ReviewAnnals of maxillofacial surgery
Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome.
Review in Annals of maxillofacial surgery. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 35 citations in OpenAlex.
- An evidence-based surgical algorithm for management of odontogenic keratocyst.Oral and maxillofacial surgery · 2023Pooled it
- Multiple Basal Cell Carcinomas in an Adolescent with an MLPA-DetectedBalkan journal of medical genetics : BJMG · 2026Article
- Multiple Odontogenic Keratocysts as the Initial Manifestation of Gorlin Syndrome in a Child: A Case Report With Conservative Management and Diagnostic Considerations.Case reports in dentistry · 2026Article
- Low-dose oral minoxidil for the management of vismodegib-induced alopecia.JAAD case reports · 2025Article
- Skin Signals: Exploring the Intersection of Cancer Predisposition Syndromes and Dermatological Manifestations.International journal of molecular sciences · 2025Review
- Article
- Tolerance of sonidegib after intolerance of vismodegib-Experience in two patients with nevoid basal cell carcinoma syndrome (Gorlin syndrome).Skin health and disease · 2023Article
- The Role of 5-Fluorouracil in Preventing Recurrence After Enucleation of Odontogenic Keratocyst: A Case Report.Cureus · 2023Article
- Review
- Basal Cell Carcinoma and Hedgehog Pathway Inhibitors: Focus on Immune Response.Frontiers in medicine · 2022Review
- Challenges in Treating Genodermatoses: New Therapies at the Horizon.Frontiers in pharmacology · 2021Review
- Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research.International journal of molecular sciences · 2020Review
- Immunohistochemical Characterization of Reactive Epithelial Changes in Odontogenic Keratocysts.Pathology oncology research : POR · 2020Article
- Serial ultra-deep sequencing of circulating tumor DNA reveals the clonal evolution in non-small cell lung cancer patients treated with anti-PD1 immunotherapy.Cancer medicine · 2019Article
- Effect of the sonic hedgehog inhibitor GDC-0449 on an in vitro isogenic cellular model simulating odontogenic keratocysts.International journal of oral science · 2019Article
- The relevance of a suppressor of fused (SUFU) mutation in the diagnosis and treatment of Gorlin syndrome.JAAD case reports · 2018Article
- Targeted therapy of gastroenteropancreatic neuroendocrine tumours: preclinical strategies and future targets.Endocrine connections · 2018Review
- Development of mediastinal lymphoma after radiotherapy for concurrent medulloblastoma and PNET in a patient with Gorlin syndrome.World journal of surgical oncology · 2016Article
- Molecular Signaling in Benign Odontogenic Neoplasia Pathogenesis.Current oral health reports · 2016Article
- Transcriptome Variability in Keratocystic Odontogenic Tumor Suggests Distinct Molecular Subtypes.Scientific reports · 2016Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Vismodegib hedgehog signaling inhibition treatment has potential for reducing the burden of multiple skin basal cell carcinomas and jaw keratocystic odontogenic tumors. They are major criteria for the diagnosis of Gorlin syndrome, also called nevoid basal cell carcinoma syndrome. Clinical features of Gorlin syndrome are reported, and the relevance of hedgehog signaling pathway inhibition by oral vismodegib for maxillofacial surgeons is highlighted. In summary, progressed basal cell carcinoma lesions are virtually inoperable. Keratocystic odontogenic tumors have an aggressive behavior including rapid growth and extension into adjacent tissues. Interestingly, nearly complete regression of multiple Gorlin syndrome-associated keratocystic odontogenic tumors following treatment with vismodegib. Due to radio-hypersensitivity in Gorlin syndrome, avoidance of treatment by radiotherapy is strongly recommended for all affected individuals. Vismodegib can help in those instances where radiation is contra-indicated, or the lesions are inoperable. The effect of vismodegib on basal cell carcinomas was associated with a significant decrease in hedgehog-signaling and tumor proliferation. Vismodegib, a new and approved drug for the treatment of advanced basal cell carcinoma, is a specific oncogene inhibitor. It also seems to be effective for treatment of keratocystic odontogenic tumors and basal cell carcinomas in Gorlin syndrome, rendering the surgical resections less challenging.
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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.