ArticleEndocrine-related cancer2019
SHH pathway inhibition is protumourigenic in adamantinomatous craniopharyngioma.
Article in Endocrine-related cancer, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 38 citations in OpenAlex.
- Aging increases ovarian cancer growth, metastasis, and immunosuppression that can be alleviated by inhibiting hedgehog signaling.bioRxiv : the preprint server for biology · 2026Article
- Loss of primary cilia in late pituitary organogenesis does not cause endocrine dysfunction.Journal of anatomy · 2026Article
- The future of targeted therapies for pediatric central nervous system tumors.Neuro-oncology advances · 2026Review
- Spatially Resolved Multiomics Reveals Metabolic Remodeling and Autophagy Activation in Adamantinomatous Craniopharyngiomas.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 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
- Pharmacological Treatment for Adamantinomatous Craniopharyngioma.Current oncology reports · 2025Review
- Targeted Treatment of Pediatric Craniopharyngioma: Lesson Learnt After 20-Year Experience with Intracystic Interferon Alpha.Advances and technical standards in neurosurgery · 2025Article
- Recent advances in craniopharyngioma pathophysiology and emerging therapeutic approaches.Frontiers in endocrinology · 2025Review
- Sonic hedgehog signalling pathway in CNS tumours: its role and therapeutic implications.Molecular brain · 2024Review
- Adamantinomatous craniopharyngioma: evolution in the management.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Review
- Pituitary Tumorigenesis-Implications for Management.Medicina (Kaunas, Lithuania) · 2023Review
- The molecular pathogenesis of craniopharyngiomas.Archives of endocrinology and metabolism · 2023Review
- Molecular biological features of cyst wall of adamantinomatous craniopharyngioma.Scientific reports · 2023Article
- The hidden hedgehog of the pituitary: hedgehog signaling in development, adulthood and disease of the hypothalamic-pituitary axis.Frontiers in endocrinology · 2023Review
- Treatment of Cystic Craniopharyngiomas: An Update.Advances and technical standards in neurosurgery · 2022Article
- Medical Therapy for Craniopharyngiomas.TouchREVIEWS in endocrinology · 2021Review
- Clinical impact of craniopharyngioma classification based on location origin: a multicenter retrospective study.Annals of translational medicine · 2021Article
- Adamantinomatous craniopharyngioma as a model to understand paracrine and senescence-induced tumourigenesis.Cellular and molecular life sciences : CMLS · 2021Review
- Status Quo and Research Trends of Craniopharyngioma Research: A 10-Year Bibliometric Analyses (From 2011 to 2020).Frontiers in oncology · 2021Article
Corrections and comments
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Authors and funding
17 authors at 4 institutions in 3 countries.
Funding
Abstract
Pharmacological inhibition of the sonic hedgehog (SHH) pathway can be beneficial against certain cancers but detrimental in others. Adamantinomatous craniopharyngioma (ACP) is a relevant pituitary tumour, affecting children and adults, that is associated with high morbidity and increased mortality in long-term follow-up. We have previously demonstrated overactivation of the SHH pathway in both human and mouse ACP. Here, we show that this activation is ligand dependent and induced by the expression of SHH protein in a small proportion of tumour cells. We investigate the functional relevance of SHH signalling in ACP through MRI-guided preclinical studies using an ACP mouse model. Treatment with vismodegib, a clinically approved SHH pathway inhibitor, results in a significant reduction in median survival due to premature development of highly proliferative and vascularised undifferentiated tumours. Reinforcing the mouse data, SHH pathway inhibition in human ACP leads to a significant increase in tumour cell proliferation both ex vivo, in explant cultures, and in vivo, in a patient-derived xenograft model. Together, our results demonstrate a protumourigenic effect of vismodegib-mediated SHH pathway inhibition in ACP.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.