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, bcatenin, 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.

A Gigantic Desmoplastic Fibroma Resembling A Malignant Neoplasm: An Intriguing Case Report

“…Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, as seen in the current case report and has a moderate to high recurrence rate [6,7]. Activating CTNNB1 hotspot mutations or APC mutations are driving events [8,9] for these locally aggressive tumors.…”

“…It is seen in patients aged less than 30 years. Desmoplastic fibromas are very common in mandible as they constitute approximately 86% cases in mandible [4] with a predilection for the ramus and angle region, Usually the lesions are slow growing [6] and radiographically well defined but the presence of such a massive lesion which has grown in such a short time span with multiple buccal and lingual cortical plate perforations and infiltration of floor of mouth and soft tissues is a very rare finding [10] when we compare current case report with past cases.…”

Multiple Bone Lesions in an 8-Month-Old Child Presenting with Pathologic Fracture

“…Immunohistochemistry is positive for smooth muscle and muscle-specific actins, but negative for S100, desmin, and CD34. Beta-catenin has variable expression in the osseous lesions, with nuclear expression in the soft-tissue counterpart of up to 80% to 90% [13][14][15][16] .…”

Fibrome desmoïde mandibulaire : savoir y penser !

“…Un trismus peut être expliqué par un envahissement des muscles masticateurs, comme cela a été rapporté dans notre 4 e observation. Les troubles de la sensibilité sont exceptionnels dans les localisations mandibulaires [1,3,7]. Aucun de nos patients ne rapportait de troubles sensitifs.…”

“…Cependant, seule l'histologie complétée par une analyse immunohistochimique permet de poser le diagnostic. Le traitement est chirurgical et la qualité de ce traitement conditionne la survenue de récidives ultérieures [1].…”