Objectives: The desmoplastic fibroma (DF) is a rare, benign, diffuse, infiltrative proliferation of fibroblasts and mature collagen that typically occurs in the first three decades of life. Jaffe first outlined it in 1958.The mandible represents the fourth most affected site, and mandibular (84%) posterior location is favored. The clinical, radiological and histological presentation of desmoplastic fibroma can resemble various other jawbone lesions. The most common treatment for desmoplastic fibroma is block resection or wide local excision, a follow up period of 3 years is recommended. Case Report: A 12-year-old male applied to our clinic with an expanding swelling at the anterior of his mandible. The patient had a stable medical history. Extraoral examination revealed a slight expansion of the right anterior border of the mandible. Intraoral swelling resembled a hard bony texture and there was no inflammation noted. A panoramic radiograph indicated periost reaction at the lower border of the mandible and radiolucent lesion. Incisional biopsy taken. Microscopic examination pointed DF. Treatment option was marginal resection due to aggressive behavior of DP. Resection included 3 mm of healthy bone tissue around the lesion. No recurrencies was observed after 3-and 6-month follow-ups and will be followed for several years. Conclusion: The purpose of this case report is to examine the diagnosis and treatment approaches for desmoid tumors in the maxillofacial region, a condition with very few cases reported so far, in the context of existing literature.
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