Best practice & research. Clinical endocrinology & metabolism最新文献

New advances in craniopharyngiomas 颅咽管瘤的新进展。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.102048

Niki Karavitaki

引用次数: 0

Metabolic complications and their mechanisms in patients with craniopharyngioma 颅咽管瘤患者的代谢并发症及其机制。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.101999

Eva Marie Erfurth , Hermann L. Müller

{"title":"Metabolic complications and their mechanisms in patients with craniopharyngioma","authors":"Eva Marie Erfurth , Hermann L. Müller","doi":"10.1016/j.beem.2025.101999","DOIUrl":"10.1016/j.beem.2025.101999","url":null,"abstract":"<div><div>After diagnosis of craniopharyngioma, patients frequently develop a rapid weight gain leading to morbid hypothalamic obesity due to disease- and/or treatment-associated hypothalamic lesions. Hypothalamic obesity should be diagnosed and treated in the context of hypothalamic syndrome. Hypothalamic syndrome includes neuroendocrine deficiencies, disruption of circadian rhythm, disturbed hunger-satiety and thirst feelings, temperature dysregulation, and neurocognitive, sleep and psychosocial behavioral problems. Long-term prognosis is frequently impaired by increased risk for metabolic syndrome, cardiovascular problems, severe impairments of health-related quality of life, and premature mortality. Treatment of hypothalamic syndrome is challenging. Recently, an algorithm for personalized, risk-specific treatment of hypothalamic syndrome has been published. Dextro-amphetamines and other central stimulating agents as well as glucagon-like peptide-1 receptor (GLP-1R) agonists may cause weight loss. Bariatric surgery is effective. However, non-reversible procedures are controversial due to ethical and legal considerations in minors. Hypothalamus-sparing treatment strategies and research on novel therapeutic agents for hypothalamic syndrome are warranted.</div></div>","PeriodicalId":8810,"journal":{"name":"Best practice & research. Clinical endocrinology & metabolism","volume":"39 5","pages":"Article 101999"},"PeriodicalIF":6.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144060561","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Ectopic craniopharyngiomas 异位的适应证。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.102047

Georgia Ntali (Consultant Endocrinologist and Honorary Medical Research Fellow in Endocrinology) , Taufiq Khan (Spesialist Registrar in Neurosurgery) , Niki Karavitaki (Professor of Endocrinology and Honorary Consultant Endocrinologist) , Georgios Tsermoulas (Consultant Neurosurgeon and Honorary Associate Professor in Neurosurgery)

{"title":"Ectopic craniopharyngiomas","authors":"Georgia Ntali (Consultant Endocrinologist and Honorary Medical Research Fellow in Endocrinology) , Taufiq Khan (Spesialist Registrar in Neurosurgery) , Niki Karavitaki (Professor of Endocrinology and Honorary Consultant Endocrinologist) , Georgios Tsermoulas (Consultant Neurosurgeon and Honorary Associate Professor in Neurosurgery)","doi":"10.1016/j.beem.2025.102047","DOIUrl":"10.1016/j.beem.2025.102047","url":null,"abstract":"<div><div>Craniopharyngiomas (CPs) are rare embryonic malformational tumours of the sellar/parasellar region, classified by the World Health Organization (WHO) 5th edition as grade 1 tumours. They may arise anywhere on the path of embryonic cell migration between the midline sphenoid bone and the floor of the sella turcica. On rare occasions, they can be found remotely in areas like the nasopharynx, the orbit, the posterior fossa, the brain parenchyma and the spine. Such ectopic CPs may present as primary in an atypical location, or as secondary, which represent distant recurrence after initial surgical excision in an orthotopic location. The pathogenesis of primary and secondary ectopic CPs is considered distinct, reflecting differences in developmental biology versus tumour dissemination. Primary ectopic CPs are thought to arise either from embryologically misplaced tissue or from genetically driven tumourigenesis outside the sella. Pathogenesis of secondary ectopic CPs reflects tumour cell dissemination through surgical tract implantation, cerebrospinal fluid (CSF) seeding or meningeal seeding. Ectopic cases pose unique diagnostic and therapeutic challenges. In this review which encompasses 97 cases (42 primary and 55 secondary ectopic CPs) published during the period, 2000–2025, we discuss the pathogenesis, clinical presentation, diagnosis and management of these distinctive tumours.</div></div>","PeriodicalId":8810,"journal":{"name":"Best practice & research. Clinical endocrinology & metabolism","volume":"39 5","pages":"Article 102047"},"PeriodicalIF":6.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145254157","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Decoding craniopharyngioma: From mechanisms to therapy 解码颅咽管瘤：从机制到治疗

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.102051

Jose Mario Gonzalez-Meljem (Assistant Professor) , Lei Cao (Paediatric and Adult Neurosurgeon, Associate Professor) , John Richard Apps (Associate Clinical Professor and Paediatric Neuro-Oncologist) , Juan Pedro Martinez-Barbera (Professor of Developmental Biology and Cancer)

