Pituitary最新文献

Hypertension affects nearly half of patients with acromegaly, yet current studies inconsistently report blood pressure phenotypes due to variability in the use of office, home, and ambulatory monitoring. This companion article proposes a standardized, risk-stratified approach using ambulatory and home blood pressure monitoring to classify sustained, masked, and nocturnal hypertension and to guide antihypertensive de-escalation in relation to biochemical control. The framework incorporates key timepoints-diagnosis, early post-therapy reassessment, and long-term follow-up-while emphasizing modality-specific diagnostic thresholds, dipping patterns, and cardiovascular risk profiles. Structured follow-up using periodic ambulatory blood pressure monitoring and validated home monitoring enables more precise phenotyping, optimizes treatment decisions, and improves comparability across future acromegaly cohorts.

Purpose: To investigate the prevalence and risk factors of vertebral fractures (VFs) and to compare bone mineral density (BMD) assessments by dual-energy X-ray absorptiometry (DXA) and quantitative computed tomography (QCT) in patients with acromegaly.

Methods: This cross-sectional study included 149 patients with acromegaly. Clinical, biochemical, and imaging data were collected, and logistic regression analyses were performed to identify VF risk factors.

Results: VFs were detected in 39.6% of patients and were associated with older age, longer disease duration, higher body mass index(BMI), elevated IGF-1 levels, and lower serum phosphorus. QCT-derived volumetric BMD (vBMD) was significantly lower in patients with VFs, whereas DXA-based lumbar areal BMD (aBMD) did not differ between groups. Elevated IGF-1 (%ULN) and reduced vBMD were identified as independent risk factors for VFs.

Conclusion: Vertebral fractures are common in acromegaly. In one of the largest Chinese acromegaly cohorts to date, QCT demonstrated superior discrimination of VF risk compared with DXA, supporting its potential complementary role in skeletal fragility assessment in acromegaly.

Context: Carney Complex (CNC) is a rare multiple endocrine neoplasia syndrome, due to PRKAR1A mutation, that causes GH-secreting pituitary adenomas (PA) in 10% of patients. PRKAR1A is not currently analyzed in patients with isolated sporadic or familial PA, in contrast to MEN1 or AIP.

Objective: We report the case of a young man diagnosed with a PA at age 21 during melanoma follow-up, with a family history of PA. He was initially misdiagnosed with FIPA due to a misclassified AIP mutation, before a final diagnosis of CNC was established. We subsequently retrospectively analysed PRKAR1A in a cohort of patients with PA to assess the relevance of includingPRKAR1A in PA predisposition gene panels.

Methods: After AIP variant reclassification and whole genome analysis of the patient, we performed a retrospective analysis of the genetic and clinical data of patients who underwent germline genetic testing for hereditary predisposition to PA.

Results: Two hundred and twenty patients were included, of whom 16 (7.3%) had a family history of PA, 162 (72.7%) had macro-PA, and 54 (24.6%) had GH- or GH/PRL-secreting PA. Four patients (1.8%) carried pathogenic variants in AIP or MEN1, but none in PRKAR1A.

Conclusion: This case underscores the importance of periodically reassessing genetic variants, as reclassification can significantly impact patient management. It also highlights the clinical variability of CNC and the need to screen for CNC features in young patients with acromegaly. Further research is warranted to determine the value ofPRKAR1A testing in isolated GH- and GH/PRL-secreting PA.

Purpose: Pituitary stalk lesions (PSL) present unresolved etiological and diagnostic challenges. This study provides a comprehensive clinical analysis to address these gaps.

Methods: We retrospectively evaluated 98 adults with pituitary stalk (PS) abnormalities, assessing PS features, demographics, clinical data, hormonal profiles, imaging, pathology, diagnoses, and treatments.

Results: Among 98 patients (59 F/39 M; median age 37 years), PS thickening was most frequent (52%), followed by thinning (18.4%) and nodularity (17.3%). The distribution of lesion types varied significantly across etiological categories (p < 0.001). Thickening was most frequent in inflammatory etiologies, thinning in unclassifiable cases, and nodularity in inflammatory etiologies. Solid organ metastases occurred in older patients. Arginine vasopressin deficiency (AVP-D) rates ranged from 57% to 61% in non-adenomatous local neoplastic lesions, inflammatory etiologies, and solid organ metastases. Congenital cases mainly had growth retardation and gonadal dysfunction. Extrapituitary involvement was highest in solid organ metastases, followed by inflammatory etiologies, and non-adenoma local neoplastic lesions. PS thickening was more pronounced in solid organ metastases. Isolated PS involvement was most often (25%) due to Langerhans cell histiocytosis. In PSIS, all had ectopic neurohypophysis, lacked AVP-D, and showed at least one anterior pituitary hormone deficiency.

