Familial Florid Cemento‐Osseous Dysplasia: A Systematic Review Based on Contemporary Diagnostic Criteria
Paula de Sousa Lopes Cascaes, Sofia Junqueira Weigel, Monikelly do Nascimento, Gustavo Pereira Tavares, Andresa Borges Soares, José Luiz Cintra Junqueira, Mariana Quirino Silveira SoaresABSTRACT
Purpose
Familial florid cemento‐osseous dysplasia (FFCOD) is a rare fibro‐osseous jaw disorder characterized by multifocal lesions and familial aggregation. This systematic review aimed to synthesize the evidence on the demographic, clinical, radiographic, histopathological, genetic, therapeutic, and follow‐up characteristics of FFCOD.
Methods
A systematic review was conducted in accordance with PRISMA 2020 guidelines and registered in PROSPERO (CRD420261295922). Searches were performed in PubMed, Embase, Scopus, Web of Science, Cochrane Library, SciELO, LIVIVO, ProQuest, OpenGrey, IBICT/BDTD, Google Scholar, and reference lists through April 2025. Studies following the current World Health Organization (WHO) diagnostic criteria were eligible. Two independent reviewers performed study selection, data extraction, and risk‐of‐bias assessment using the Joanna Briggs Institute critical appraisal tool for case reports.
Results
Eight studies comprising 25 affected individuals were included. Females accounted for 60.0% of cases, and the mean age was 34.8 ± 19.9 years. Multifocal involvement of both jaws (48.0%) and all four quadrants (32.0%) was common. All included cases exhibited a mixed radiolucent‐radiopaque appearance (100.0%), while bone expansion was the most frequent clinical manifestation (33.3%). Histopathological examination consistently demonstrated fibrocellular stroma associated with cemento‐ and/or bone‐like mineralized material. Molecular analysis was reported in one study and identified an ANO5 missense variant. Osteomyelitis, osteonecrosis, and bone exposure/sequestration were the most frequently reported complications.
Conclusion
FFCOD demonstrates consistent clinicopathological characteristics, including multifocal jaw involvement, bone expansion, and a mixed radiolucent‐radiopaque appearance. Although many individuals remain asymptomatic, infectious and necrotic complications may occur, emphasizing the importance of early recognition, careful treatment planning, and long‐term clinical and radiographic follow‐up.