Benign mesenchymal odontogenic tumors

2025-11-18 Paola Dal Cin, PhD Affiliation

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

{FOS} rearrangement ,{CDC73} mutation,{PRKAR1A} mutations

Odontogenic and maxillofacial bone tumors	Genetic marker(s)
Benign mesenchymal odontogenic tumors
Odontogenic fibroma	No pathogenic mutations were identified sofar in odontogenic fibroma.¹
Cementoblastoma	Cementoblastoma shows c-FOS overexpression and harbors the same FOS rearrangement as osteoblastoma, a histologic mimicker.²
Cemento-ossifying fibroma (COF)	A variety of infrequent molecular alterations have been reoprted so far e.g., 1) CDC73 a.k.a. CDC73 mutation in a minority of the cases , usually as part of hyperparathyroidism-jaw tumour syndrome OMIM:145001 or ANO5 mutation as part of gnathodiaphyseal dysplasia,OMIM:16200 2 ) copy number alterations (CNAs) involving chromosomes 7 and 12; 3) no recurrent fusions or pathogenic mutations have been so far identified. ³
Odontogenic myxoma	A small subset of odontogenic myxomas harbor PRKAR1A mutations.⁴ Most instead show recurrent copy number alterations,⁵ specifically recurrent extensive haploidization.⁶ Methylation analysis demonstrates odontogenic myxoma to form a distinct cluster from other tumor types in the differential diagnosis.⁵

Reference Number	Pubmed ID	Last Year	Title	Authors
1	28597929	2017	Next-generation sequencing of oncogenes and tumor suppressor genes in odontogenic myxomas.	Santos JN et al
2	33653978	2021	FOS Rearrangement and Expression in Cementoblastoma.	Lam SW et al
3	37995913	2024	Comprehensive Genomic Analysis of Cemento-Ossifying Fibroma.	Gomez RS et al
4	25870248	2015	Defects of the Carney complex gene (PRKAR1A) in odontogenic tumors.	Sousa SF et al
5	39289817	2024	Odontogenic Myxomas Harbor Recurrent Copy Number Alterations and a Distinct Methylation Signature.	Kleijn TG et al
6	40071647	2025	Odontogenic Myxoma Harbors Widespread Loss of Heterozygosity and Not Trisomies.	Odintsov I et al

Paola Dal Cin, PhD

Benign mesenchymal odontogenic tumors

Atlas Genet Cytogenet Oncol Haematol. 2025-11-18

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