Conclusion: In conclusion, we describe a unique series of PSL that contribute to the understanding of the heterogeneous spectrum of PSL. In this study, we discuss the clinical and radiological features of PSL and provide current recommendations for differential diagnosis.

Pituitary adenomas are prevalent intracranial tumors that, though benign, present surgical challenges due to their anatomy and hormonal behavior. Transsphenoidal surgery is the standard approach, but differentiation between normal gland and adenoma limits complete resection. Traditional imaging methods like MRI and CT are costly and disruptive, while intraoperative ultrasound (IOUS) offers a real-time, practical alternative for enhancing localization and surgical confidence. Recent advances in IOUS technology show promise, yet challenges such as spatial resolution and operator dependency hinder widespread use. This editorial discusses the potential of IOUS as a vital tool for improving safety and outcomes in pituitary adenoma surgeries.

Purpose: To investigate the protein expression levels of circadian clock genes in pituitary adenomas (PAs) using the immunohistochemical staining method.

Methods: Patients who had regular follow-up at the pituitary center, underwent transsphenoidal surgery (TSS) for PA between 8:00 AM and 2:00 PM, and had sufficient clinical data were enrolled. Patients with a known diagnosis of depression, those receiving psychiatric medication or melatonin agonists that may affect the protein expression of circadian clock genes, or those working night shifts during the preoperative period were excluded. Formalin-fixed paraffin-embedded tissue samples from patients with somatotroph, gonadotroph, lactotroph, and corticotroph adenomas were immunohistochemically stained for BMAL1, CLOCK, CRY1, CRY2, and PER2. Non-neoplastic adenohypophysis tissue adjacent to the adenoma in surgical specimens underwent the same staining procedure and served as the control group. Protein expression levels were then evaluated.

Results: A total of 86 PAs and 17 non-neoplastic adenohypophysis tissue samples were evaluated. Total BMAL1 scores tended to be higher in PAs than in control tissues (170.93 ± 59.81 vs. 142.06 ± 47.40; p = 0.064), whereas total CRY2 scores tended to be lower (12.5 [0-59.25] vs. 35 [10-60]; p = 0.053). Total CLOCK scores were lower in patients with recurrent disease compared to those without recurrence (225 [165-270] vs. 250 [212.5-280]; p = 0.038), and preoperative maximum tumor size was negatively correlated with total CRY2 scores (r=-0.463, p < 0.001). Among PA subtypes, CRY2 was the circadian clock gene showing the most prominent differences.

Conclusion: Alterations in the protein expression levels of circadian clock genes may contribute to the development and behavior of PAs.

Purpose: Pituitary adenomas (PAs) are common intracranial tumours with heterogeneous behaviour. Accurate risk stratification is critical for predicting recurrence/progression and for guiding management. The pituitary adenoma nomenclature 3 (PANOMEN 3) classification, introduced in 2024, integrates clinical, radiological, and pathological factors; however, its prognostic utility and histopathological criteria remain uncertain.

Methods: This multicentre retrospective cohort study included 804 patients with PAs surgically treated between January 2010 and December 2023. Patients were classified using the original PANOMEN 3 system and a modified system incorporating silent corticotroph adenomas (SCAs) as high-risk subtypes, revised proliferative activity criteria (Ki-67 ≥ 3%, mitotic index > 2/10 high-power fields, or p53 positivity), and Trouillas clinicopathological grading. Recurrence-free survival was analysed using Kaplan-Meier and Cox regression models. Predictive performance was assessed using receiver operating characteristic curves. Health-related quality of life (HRQoL) was measured using the EuroQol 5 Dimensions-3 Levels (EQ-5D-3 L) and EuroQol Visual Analogue Scale (EQ-VAS) preoperatively and six months postoperatively.

Results: Over a median follow-up of 5.0 years, 169 patients (21.0%) experienced recurrence/progression. The original PANOMEN 3 classification showed moderate predictive accuracy. The modified classification markedly improved predictive performance to match Trouillas' grading. Higher PANOMEN 3 grade was associated with poorer HRQoL outcomes.

Conclusion: The PANOMEN 3 classification provides clinically relevant prognostic value for recurrence/progression and postoperative HRQoL in PAs. The inclusion of SCAs and refined proliferative criteria enhanced predictive accuracy and demonstrated its potential as a comprehensive risk stratification tool. Prospective studies, including non-surgical cohorts, are warranted to confirm its generalisability.

