Desmoplastic fibromas of bone have been described as a rare, non-metastasizing, locally aggressive, benign lesions that histologically resemble a desmoid tumour of the soft tissue. Since his original description, a number of case reports have been published, as many as 150. They are very common in mandible but here we report a fascinating case in a 21 year old young boy in the body of mandible which has grown to gigantic proportion in a short period of time and showed very aggressive radiographic presentation unlike conventional cases. In the present case, we have described clinical, radiological and histopathological features of Desmoplastic fibroma in detail. The gigantic size and aggressiveness of the lesion are the highlights in this case report. Orthopantomogram (OPG) showed a well defined radiolucent lesion extending from 35 to 38 involving lower border of mandible. The lesion has displaced the roots of 35 and 38. There is no evident root resorption. Scalloped areas were prominent in the lower border of mandible. There were no calcified foci in the radiolucent area. There is trace of mandibular canal posterior to 38 but it appears to be involved or pushed in the lesional area ( Figure 3 Keywords: As of now it is quite clear that a. It is primarily a bone pathology (Intra-osseous Origin):because a pathologic process beginning within bone and secondarily extending into soft tissues typically follows inside-out principle, where the soft tissue mass is smaller than the degree of bone destruction.b. It's a Spindle cell neoplasm: Histopathologic differentials which we have considered based on histological findings are Cellular benign fibrous histocytoma, fibromatosis, nodular fascitis, low grade fibrosarcoma, inflammatory myofibroblastic tumor.Immunohistochemistry was performed and the neoplastic cells are negative for desmin, S-100, CD-34, EMA and Ki-67 is very low i.e. less than 1/10 HPF. So based on the morphological features and exclusion of sarcomas based on immunohistochemistry findings, the final diagnosis of Desmoplastic fibroma was given. DiscussionThe desmoplastic fibroma (DF) is a rare, fibroblastic lesion of bone that resembles histological counterpart of desmoid tumor of soft tissue. 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.According to World Health Organization (WHO) Desmoplastic fibroma is defined as a locally aggressive (myo) fibroblastic lesion of bone. It is also known as the desmoid tumor of bone. 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 pre...
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