A case of a desmoplastic fibroma of the mandible in a 10-year old boy that was misdiagnosed as TMJ dysfunction is presented here.
Histological findings of incisional biopsy from the left retromolar area were compatible with desmoplastic fibroma.
The combined histological and immunohistochemical findings were compatible with desmoplastic fibroma of the mandible (Fig 4).
The case presented was the only desmoplastic fibroma treated in our Department (referral age up to 14-years-old), over the past 7 years and it represented 0.
Although considered a benign lesion, desmoplastic fibroma can be very aggressive locally.
Desmoplastic fibroma is generally considered a bone counterpart of aggressive fibromatosis of soft tissue (desmoid tumor).
In summary, this case demonstrates characteristic radiological and histologic features of desmoplastic fibroma, an extremely rare bone tumor, which occurred in an unusual location.
Desmoplastic fibroma of bone can imitate the pattern of a low-grade intraosseous osteosarcoma, but DF lacks osteoid matrix production, as previously discussed.
Desmoplastic fibroma is generally classified as a benign tumor and has not been shown to metastasize.
Desmoplastic fibroma of the bone: a report of two patients, review of the literature, and therapeutic implications.