The Desmoid Tumor Research Foundation

September 2024

Desmoplastic Fibroma of the Gnathic Bones-A Systematic Review

Author Information

Felippe José Almeida Loureiro 1, Wictor Rodrigues Nogueira 2, Mateus José Dutra 1, Grazielle Oliveira Stelter 2, Lauren Frenzel Schuch 3, Felipe Martins Silveira 3, Vivian Petersen Wagner 4, Manoela Domingues Martins 5, Marco Antônio Trevizani Martins 2

1Department of Oral Diagnosis, Piracicaba Dental School, Universidade de Campinas, Piracicaba, São Paulo, Brazil. 2Department of Stomatology, Porto Alegre Clinics Hospital (HCPA/UFRGS), Porto Alegre, Rio Grande do Sul, Brazil. 3Facultad de Odontologia, Universidad de la República, Montevideo, Uruguay. 4Department of Oral Pathology, School of Dentistry, University of São Paulo, São Paulo, São Paulo, Brazil. 5Department of Oral Pathology, School of Dentistry, Federal University of Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil.

Publication

Oral Dis

Summary

Objective: To summarize published information about the desmoplastic fibroma of the gnathic bones into a descriptive analysis of the main features of this condition. Material and methods: A systematic review according to the PRISMA guidelines was conducted. Electronic search was performed in four databases and in the gray literature. Case reports and case series were included. Frequencies were obtained for descriptive analysis. Results: We identified 66 articles, for a total of 96 cases. Female patients (55.8%) in the first decade of life (40.6%) with a mean age of 18.2 years were more affected. The mandible was the most affected bone with 81.2% of the cases. The main clinical feature was painless swelling (54.2%). Most of the imaging examinations (radiological, computed tomography, and magnetic resonance) showed well-defined radiolucencies (65.4%) lesions. The treatment was surgical removal in all cases. The recurrence rate was 10.8% and all in the posterior mandible. Spindle cell fibroblasts in a collagenized stroma were often described in the histopathological features. Vimentin, smooth muscle actin, and β-catenin were common immunohistochemical markers. Conclusion: Desmoplastic fibroma is a locally aggressive lesion that commonly affects the jaws in children. Histopathology is essential for diagnosis, and the pathogenesis of this tumor should be further investigated.

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