identification, multidimensional assessment, and coordinated multidisciplinary care, with future research prioritizing preventive strategies and robust clinical trials to improve outcomes.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 4","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148685444","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":"Assessment of nurses' knowledge and awareness of acromegaly in a high-volume tertiary center: a cross-sectional study.","authors":"Birgul Genc, Sefika Burcak Polat, Aysegul Koc, Reyhan Ersoy","doi":"10.1007/s11102-026-01737-y","DOIUrl":"10.1007/s11102-026-01737-y","url":null,"abstract":"<p><strong>Background and aim: </strong>Acromegaly is a rare endocrine disorder characterized by delayed diagnosis and substantial multisystem morbidity. Nurses play an important role in early recognition and multidisciplinary management of the disease; however, evidence regarding their knowledge of acromegaly is limited. This study aimed to assess the knowledge and awareness of nurses regarding acromegaly and to identify factors associated with knowledge level.</p><p><strong>Methods: </strong>A descriptive cross-sectional study was conducted between December 2024 and March 2025 at Ankara Bilkent City Hospital, Türkiye. A total of 884 nurses participated voluntarily. Data were collected using a researcher-developed, content-validated Acromegaly Experience and Knowledge Questionnaire (Cronbach's α = 0.852). Binary logistic regression analysis was performed to identify independent predictors of knowledge level.</p><p><strong>Results: </strong>The mean age of participants was 29.8 ± 6.3 years; 84.8% were female and 92.0% held a bachelor's degree. Most participants (89.8%) reported little or no previous experience with acromegaly. Knowledge levels were classified as low in 11.7%, moderate in 47.0%, and high in 41.3% of participants. In multivariable analysis, female sex was the only independent predictor of higher knowledge (OR = 1.83, 95% CI: 1.22-2.76, p = 0.004).</p><p><strong>Conclusions: </strong>Most nurses demonstrated moderate to high knowledge of acromegaly despite limited clinical experience. However, important knowledge gaps remain, highlighting the need for structured educational programs and continuing professional development to strengthen nurses' preparedness for the care of patients with rare endocrine disorders.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 4","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148649027","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":"Higher surgical cure of pediatric gigantism with endoscopic endonasal surgery: case series and review of the literature.","authors":"Andrew L A Garton, Sergio W Guadix, Patricia M Vuguin, Jeffrey P Greenfield, Theodore H Schwartz","doi":"10.1007/s11102-026-01738-x","DOIUrl":"10.1007/s11102-026-01738-x","url":null,"abstract":"<p><strong>Objectives: </strong>Pediatric gigantism associated with somatotropinomas is exceedingly rare. Although transsphenoidal surgery (TSS) constitutes first-line management, tumor recurrence is common. However, it remains unclear how surgical cure rates differ between transsphenoidal microsurgery (TMS) and the more contemporary endoscopic endonasal approach (EEA).</p><p><strong>Methods: </strong>Three children with somatotropinoma-induced gigantism who underwent EEA between December 2010 and October 2021 were identified via retrospective chart review. Primary outcomes collected included complications, length of stay (LOS), and rates of postoperative biochemical remission according to the 2010 Acromegaly Consensus Group criteria. A literature review compiled surgical cure rates for pediatric gigantism, stratified by TSS technique (TMS vs EEA).</p><p><strong>Results: </strong>Three children (2F, 1 M) presented with clinical signs of gigantism at an average age of 12 years [range: 10-14y]. Two patients failed prior somatostatin analogue therapy and one underwent attempted but incomplete EEA at an outside institution. Mean tumor diameter was 0.9 ± 0.2 cm. EEA was performed without complications and a mean LOS of 4.6 days [range: 3-7d]. Biochemical remission was achieved in all patients without adjuvant therapy during follow-up [range: 1-11y]. Nineteen historical studies report surgical outcomes for 325 total pediatric patients with gigantism. Overall surgical cure rates are estimated at 24.7% (73/296). Of these, 17 studies distinguished between transsphenoidal approaches, reporting surgical cure rates of 20.5% (54/264) with TMS and 66.7% (6/9) for EEA. Rates of hypopituitarism/diabetes insipidus were notably higher in TMS compared with EEA patients (31.1% vs 6.8%).</p><p><strong>Conclusion: </strong>Pediatric gigantism is a rare clinical entity associated with historically low surgical cure rates. Literature review indicates that EEA is associated with higher cure rates, which is further supported by our small series in which all patients were cured. The surgical cure rate for gigantism may be higher in the contemporary endoscopic era and merits further study with larger populations of patients.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 4","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148648966","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":"Delayed diagnosis of ectopic ACTH-secreting tumors following transsphenoidal surgery for presumed MRI-negative Cushing's disease: implications for long-term surveillance.","authors":"Risa Waki, Atsushi Ishida, Go Matsuoka, Koji Takano, Noriaki Fukuhara, Akira Takeshita, Yutaka Oki, Shozo Yamada","doi":"10.1007/s11102-026-01741-2","DOIUrl":"10.1007/s11102-026-01741-2","url":null,"abstract":"<p><strong>Purpose: </strong>To determine the long-term diagnostic outcomes of rigorously defined magnetic resonance imaging (MRI)-negative ACTH-dependent Cushing's syndrome.</p><p><strong>Methods: </strong>We retrospectively reviewed 530 patients who underwent transsphenoidal surgery (TSS) for Cushing's disease (CD) between 1992 and 2026. Among them, 59 patients had no identifiable pituitary adenoma on the highest-quality preoperative MRI. After excluding two patients with insufficient follow-up, 57 patients (10.8%) were included in the analysis.</p><p><strong>Results: </strong>During long-term follow-up, 5 of 57 patients (2 men and 3 women; median age, 58 years; range, 34-61 years) were ultimately diagnosed with ectopic ACTH-secreting tumors. Preoperative endocrinological evaluation suggested cyclic CD in one patient and CD in four patients. Bilateral inferior petrosal sinus sampling (BIPSS) indicated a pituitary source in four patients and an ectopic source in one; however, no definitive ectopic lesion was identified by radiological or functional imaging in any case. TSS was performed after informed consent, but no tumor was identified intraoperatively or on pathological examination. During follow-up, ectopic tumors were detected approximately 2 years after surgery in three patients (pulmonary, adrenal, and pancreatic neuroendocrine tumors), whereas a pulmonary neuroendocrine tumor and a thymic neuroendocrine tumor were identified 9 and 18 years later, respectively, in the remaining two patients. All tumors were surgically resected, confirmed to be ectopic ACTH-secreting tumors, and resulted in complete remission.</p><p><strong>Conclusion: </strong>Differentiating between pituitary adenoma and ectopic tumors remains challenging in MRI-negative cases. In patients who fail to achieve remission after TSS, careful long-term follow-up, with continued consideration of ectopic ACTH secretion, is essential.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"29 4","pages":""},"PeriodicalIF":3.8,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148606713","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}