Desmoplastic fibroma of the mandible: a series of three cases and review of literature

Abstract

The desmoplastic fibroma (DF) is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. We present three cases of DF in the mandible: the first in a 13 year old female involving the mandibular body in the region of teeth #s 27-#28, the second in a 57 year old female with a lesion apical to tooth #30, and the third in a 20-year-old female involving the left posterior mandible. Clinical, histologic, immunohistochemical (IHC) and radiographic features of this rare neoplasm are discussed. The challenges encountered in establishing an accurate diagnosis due to significant microscopic overlap with other spindle cell lesions are also detailed. Additionally, the findings of IHC stains including vimentin, smooth muscle actin, S-100 protein, β-catenin, HHF-35 and proliferation marker, Ki-67 on 3 cases are reported. The potential for misdiagnosis is high, especially in early lesions, since immunohistochemistry has been reported in literature to be inconsistent when differentiating DFs from other spindle cell lesions. A comparative review of DF and similar entities in the jaws with current considerations in treatment and prognosis is presented.

Fig. 1

Case 1. Panoramic radiograph at initial visit demonstrating the multilocular mixed lucency between apices of teeth #27–#28 and the periapical radiograph of the same area

Fig. 2

Case 1. a Photomicrograph from the incisional biopsy sample demonstrating a spindle cell neoplasm with viable bone inside and at the periphery (H&E ×2 magnification). b Photomicrograph depicting dense and acellular fibrous connective tissue with viable bone, relatively acellular stroma with minimal vascularity and viable bone exhibiting osteocytes within lacunae (H&E ×10 magnification)

Fig. 3

Case 2. A pre-operative panoramic radiograph depicting a well-circumscribed mixed radiolucent lesion apical to teeth #s 30 and 31

Fig. 4

Case 2. a Photomicrograph of the excisional biopsy depicting a fibrous proliferation with moderate cellularity with viable bone associated with the connective tissue stroma (H&E ×20 magnification). b Photomicrograph demonstrating bland fibroblasts with no cellular pleomorphism and no mitoses (H&E ×10 magnification)

Fig. 5

Case 3. A pre-operative panoramic radiograph showing the multi-locular mixed lucent defect in the left mandibular angle and displacement of the mandibular canal and tooth #17

Fig. 6

Case 3. a Photomicrograph of a cellular neoplastic proliferation of fibroblasts in a vascular stroma. (H&E ×20 magnification). b Photomicrograph showing monomorphic, wavy, spindled cells with elongated nuclei in a background of collagenous stroma. (H&E ×20 magnification)