{"title":"Decoding craniopharyngioma: From mechanisms to therapy","authors":"Jose Mario Gonzalez-Meljem (Assistant Professor) , Lei Cao (Paediatric and Adult Neurosurgeon, Associate Professor) , John Richard Apps (Associate Clinical Professor and Paediatric Neuro-Oncologist) , Juan Pedro Martinez-Barbera (Professor of Developmental Biology and Cancer)","doi":"10.1016/j.beem.2025.102051","DOIUrl":"10.1016/j.beem.2025.102051","url":null,"abstract":"<div><div>Research from the last 15 years has profoundly advanced our understanding of craniopharyngioma, a challenging tumour of the sella. Genetically and histologically distinct subtypes — adamantinomatous (ACP) and papillary (PCP) — have been decoded. ACP is primarily driven by CTNNB1 mutations, leading to β-catenin accumulation and WNT pathway activation, while PCP is characterized by BRAF-V600E mutations. Sophisticated ACP mouse models and human studies have proposed a mechanism of senescence-driven pathogenesis in which senescent epithelial cells secrete growth and inflammatory factors that orchestrate a tumour-promoting microenvironment through paracrine signalling. Single-cell RNA sequencing has confirmed this view and revealed intricate tumour ecosystems. These foundational insights are now directly informing novel therapies. Promising targeted approaches, including BRAF/MEK inhibitors for PCP and small molecules disrupting the senescence-associated secretory phenotype (SASP) in ACP are transitioning from bench to bedside, heralding a new biology-driven era for patients.</div></div>","PeriodicalId":8810,"journal":{"name":"Best practice & research. Clinical endocrinology & metabolism","volume":"39 5","pages":"Article 102051"},"PeriodicalIF":6.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145277922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Growth hormone replacement therapy in childhood-onset craniopharyngioma 生长激素替代疗法在儿童期颅咽管瘤中的应用。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.101998

Laura van Iersel Paediatric resident in training , Jiska van Schaik Paediatric resident in training , Hanneke M. van Santen Professor of Paediatric Endocrinology

引用次数: 0

The management of hypothalamic obesity in craniopharyngioma 颅咽管瘤患者下丘脑肥胖的处理。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.102018

Paul Dimitri

{"title":"The management of hypothalamic obesity in craniopharyngioma","authors":"Paul Dimitri","doi":"10.1016/j.beem.2025.102018","DOIUrl":"10.1016/j.beem.2025.102018","url":null,"abstract":"<div><div>Hypothalamic obesity (HO) is a severe, treatment-refractory metabolic disorder resulting from hypothalamic injury secondary to craniopharyngioma directly or from its surgical resection. Characterised by dysregulated energy balance from disruption of complex hypothalamic neuroregulatory circuits, hyperphagia, and reduced sympathetic tone, HO arises due to impaired leptin-melanocortin signalling and autonomic dysfunction. Conventional lifestyle modifications remain largely ineffective, necessitating pharmacotherapeutic approaches targeting neuroendocrine and metabolic pathways. Amelioration of sleep disturbances and pituitary dysfunction serve as an important foundation for management of HO. The use of dextroamphetamine in some HO patients has proved effective. Emerging therapies include melanocortin-4 receptor (MC4R) agonists such as setmelanotide, which restore anorexigenic signalling, glucagon-like peptide-1 (GLP-1) receptor agonists that enhance satiety and energy expenditure, and combination strategies integrating adrenergic modulation (Tesomet). Despite promising preliminary data, long-term efficacy and safety profiles require further validation. Optimizing precision medicine approaches incorporating polypharmacotherapy and neuroendocrine modulation may redefine therapeutic paradigms for HO management.</div></div>","PeriodicalId":8810,"journal":{"name":"Best practice & research. Clinical endocrinology & metabolism","volume":"39 5","pages":"Article 102018"},"PeriodicalIF":6.1,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144295495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Mortality in craniopharyngiomas: Data from the last two decades 颅咽管瘤的死亡率：近二十年的数据

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-09-01 DOI: 10.1016/j.beem.2025.102049

Georgia Ntali (Consultant endocrinologist and Honorary Medical Research Fellow) , Abdul Muktadir Shafi , Niki Karavitaki

引用次数: 0

Cardiometabolic outcomes of early onset hypogonadism in males 男性早发性腺功能减退的心脏代谢结果。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-07-01 DOI: 10.1016/j.beem.2025.102004

Mariska Peck (Academic Foundation Programme Doctor) , Paul Connelly (Consultant in Endocrinology) , Angela K. Lucas-Herald (Senior Lecturer in Child Health, Honorary Consultant in Paediatric Endocrinology)

引用次数: 0

The epidemiology of disorders of sex development 性发育障碍的流行病学。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-07-01 DOI: 10.1016/j.beem.2025.102002

Agnethe Berglund , Simon Chang , Marie Lind-Holst , Kirstine Stochholm , Claus Højbjerg Gravholt

引用次数: 0

Differences & disorders of sex development: A focus on outcomes 性发展的差异和障碍：关注结果。

IF 6.1 1区医学

Best practice & research. Clinical endocrinology & metabolism Pub Date : 2025-07-01 DOI: 10.1016/j.beem.2025.102029

S. Faisal Ahmed , Olaf Hiort

引用次数: 0