Pituitary最新文献

Background: Craniopharyngioma is a rare, parasellar tumor of low histological malignancy (WHO grade 1) arising from remnants of the Rathke pouch, which can disrupt hypothalamic modulation. Sleep disturbances, including excessive daytime sleepiness (EDS), are commonly reported in patients with craniopharyngioma, although findings have been inconsistent. This systematic review and meta-analysis was conducted to aggregate prior sleep-related findings in craniopharyngioma patients, with the aim of determining the prevalence and severity of sleep disorders, as well as quantifying alterations in sleep structure and sleep breathing.

Method: Databases including PubMed, Web of Science, EBSCO, and Cochrane library were searched up through April 8, 2025, to identify studies investigating sleep disturbances in craniopharyngioma patients. Random-effect meta-analysis was employed to calculate the pooled proportion of patients with sleep disorders, pooled means of sleep scale and polysomnography parameters, and the standard mean difference in sleep parameters between craniopharyngioma patients and control group.

Results: A total of 22 studies, encompassing 1137 patients and 370 matched controls, were included. The meta-analysis revealed that the pooled mean score of Pittsburgh sleep quality index in craniopharyngioma patients was 6.76 (95% CI: 6.17-7.35). The overall prevalence of EDS was 50% (95% CI: 40%-61%), with a higher estimate from the multiple sleep latency test (MSLT) (55%, 95% CI: 43%-67%) and a lower estimate from Epworth sleepiness scale (ESS) (36%, 95% CI: 25%-48%). The prevalence of narcolepsy and obstructive sleep apnea were 27% (95% CI: 21%-33%) and 14% (95% CI: 5%-24%), respectively. The periodic limb movements index was obviously abnormal across age subgroups in craniopharyngioma. Compared to healthy controls, patients with craniopharyngioma exhibited significantly reduced rapid eye movement sleep; and compared to obese controls, craniopharyngioma patients showed shorter sleep onset latency but no reduction in non-rapid eye movement stage 3. Additionally, the pooled mean sleep latency for patients with craniopharyngioma on the MSLT was 8.45 min, significantly shorter than in healthy and obese controls.

Conclusions: Our findings imply sleep-related impairments in patients with craniopharyngioma during both of diurnal and nocturnal phases, particularly the daytime hypersomnolence and narcolepsy. Discrepancies between subjective ESS and objective MSLT of EDS assessments underscore the need for the MSLT in pediatric patients. These results suggest that the tumor and/or its treatment may affect the coordination of hypothalamic sleep-wake regulatory nuclei or orexin neurons.

Purpose: This review aims to identify and evaluate the management options for pituitary tuberculosis in the pediatric population.

Methods: The study was conducted based on a comprehensive literature review using the PubMed, Cochrane Library, and ScienceDirect electronic databases. The following keywords were used: pituitary gland and tuberculosis or sellar tuberculoma and children or pediatric or adolescent. We limited the results to publications from 2014 to 2025. The study is a systematic review supplemented by a new case presentation.

Results: Eight publications containing reports of 8 children with sellar/suprasellar tuberculosis were included to the review. The clinical data, including sex, age at diagnosis, country of origin, symptoms, endocrine disturbances, intracranial location, MRI findings, and treatment, were analyzed. The study was supplemented by a new case presentation in our department. Out of the 9 patients, 6 were female and 3 were male. The mean age was 10.6 years (with the range between 2 and 17 years).

Conclusions: Despite undeniable advances in medicine, the development of diagnostic and therapeutic methods, and the use of prophylactic vaccination, BCG (Bacillus Calmette-Guérin) tuberculosis is still a huge healthcare problem. Intrasellar tuberculoma presents a diagnostic challenge for clinicians since it mimics a pituitary adenoma and, therefore, should be included in the differential diagnosis. The lack of clinical and radiological data on tuberculosis, does not rule out the diagnosis of pituitary tuberculosis. The gold standard for the diagnosis of pituitary tumors is a biopsy, performed by a minimally invasive transsphenoidal approach. A delayed diagnosis may lead to permanent endocrine dysfunction. Regular follow-ups are crucial for evaluating the patient's hormone profile and may require lifelong hormone replacement.

Background and aim: Monoclonal immunoassays offer greater specificity than polyclonal assays for cortisol measurement, necessitating assay-specific cut-off limits, which have not yet been defined for the insulin tolerance test (ITT). The study aim was to define the normative cortisol response to the ITT with the first- and second-generation Roche Elecsys^®Cortisol immunoassays (Elecsys^®Cort I and II).

Methods: Ninety participants underwent an ITT after an overnight fast. Blood samples were analysed by both Elecsys^®Cort I and II. The normative cortisol cut-off limit to the ITT was defined as the 2.5th percentile of the peak cortisol level for each assay. The percentage of healthy participants with peak cortisol below the cut-off limits defined for the ACTH test, 500 nmol/L for Elecsys^®Cort I and 420 nmol/L for Elecsys^®Cort II, respectively, was calculated as the false-positive rate.

