Pituitary最新文献

Objective: This study evaluated temperament, character dimensions, anxiety, depression, and quality of life (qol) in patients with acromegaly, and examined their associations with clinical variables including disease activity, duration, treatment modality, and tumor characteristics.

Methods: In a cross-sectional study, 77 patients with acromegaly and 99 healthy controls with no difference in age and sex compared to patients completed the Temperament and Character Inventory-Revised (TCI-R), Hospital Anxiety and Depression Scale (HADS), and Acromegaly Quality of Life Questionnaire (AcroQoL). Patients were subgrouped by remission status, treatment type, and histopathological findings. Between-group comparisons and correlation analyses were performed.

Results: Compared with controls, patients with acromegaly exhibited higher impulsiveness but lower levels of emotional warmth, persistence, and self-directedness. Higher self-acceptance scores were associated with remission, while female patients exhibited significantly lower AcroQoL-Physical scores than male patients. IGF-1 levels positively correlated with novelty seeking. Qol was negatively correlated with harm avoidance, anxiety, and depression, whereas both anxiety and depression were positively associated with harm avoidance.

Conclusions: Acromegaly is associated with distinct personality patterns that may influence coping, well-being, and treatment experiences. These findings underscore the potential value of integrating psychosocial assessment and personalized support into the management of acromegaly, and highlight the need for further studies to clarify underlying neurobiological and cultural influences.

Purpose: Isolated growth hormone deficiency (IGHD) is one of the treatable causes of short stature. We aimed to describe the clinical, biochemical, and molecular characteristics of IGHD and identify clinical predictors of mutation positivity and first-year height response to recombinant human growth hormone (rhGH).

Methods: Sixty-three children with IGHD who were on a minimum of one year of rhGH therapy were included. Detailed auxology, growth hormone provocative testing, and pituitary MRI were performed. Genetic variants were identified by whole-exome sequencing and correlated with auxological and biochemical parameters. The first-year height response was assessed as ΔHeight SDS, and predictors of response were analysed using regression models.

Results: The mean age at presentation was 8.9 ± 2.9 years; 57% were born to consanguineous parents. Severe auxological impairment was observed, with a mean height SDS of - 4.29 ± 1.09, and a mean genetic height deficit of - 2.42 ± 1.12 SDS. Genetic variants were identified in 49% of the cohort, predominantly in GHRHR (39.7%), with the p.Glu72Ter being the most frequent, while GH1 variants were less prevalent (4.8%). Genetic variant-positive children presented at a younger age, with more severe growth failure, and a better response to rhGH therapy. Regression analysis identified genetic variant-positive status as an independent predictor of favourable first-year growth response (ΔHeight-SDS:1.24 ± 0.50).

Conclusion: The South Indian IGHD cohort exhibits a distinct genetic profile, with GHRHR p.Glu72Ter being the most frequent variant, consistent with a founder effect. Severe short stature, low peak stimulated growth hormone levels, and superior first-year growth response indirectly predicted mutation positivity.

Purpose: This study aimed to evaluate the effectiveness and safety of two-stage endoscopic surgery for the complete resection of cystic craniopharyngiomas.

Methods: We retrospectively evaluated 11 patients who underwent two-stage endoscopic surgery between March 2020 and July 2025. The lesions showed suprasellar and lateral extension toward the optic chiasm or the foramen of Monro, resulting in visual impairment or obstructive hydrocephalus. Cyst fenestration (CF) was initially performed using a transventricular approach with a flexible endoscope. One to two months later, endoscopic transsphenoidal surgery (eTSS) was performed to achieve complete resection. Tumor size (vertical diameter, horizontal diameter, and volume) was measured at three time points: pre-CF, post-CF (≤ 4 days), and pre-eTSS. Extent of resection, recurrence, and pituitary and hypothalamic function were assessed post-eTSS.

Results: Complete resection was achieved in 10 of 11 patients, with no recurrence during a median follow-up of 22.8 months (range, 3.6-65.3). At a median interval of 54 days (range, 27-86) between CF and eTSS, the mean linear reduction was 45.3% superiorly and 39.3% laterally; mean tumor volume decreased by 68.0%. In some cases, areas typically difficult to access via eTSS alone decreased in size. All preoperative symptoms, except for hypopituitarism and diabetes insipidus, improved immediately after CF. No hypothalamic dysfunction was observed.

Conclusion: Two-stage endoscopic surgery for cystic craniopharyngiomas is effective and safe, enabling complete resection with low recurrence during short- to mid-term follow-up.

Background: The management of pituitary adenomas in very elderly patients (age ≥ 85 years) remains challenging due to age-related frailty and variability in treatment tolerance. This study aimed to identify clinical and sociodemographic factors associated with survival in patients age ≥ 85 years with pituitary adenoma and assess the plausibility of surgical resection.

Methods: Patients aged 85 and up (very elderly cohort) were queried from the SEER database. Additionally, patients from the age range of 75-84 (elderly cohort) were queried for comparisons. Demographic variables, adenoma size, and treatment variables were assessed, and differences were identified using a Chi-squared tests for independence and two-sample t-tests. Differences in survival were assessed using multivariable Cox proportional hazards models stratified by age cohort. Kaplan-Meier plots were generated to assess survival differences among treatment groups in the very elderly cohort.

Results: Across all cohorts, subtotal resection and female sex were independently associated with improved survival and age was associated with worse survival. For the very elderly cohort, adenoma size (HR = 1.004; p = 0.005) was associated with worse overall survival, while female sex (HR = 0.656; p < 0.001) and subtotal resection (HR = 0.557; p = 0.005) were associated with increased overall survival. Black race was associated with worse survival in the 75-84 elderly cohort only (HR = 1.479; p < 0.001).

Conclusion: In patients ≥ 85 years of age with pituitary adenoma, surgical resection appears to provide an overall benefit to survival and should be considered among other factors.

Objective: This study aims to evaluate the clinical utility of minimally invasive intraoperative ultrasound as a real-time imaging adjunct during endoscopic endonasal pituitary adenoma surgery. The goal is to determine its effectiveness in tumor localization, identification of critical structures, and detection of residual tumor tissue.

Methods: A retrospective analysis was conducted on 22 patients who underwent endoscopic endonasal pituitary surgery with intraoperative ultrasound at our institution from August 2024 to June 2025. The ultrasound probe was used before dural opening to identify the tumor, normal gland, and adjacent structures, including the internal carotid artery (ICA). After tumor resection, ultrasound was employed to assess residual tumor. Clinical and surgical outcomes, including extent of resection and perioperative complications, were reviewed.

Results: Intraoperative ultrasound enabled clear visualization of the tumor-normal gland interface in both solid and cystic macroadenomas, facilitating safe and precise resection. The technique aided in identification of the ICA during cavernous sinus invasion, reducing vascular injury risk. In cases where tumors were not visible on preoperative magnetic resonance imaging (MRI), intraoperative ultrasound successfully detected the lesions, allowing targeted resection. Gross total resection (GTR) was achieved in 86.4% of cases. The median follow-up was 8 months (range, 3-13 months). There were no new postoperative hormonal deficits. Transient diabetes insipidus occurred in three cases, and all resolved within three months.

Conclusion: Intraoperative ultrasound provides real-time, high-resolution imaging that enhances the precision and safety of endoscopic pituitary surgery. The technique is particularly valuable for tumor localization, identification of critical neurovascular structures, and detection of residual tumor tissue. Further studies are warranted to validate a broader applicability and refine its role in differentiating tumor from normal gland tissue.

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.

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.