Pituitary

Pituitary tuberculosis in the pediatric population: a systematic review and case report
Gierlotka A, Sordyl R, Bielecki I and Mandera M
This review aims to identify and evaluate the management options for pituitary tuberculosis in the pediatric population.
Defining normative cortisol responses to the insulin tolerance test: A comparison of ElecsysCort I and II assays
Okutan S, Borresen SW, Hilsted L, Feldt-Rasmussen U and Klose M
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 ElecsysCortisol immunoassays (ElecsysCort I and II).
Patient perceptions of pituitary incidentaloma diagnosis and follow-up: a Pituitary Society international patient survey
Fukuoka H, Glezer A, Karavitaki N, McCormack A, Gurnell M, Greenman Y, Langlois F, Schwartz TH, Melmed S and Fleseriu M
Detection of pituitary incidentalomas is increasing in frequency with the use of advanced imaging techniques. As an adjunct to publication of consensus guidelines on management of pituitary incidentalomas, the Pituitary Society sought to understand patient perceptions of their diagnosis, prognosis, and follow-up.
Sleep disturbances in craniopharyngioma: a systematic review and meta-analysis
Liang Q, Yang T, Hu T, Spruyt K, Zhang X and Li Q
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.
Sex-Specific transcriptomic changes in adipose tissue following adult-onset disruption of growth hormone receptor
Duran-Ortiz S, Young JA, List EO, Basu R, Walsh C, Gotte EA, Berryman DE and Kopchick JJ
Reduction in growth hormone (GH) signaling throughout life is known to extend lifespan and enhance healthspan in mice, and congenital GH receptor (GHR) mutations in both mice and humans confer protection against age-related diseases such as cancer, diabetes, and cognitive decline. To explore the health effects of disrupting GH action during adulthood, we previously generated adult-onset GHR knockout (6mGHRKO) mice by ablating GHR at 6 months of age. Both male and female 6mGHRKO mice exhibited reduced oxidative stress, with males showing improved insulin sensitivity and resistance to cancer, while females demonstrated extended lifespan. In the current study, we performed RNA sequencing on subcutaneous adipose tissue (Subq AT) from 6mGHRKO and control mice to investigate molecular mechanisms underlying these health benefits. Differential gene expression, gene ontology, pathway enrichment, and upstream regulator analyses revealed that GHR ablation predominantly downregulated gene expression, particularly in males. Sex-specific gene expression differences were more pronounced in control mice than in 6mGHRKO counterparts. Among the enriched processes, pathways related to extracellular matrix (ECM) organization emerged as differentially regulated between sexes and genotypes. These transcriptomic findings are exploratory and hypothesis-generating, highlighting ECM remodeling as a potential area for future mechanistic validation.
Somatostatin 5 receptor expression in prolactinomas: Is there a role for Pasireotide in the management of prolactinomas?
Agrawal N, Mehta S, Feelders RA, Skwiersky S, Campana C, Dogan F, van Koetsveld PM, Neggers SJCMM, Wright K, Kim H, Zagzag D and Hofland LJ
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 SST and SST mRNA. The somatostatin analog (SSA) pasireotide, which has 40-fold greater binding affinity to SST 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), SST and SST 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 SST and 41.2% for SST. In the majority of SST positive cases SST 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. SST 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 SST 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 SST with pasireotide may be a potential treatment modality for further clinical investigation in the treatment of a subset of DA resistant or intolerant prolactinomas.
Acromegaly treatment and bone: a bidirectional relationship
Chiloiro S, Palumbo C, Giampietro A, De Marinis L, Bianchi A, Giustina A and Pontecorvi A
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.
Craniopharyngioma - What's next
Müller HL
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.
Radiolabeling molecular biomarkers of invasive pituitary adenomas: a narrative review
Marche C, Knop JCM, Khanapur S, Suero Molina E, McCord M, Smith V and Catalino MP
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.
Loss of heterozygosity and absence of MAX immunostaining in a prolactinoma associated with multiple endocrine neoplasia type 5 (MEN5)
Delemer B, Florea SM, Decoudier B, Boulagnon-Rombi C, Karna B, Pellegata NS, Buffet A, Beckers A, Pétrossians P and Daly AF
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.
Endoscopic endonasal resection of giant pituitary adenomas - case series from the referral pituitary center
Kunicki J, Buchalska B, Maksymowicz M, Baluszek S and Mandat T
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.
SAGIT, ACRODAT, PANOMEN 3… time to implement tools for management of acromegaly..?