Results: The mean peak cortisol was 608 ± 102 nmol/L with Elecsys^®Cort I and 501 ± 89 nmol/L with Elecsys^®Cort II. The 2.5th percentile was 404 nmol/L with Elecsys^®Cort I and 320 nmol/L with Elecsys^®Cort II; 5th percentiles were 451 nmol/L and 348 nmol/L, respectively. False-positive rates were 15% with Elecsys^®Cort I and 18% with Elecsys^®Cort II.

Conclusion: Elecsys^®Cort II demonstrated a lower normative cortisol response to the ITT compared with Elecsys^®Cort I, and the commonly used ACTH test thresholds. These data underline the need for assay- and test-specific cut-off limits to confirm or reject adrenal insufficiency.

Background: Multiple endocrine neoplasia type 5 (MEN5) is an emerging syndrome caused by germline pathogenic variants involving the MYC Associated Factor X (MAX) gene. Affected individuals typically have pheochromocytomas, often bilateral, at a relatively early age. In MAX pheochromocytoma cohorts, pituitary adenomas are rarely reported. The role of MAX as a tumor suppressor gene in the pituitary gland has not been directly proven to date.

Methods: The propositus came from a pheochromocytoma kindred with a germline pathogenic MAX variant c.97 C>T (p.R33*). In his late thirties he developed asynchronous bilateral pheochromocytomas and underwent bilateral adrenalectomy. At age 46, he developed hyperprolactinemia (45.1 μg/L; 3x ULN) and increased IGF-1 (460 ng/mL; 1.9x ULN). Total testosterone was low (1.5 ng/mL) as was LH (1.2 IU/L). Pituitary MRI showed a microadenoma (6 mm), which was resected and his prolactin, IGF-1, and testosterone levels normalized. A Pituitary adenoma was confirmed on pathology, which showed positivity for prolactin only and a Ki67 of 2%.

Results: MAX immunohistochemical staining was lost in the pituitary adenoma cells. Tumoral DNA analysis (120X read depth) showed that at the MAX locus the pathogenic variant c.97 C>T constituted > 90% of the sequencing reads supporting tumoral loss of heterozygosity (LOH).

Conclusions: Loss of MAX staining and the identification of tumor LOH at the MAX locus confirms pituitary adenomas as a component tumor in the emerging MEN5 syndrome due to germline pathogenic MAX variants.

Purpose: Pituitary adenoma, are common intracranial neoplasms that can exhibit invasive behavior, leading to increased morbidity, recurrence, and resistance to treatment. Identifying biomarkers associated with tumor invasiveness could improve early diagnosis and guide therapeutic interventions. This review evaluates molecular biomarkers linked to pituitary adenoma invasiveness and explores the potential of radiolabeling for noninvasive detection.

Methods: A systematic search of PubMed and Embase databases was conducted to identify studies evaluating molecular markers associated with invasive pituitary adenoma. Biomarkers were selected based on their proposed role in tumor invasion, and evidence supporting their clinical relevance was summarized. Additionally, existing radiolabeling techniques for biomarker detection were reviewed.

Results: Five key biomarker groups were identified: matrix metalloproteinases (MMPs), urokinase plasminogen activator (uPA) system, myosin 5 A (MYO5A), vascular endothelial growth factor (VEGF), and survivin. MMPs were strongly linked to extracellular matrix degradation and invasion, while uPA facilitated invasion via MMP activation. MYO5A and survivin were implicated in epithelial-mesenchymal transition and tumor motility, and VEGF promoted angiogenesis. Radiolabeling techniques for MMPs, uPA/uPAR, VEGF, and survivin demonstrated feasibility for imaging tumor invasiveness, though limitations such as non-specific tracer accumulation remain.

Conclusions: This review highlights the potential of molecular biomarkers in predicting pituitary adenoma invasiveness and the emerging role of radiolabeled probes in noninvasive imaging. Future research should focus on validating these biomarkers in longitudinal studies and refining radiolabeling techniques to improve diagnostic accuracy and therapeutic targeting of invasive pituitary adenomas.

Acromegaly is a rare disease caused by the elevated and autonomous secretion of growth hormone (GH) from a pituitary somatotroph tumor or neuroendocrine tumors, and the subsequent hypersecretion of insulin-like growth factor I (IGF-I) in peripheral tissues. Excess GH and IGF-I cause several chronic and systemic complications that impact mortality, morbidity, and quality of life in patients with acromegaly. Excess GH and IGF-I play a crucial role in bone remodeling by increasing osteoclastogenesis and impairing osteoblastogenesis. Several studies have demonstrated an increased prevalence and incidence of fragility vertebral fractures (VFs) in patients with acromegaly. Long-term exposure to high levels of GH and IGF-I is recognized as a risk factor for fragility fractures in patients with acromegaly. Recent studies have shown that first- and second-generation somatostatin receptor ligands (SRLs) can reduce the incidence of vertebral fractures (i-VFs). However, a direct effect of these molecules on bone metabolism has not yet been reported. Aims: This review summarizes the results of studies investigating the frequency of i-VFs according to different GH/IGF-I-lowering drugs and the potential effects of these treatments on bone metabolism, as well as preclinical data on potential molecular pathways that interact between GH/IGF-I-lowering drugs and bone metabolism.