Background: The management of pregnancy in patients with combined pituitary hormone deficiency (CPHD) represents a unique challenge due to the complex interplay of multiple pituitary hormone deficiencies, higher risk of feto-maternal complications, and lack of established evidence-based clinical guidelines. Though improvements in assisted reproductive techniques (ART) and multidisciplinary care over the last decades have increasingly enabled successful pregnancies in hypopituitary women, genetic causes of CPHD are rarely identified and data on pregnancy outcomes in affected women are scarce, underscoring the need for further case documentation to better inform clinical practice.

Aims: We describe the case of a woman with genetically confirmed PROP1-related CPHD who achieved and completed a successful pregnancy through ART and tailored endocrine management throughout gestation and the postpartum period. This case report highlights the importance of preconception counseling, careful hormone replacement, and close monitoring and collaboration among expert endocrinologists, obstetricians, and neonatologists to optimize maternal and fetal outcomes in women with genetic CPHD. Furthermore, we provide a comprehensive literature review exploring key issues related to the main clinical and therapeutic challenges in the management of hypopituitarism during pregnancy, and particularly in the context of CPHD.

Purpose: Growth hormone (GH) and insulin-like growth factor-1 (IGF-1) influence erythropoiesis; however, data on the prevalence and determinants of anemia in acromegaly are limited. This study aimed to investigate the frequency, characteristics, and risk factors of anemia in patients with acromegaly.

Methods: We retrospectively reviewed medical records of 381 patients with acromegaly followed at a tertiary referral center. Clinical, hormonal, radiological, and hematological data were analyzed.

Results: Anemia was identified in 219 of 381 patients with acromegaly (57.5%). Most cases were normocytic (67%) and of mild severity (45%), while moderate and severe anemia accounted for 11.3% and 0.8%, respectively. Iron deficiency anemia was present in 41 of 219 anemic patients (18.7%) more frequently in women than men (27/120 [22.5%] vs. 14/98 [14.3%]), and thalassemia minor was detected in 4% of cases. No folate- or vitamin B12-deficiency anemia was observed. The majority of anemic cases (77.3%) remained unexplained. Sex-specific analyses showed that microcytic anemia was predominantly seen in women (49/73, 67.1%), whereas normocytic anemia displayed a nearly equal distribution between men (74/145, 51.0%) and women (71/145, 49.0%). Compared with non-anemic patients, those with anemia more frequently had macroadenomas with invasive features, residual tumor on postoperative imaging, and hypopituitarism, together with higher postoperative GH, IGF-1, and prolactin levels (all p < 0.05). They required more intensive treatment, including somatostatin receptor ligands (SRLs) (71.1% vs. 49.7%), dopamine agonists (18.8% vs. 10.4%), and radiotherapy (29.8% vs. 9.2%). Cancer prevalence was also higher in anemic patients (17% vs. 10.4%), although not statistically significant. Multivariate analysis identified macroadenoma, SRL therapy, and L-thyroxine use as independent predictors of anemia.

Conclusion: Anemia is a frequent and clinically relevant comorbidity in acromegaly, associated with tumor burden, hypopituitarism, treatment intensity. These findings highlight the need for systematic evaluation of anemia in acromegalic patients and underscore the importance of future prospective studies to clarify its complex pathophysiology.

Purpose: Factors related to the development of new pituitary hormone deficiencies following transsphenoidal surgery for non-functioning pituitary adenomas are multifactorial and remain poorly understood. We explored associations with brain injury biomarkers and investigated intraoperative hypotension (IOH) as a potential mediator.

Methods: This prospective study included 100 patients undergoing endoscopic transsphenoidal surgery. Two individual outcomes at 12-months postsurgery were analysed: new anterior pituitary hormone deficiency (APH-D) and new arginine vasopressin deficiency (AVP-D). Plasma concentrations of glial fibrillary acidic protein (GFAP), neurofilament light chain (NfL), and tau were measured preoperatively and on postoperative days 1 and 5. IOH was assessed using two definitions: duration below an absolute MAP threshold of 65 mmHg and duration below a relative threshold of 20% below preoperative MAP. Associations between new deficiency and biomarkers were assessed using mixed-effects models, and associations with IOH were evaluated using the Mann-Whitney U test.

Results: Elevated postoperative GFAP, NfL, and tau were associated with new APH-D, with GFAP also linked to new AVP-D. Patients who experienced new APH-D demonstrated longer durations of relative IOH (median [IQR] 155 min [54-216] vs. 82 min [20-154]; p = 0.03). There was no difference in relative or absolute IOH for those with new AVP-D.

Conclusion: Elevated postoperative plasma GFAP, NfL, and tau might indicate increased risk of long-term postoperative pituitary hormone deficiency. Relative IOH may also contribute to these deficiencies.