Chanson P
Exercise ventilatory inefficiency may be a specific feature of patients with acromegaly
Dorelli G, Venturelli M, Sartori G, Fantin A, Cominacini M, Braggio M, Dalle Carbonare L, Piccinno E, Davì MV, Schena F and Crisafulli E
Reduction in exercise capacity and peak oxygen uptake (V̇O) are common in acromegaly, but ventilatory alterations during exercise remain unstudied. We evaluated the exercise ventilatory response in terms of efficiency in patients with acromegaly.
Authors' reply to the letter "Should children born small for gestational age without catch-up growth be tested for growth hormone deficiency?"
Arlien-Soborg MC, Boguszewski M, Jorgensen J and
Mortality in patients with dopamine agonist-treated hyperprolactinemia: a large matched-cohort study
Ayalon-Dangur I, Shimon I, Shochat T, Kushnir S, Rudman Y, Dotan I, Agrawal N, Fleseriu M and Akirov A
Data on mortality risk in patients with hyperprolactinemia is limited. This study aimed to evaluate all-cause mortality in a cohort of dopamine agonist (DA)-treated patients with hyperprolactinemia.
Severe symptomatic arterial vasospasm following pituitary surgery: a rare case and systematic review of the literature
Doat-Sarfati V, Lefevre E, Chiaroni PM, Shotar E, Abdennour L, Degos V and Jacquens A
Pituitary adenomas are common, and transsphenoidal surgery remains the standard treatment for symptomatic cases. While most postoperative complications are well described, symptomatic arterial vasospasm after pituitary surgery is exceptionally rare but potentially life-threatening, and no consensus exists regarding its management.
Less invasive skull base reconstruction using gelatin sponge and collagen matrix to prevent cerebrospinal fluid leakage after endoscopic transsphenoidal surgery: experience in 558 cases
Yonenaga M, Fujio S, Makino R, Sugata J, Hanada T, Nagano Y, Higa N, Yamahata H, Arita K, Yoshimoto K and Hanaya R
We evaluated the effectiveness of a less invasive skull base reconstruction technique using a fibrin glue-soaked gelatin sponge (FGGS) and collagen matrix to prevent cerebrospinal fluid (CSF) leakage following endoscopic transsphenoidal surgery (ETSS). This approach minimizes the need for fascia harvesting and the use of nasoseptal flaps.
A comparison of bilateral internal jugular vein sampling with bilateral inferior petrosal sinus sampling at Mayo Clinic for the subtype evaluation of corticotropin-dependent cushing syndrome
O'Keeffe DT, Maraka S, Kotwal A, Cloft H, Kallmes DF, Bancos I, Chang AY, Erickson D and Young WF
Distinguishing between pituitary corticotropin (ACTH)-dependent Cushing disease (CD) and ectopic ACTH-dependent Cushing syndrome (EADCS) is diagnostically challenging. This study compared the diagnostic performance of bilateral inferior petrosal sinus sampling (BIPSS) and bilateral internal jugular vein sampling (BIJVS).
Prevalence and evolution of hypertension in a large Iberian cohort of patients with acromegaly
Araujo-Castro M, García-Centeno R, González L, Lacerda Nobre E, de Griné Severino M, Goi J, Pérez Pena MM, Menéndez Torre E, Lozano Aida C, Novoa-Testa I, Cordido F, Sánchón R, Guerrero-Pérez F, Oliva Rodríguez R, Rodríguez-Jiménez B, Ollero García MD, Irigaray Echarri A, Simó-Servat A, Moure Rodríguez MD, Novo-Rodríguez C, Calatayud M, Villar-Taibo R, Bernabéu I, Tenorio-Jimenéz C, González Molero I, Iglesias P, Blanco C, Vidal-Ostos De Lara F, Aulinas A, Asla Roca Q, Paja Fano M, Rivera Martínez A, Cardoso LM, Marques P, Giustina A, Marazuela M, Puig-Domingo M and Biagetti B
To analyze the prevalence of hypertension in patients with acromegaly and assess the impact of acromegaly treatment on blood pressure (BP) outcomes.
Is the somatostatin receptor type 5 expression important in resistant prolactinomas?
Chinezu L and Zatelli MC
Management of water and electrolyte disorders post-pituitary adenoma surgery: experience with Tolvaptan 7.5 mg at a tertiary care centre
Yamichannaiah C, Sheth KV, Lila AR, Sarathi V, Patil V, Memon SS, Barnabas R, Karlekar M, Phadte A, Sharma A and Bandgar T
Water and electrolyte disorders (WED) are common after pituitary surgery, increasing morbidity and hospital stay. We report our single-centre experience, with emphasis on tolvaptan use for syndrome of inappropriate antidiuresis (SIAD).