Desmoplastic fibroma of the mandible: report of three cases with a 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 occurring in the mandible: the first in a 13 year old female involving the body of the mandible in the region of #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. The clinical, histologic, immunohistochemical 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. We performed immunohistochemical stains including vimentin, smooth muscle actin, S-100 protein, 2-catenin, HHF-35 and proliferation marker, Ki-67 for all 3 cases. The potential for misdiagnosis is high, especially in the 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 lesions with current considerations in treatment and prognosis is presented.