Desmoplastic fibroma (DF) is a rare benign bone tumor adopting an aggressive behavior, representing a challenge for clinical and radiographic diagnosis. This case report focused on a 31-year-old man with a large mandibular lesion with severe displacements of the mandibular teeth. Only a combination of paraclinical findings allows a definitive diagnosis to be made. Cervicofacial MRI revealed a low T1 signal intensity with peripheral enhancement after Gadolinium, and T2 hyperintense signal, while PET scan showed a moderate metabolism. Bone biopsy with immunohistochemical analysis allowed for definitive diagnosis of DF after eliminating the main differential diagnosis (fibrous dysplasia, fibrosarcoma, desmoid tumor, and osteosarcoma). The patient was successfully treated by large mandibular resection and reconstruction with a free-fibular bone flap".
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| http://dx.doi.org/10.1016/j.jormas.2024.101805 | DOI Listing |
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Article Synopsis
- The study aims to compile and summarize information about desmoplastic fibroma in the jaw bones, focusing on its key characteristics.
- A systematic review identified 66 articles detailing 96 cases, revealing a higher prevalence in females and younger patients, particularly affecting the mandible, with painless swelling as the primary symptom.
- Surgical removal is the standard treatment with a recurrence rate of 10.8%, and histopathological analysis shows specific cellular and molecular features that require more research for understanding the tumor's development.
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