Benign mixed epithelial and mesenchymal odontogenic tumors

2025-11-18 Paola Dal Cin, PhD Affiliation

1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)

{BRAF} p.V600E mutation,{CTNNB1} mutation

Odontogenic and maxillofacial bone tumors	Genetic marker(s)
Benign mixed epithelial and mesenchymal odontogenic tumors
Odontoma	Odontoma does not harbour BRAF p.V600E mutation ¹ They occur in patients with familial adenomatous polyposis OMIM:175100, Schimmelpenning syndrome OMIM:163200, odontoma-dysphagia syndrome OMIM:164330 and encephalocraniocutaneous lipomatosis OMIM:613001 syndromes.
Primordial odontogenic tumor	No mutations were identified sofar in primordial odontogenic tumor. ²
Ameloblastic fibroma	BRAF p.V600E mutation has been detected in meloblastic fibromas. ³ It may undergo malignant transformation into ameloblastic fibrosarcoma with frequent BRAF and occasional NRAS mutations. ⁴
Dentinogenic ghost cell tumor	CTNNB1 mutations have been reported in dentinogenic ghost cell tumor. ⁵

Reference Number	Pubmed ID	Last Year	Title	Authors
1	32966885	2020	Targeted Next-Generation Sequencing and Allele-Specific Quantitative PCR of Laser Capture Microdissected Samples Uncover Molecular Differences in Mixed Odontogenic Tumors.	Coura BP et al
2	29908099	2018	Pathogenesis of primordial odontogenic tumour based on tumourigenesis and odontogenesis.	Mikami T et al
3	35048058	2021	The Molecular Pathology of Odontogenic Tumors: Expanding the Spectrum of MAPK Pathway Driven Tumors.	Guimarães LM et al
4	31899815	2020	Ameloblastic fibrosarcoma: clinicopathological and molecular analysis of seven cases highlighting frequent BRAF and occasional NRAS mutations.	Agaimy A et al
5	34128137	2021	Comparative Analysis Between Dentinogenic Ghost Cell Tumor and Ghost Cell Odontogenic Carcinoma: A Systematic Review.	de Souza Vieira G et al

Paola Dal Cin, PhD

Benign mixed epithelial and mesenchymal odontogenic tumors

Atlas Genet Cytogenet Oncol Haematol. 2025-11-18

Cookies