ArticleActa neuropathologica communications2016
Adamantinomatous and papillary craniopharyngiomas are characterized by distinct epigenomic as well as mutational and transcriptomic profiles.
Article in Acta neuropathologica communications, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 95 papers, 1 of them a synthesis that pooled it.
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Who cites it
95 citing papers in PubMed, 1 synthesis or guideline pooled it, 187 citations in OpenAlex.
- Efficacy and safety of BRAF-MEK dual inhibition in BRAF-V600E mutated papillary craniopharyngioma: a systematic review.Journal of neuro-oncology · 2025Pooled it
- Patient with two rare diseases-Renal coloboma syndrome and craniopharyngioma.Radiology case reports · 2026Article
- Pediatric endocrine disorders: a review of intracranial findings and appropriate imaging.Pediatric radiology · 2026Review
- When the cure becomes the curse: Radiation-induced glioma of the pons in children surviving craniopharyngioma.Neuro-oncology practice · 2026Article
- Development and validation of a predictive model for hypothalamic obesity following craniopharyngioma resection.BMC endocrine disorders · 2026Article
- S100A8-associated inflammatory microenvironment is related to its cell-of-origin and potentiates BRAF inhibitor resistance in papillary craniopharyngioma.Clinical and experimental medicine · 2026Article
- Lethally aggressive multiply recurrent adamantinomatous craniopharyngioma without histological malignancy: illustrative cases.Journal of neurosurgery. Case lessons · 2026Article
- Evaluating temozolomide for pediatric adamantinomatous craniopharyngiomas using microspheroid-based drug screening.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Gender differences in surgical outcomes and predictors of long term endocrine and metabolic morbidity in adults with craniopharyngioma.Pituitary · 2026Article
- A Patient-Derived Organoid Biobank of Adamantinomatous Craniopharyngioma as a Platform for Drug Discovery.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Interrogation of the cellular hierarchies reveals neoplastic evolution and therapeutic vulnerability in craniopharyngioma.Neuro-oncology · 2026Article
- Mapping influence in pediatric craniopharyngioma: the 50 most cited articles.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Review
- Mapping the evolution of pediatric craniopharyngioma research: a bibliometric analysis.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Effect of Extent of Resection and Adjuvant Radiation on Recurrence of BRAF versus β-Catenin-Mutated Craniopharyngioma: A Single Institutional Case Series.Journal of neurological surgery. Part B, Skull base · 2025Article
- Accuracy of Tumor Surveillance of Pediatric Adamantinomatous Craniopharyngioma Using Noncontrast Brain MRI.AJNR. American journal of neuroradiology · 2025Article
- Ectopic Sinonasal Adamantinomatous Craniopharyngioma in an Adolescent.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2025Article
- Targeted therapy of papillary craniopharyngioma.Medical oncology (Northwood, London, England) · 2025Review
- Impaired splicing machinery in craniopharyngiomas unveils PRPF8 and RAVER1 as novel biomarkers and therapeutic targets.Acta neuropathologica communications · 2025Article
- Targeted treatment for craniopharyngioma.Journal of neuro-oncology · 2025Review
- Tumor features in adult papillary and adamantinomatous craniopharyngioma: neuroradiological evaluation of pituitary-hypothalamic-axis dysfunction and outcome prediction.Neuroradiology · 2025Article
35 more citing papers are in PubMed but not listed here.
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14 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionCraniopharyngiomas (CP) are rare epithelial tumors of the sellar region. Two subtypes, adamantinomatous (adaCP) and papillary CP (papCP), were previously identified based on histomorphological and epidemiological aspects. Recent data indicates that both variants are defined by specific genetic alterations, and influenced by distinct molecular pathways and particular origins. The fact that CP is an uncommon tumor entity renders studies on large cohorts difficult and exceptional. In order to achieve further insights distinguishing CP variants, we conducted whole genome methylation (450 k array) and microarray-based gene expression studies in addition to CTNNB1 and BRAF mutation analysis using a comprehensive cohort of 80 adaCP and 35 papCP.
resultsBRAF V600E mutations were solely found in the papCP subgroup and were not detectable in adaCP samples. In contrast, CTNNB1 mutations were exclusively detected in adaCP. The methylome fingerprints assigned DNA specimens to entity-specific groups (papCP (n = 18); adaCP (n = 25)) matching perfectly with histology-based diagnosis, suggesting that they represent truly distinct biological entities. However, we were not able to detect within the adaCP group (including 11 pediatric and 14 adult cases) a significant difference in methylation signature by age. Integrative comparison of the papCP with the adaCP group based on differential gene expression and methylation revealed a distinct upregulation of Wnt- and SHH signaling pathway genes in adaCP.
conclusionsAdaCP and papCP thus represent distinct tumor subtypes that harbor mutually exclusive gene mutations and methylation patterns, further reflected in differences in gene expression. This study demonstrates that DNA methylation analyses are an additional method to classify CP into subtypes, and implicates a role of epigenetic mechanisms in the genesis of the respective CP variants. Detection of tumor-specific signaling pathway activation enables the possibility of target-oriented intervention.
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