Following the diagnosis of craniopharyngioma, patients frequently experience rapid and excessive weight gain resulting in morbid hypothalamic obesity. This condition is commonly attributed to damage to hypothalamic structures caused either by the tumor itself or its treatment. Hypothalamic obesity should be understood and managed within the broader clinical framework of hypothalamic syndrome, a complex condition characterized by multiple neuroendocrine deficiencies, disruption of circadian rhythms, dysregulation of hunger, satiety and thirst, altered thermoregulation, as well as cognitive, sleep-related, and psychosocial impairments. The long-term outlook for affected individuals is often poor, primarily due to an elevated risk of developing metabolic syndrome, cardiovascular disease, significant reductions in health-related quality of life, and increased risk of early mortality. Management of hypothalamic syndrome remains highly challenging. Recently, a risk-adapted, personalized treatment algorithm has been proposed to guide clinical care. Therapeutic interventions such as dextroamphetamine and other centrally acting stimulants, along with glucagon-like peptide-1 receptor (GLP-1R) agonists, and setmelanotide have shown potential in promoting weight reduction. Bariatric surgery has also demonstrated efficacy; however, the use of irreversible surgical techniques in pediatric populations remains ethically and legally contentious. This report summarizes perspectives of future research and clinical progress in diagnostics, treatment, and follow-up of patients with craniopharyngioma.

The majority of prolactinomas are treated with dopamine agonists (DA) with excellent response, however DA-resistance occurs in 10% of prolactinomas. Somatostatin (SST) receptors have been increasingly studied in prolactinomas. There are five SST receptor subtypes and a significant number of prolactinomas show expression of SST₅ and SST₁ mRNA. The somatostatin analog (SSA) pasireotide, which has 40-fold greater binding affinity to SST₅ compared to first-generation SSAs, shows promising results in case reports of DA-resistant prolactinomas. This two-center retrospective cohort study investigated the expression patterns of dopamine 2 (D2R), SST₂ and SST₅ receptors in surgical specimen of 34 patients with prolactinomas, 22 of which were DA-resistant. In vitro effects of cabergoline, octreotide and pasireotide on prolactin production was also examined in cultured prolactinoma cells. Receptor expression was scored using the immunoreactivity score (IRS). 31/34(91%) patients used DA preoperatively; 22/34(64.7%) were DA-resistant. Receptor expression in the cases was 97.1% for D2R, 70.6% for SST₅ and 41.2% for SST₂. In the majority of SST₂ positive cases SST₂ expression was very low. In in vitro studies comparing the effects of octreotide, pasireotide, and cabergoline on prolactin secretion, octreotide was the least potent drug and cabergoline was the most potent. SST₅ and D2R expression was highest in prolactinomas showing the highest response to pasireotide and cabergoline in vitro (median D2R IRS 1.0 vs 8.0 for < 50% vs. > 50% inhibition by cabergoline and median SST₅ IRS 3.5 avs. 12.0 for < 50% vs. > 50% inhibition by pasireotide). In a subgroup, pasireotide inhibited prolactin secretion with comparable potency to cabergoline. Targeting SST₅ with pasireotide may be a potential treatment modality for further clinical investigation in the treatment of a subset of DA resistant or intolerant prolactinomas.

Purpose: The giant pituitary adenomas (GPAs) are still a challenging surgical problem. They comprise 10-15% of operated pituitary adenomas. The advances of endoscopic techniques allow the resection of pituitary tumors previously operated by transcranial approaches. The aim of the study was to review the surgical results in a series of patients with GPAs (diameter ≥ 40 mm) operated with endoscopic endonasal approach.

Methods: The study is a retrospective analysis of a series of 176 patients (66 women and 110 men) treated from the 2007 to 2023 by the endoscopic transsphenoidal surgeries for GPAs (> 40 mm). The mean age of the patients was 57.0 years (20-81 years), and the mean follow-up period was 7.5 years (0-16 years). Forty patients had 1a grade of the Lyon's clinicopathologic classification, 7 patients - 1b, 111 patients - 2a, and 18 patients - 2b.

Results: The gross total resection was accomplished in 73 cases (41.5%). Extended endoscopic approaches were used in 11 patients (6.3%). The mortality rate was 2.3%. Postoperatively 60% of the patients showed varying improvement in visual field defects and visual acuity. The morbidity rate was 33.5%. During a mean 7.5 years follow-up there were 29 (15.9%) cases of recurrences.

Conclusion: Our results indicate that resection with endoscopic endonasal approach can be a safe and effective method for the treatment of patients with GPAs. It is the alternative for transcranial approaches.