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| CASE REPORT |
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| Year : 2015 | Volume
: 8
| Issue : 2 | Page : 254-257 |
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Desmoplastic fibroma of mandible
Yadavalli Guruprasad, Dinesh Singh Chauhan
Department of Oral and Maxillofacial Surgery, AME'S Dental College Hospital & Research Centre, Raichur, Karnataka, India
| Date of Web Publication | 13-Mar-2015 |
Correspondence Address:
Yadavalli Guruprasad
Department of Oral & Maxillofacial Surgery, AME'S Dental College Hospital & Research Centre, Raichur - 584 103, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0975-2870.153180
Desmoplastic fibroma is a rare benign intraosseous tumor with locally aggressive behavior. The mandible is the most frequent site of involvement. It causes bone destruction with a tendency to invade the soft-tissues if untreated. It has a high propensity for local recurrence if treated inadequately. We report a case of desmoplastic fibroma of the mandible treated with a wide margin of excision. Keywords: Benign tumor, desmoplastic, fibroma, mandible
How to cite this article:
Guruprasad Y, Chauhan DS. Desmoplastic fibroma of mandible. Med J DY Patil Univ 2015;8:254-7 |
| Introduction | |  |
Desmoplastic fibroma of bone has been described as a rare, locally aggressive, benign lesion that histologically resembles a desmoid tumor of the soft-tissue. It was initially described in 1958 by Jaffe, who separated it as a distinct entity from other intraosseous fibrous tumors. Since his original description, a number of small series and case reports have brought the total number of published cases to approximately 150. This tumor constitutes <1% of bone tumors and about 0.3% of all benign osseous tumors and it usually involves the tibia, scapula and femur. [1] The mandible represents the fourth most commonly affected site. It has a predilection to affect the posterior part of the mandible, most often in the ramus-angle region. Desmoplastic Fibroma of the jaws is most commonly discovered in the second and third decades with the mean age of 15.7 years and with slight female predilection. [2]
| Case Report | | |
This was a report of a 42-year-old female patient presented with the history of swelling in the right lower jaw of 1 year duration, which was of spontaneous origin. The swelling was progressive in size and firm in consistency, confined to the anterior mandible on the right side [Figure 1]. She complained of mild pain over the swelling; however she was not finding any difficulty in opening the mouth. On examination there was a firm swelling present on the right side of the jaw with well-defined margins, fixed to the mandible. Skin over the swelling was normal; intra-orally it was extending from anterior teeth to right first molar obliterating the buccal vestibule [Figure 2]. Orthopantomograph (OPG) showed well-defined radiolucent lesion over the right mandible in relation to right premolar and anterior teeth [Figure 3]. Computed tomography (CT) axial view showing radiolucent lesion on right mandible causing expansion and thinning of both cortical plates and perforation of lingual cortical plate in anterior mandible [Figure 4]. Three-dimensional CT frontal and lingual view showing well-defined radiolucent lesion over the right mandible in relation to right premolar and anterior teeth [Figure 5] and [Figure 6]. Excision with aggressive curettage was planned under general anesthesia using intraoral approach and peripheral osteotomy was carried out with wide margins of normal bone was also resected. The excised specimen was subjected to histopathological examination which was suggestive of desmoplastic fibroma [Figure 7],[Figure 8] and [Figure 9]. Histopathological picture showed fascicles of mature fibroblasts with collagen matrix [Figure 10]. Post-operative OPG was taken after 3 months which showed normal bony margins without any signs of recurrence [Figure 11]. | Figure 1: Frontal view of the patient presenting with facial asymmetry due to extensive swelling (arrows) on the right mandible
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 | Figure 2: Intraoral photograph showing swelling over the right mandible (arrows) causing obliteration of the buccal vestibule
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 | Figure 3: Orthopantomograph showing well-defined radiolucent lesion (arrows) over the right mandible in relation to right premolar and anterior teeth
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 | Figure 4: Computed tomography axial view showing radiolucent lesion (arrows) on right mandible causing expansion and thinning of both cortical plates and perforation of lingual cortical plate in anterior mandible
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 | Figure 5: Three-dimensional computed tomography Frontal view showing well-defined radiolucent lesion (arrows) over the right mandible in relation to right premolar and anterior teeth
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 | Figure 6: Three-dimensional computed tomography lingual view showing perforation (arrows) of lingual cortical plate in anterior mandible
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 | Figure 7: Intra-operative photograph showing exposure (arrows) of the tumor mass
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 | Figure 8: Intra-operative photograph showing the surgical site (arrows) after tumor excision using peripheral osteotomy
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 | Figure 10: Histopathological picture showing fascicles of mature fibroblasts (arrows) with collagen matrix (H and E, ×100 magnification
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 | Figure 11: Post-operative orthopantomograph showing margins after tumor excision after 3 months
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| Discussion | | |
In the head and neck desmoid fibromatosis may be intraosseous (desmoplastic fibroma) or, more often, in soft-tissue, with the highest incidence in the supraclavicular region of the neck. High recurrence and persistence rates, 50% or more, accompany intralesional or marginal excision. [3]
These tumors reside in a clinical grey zone between benign fibrous lesion, e.g., keloids and malignant tumors. This is reflected, in part, by synonyms for desmoid fibromatosis: Desmoma, aggressive fibromatosis, fibrosarcoma grade 1, desmoid type and desmoplastic fibroma of bone. [4],[5] Desmoplastic fibroma of the jaw presents in the same manner as its counterpart in the long bones. The age incidence is usually in the first, second, or third decade. Neither sex is at greater risk. The site of predilection within the jawbone is the mandible, while the maxilla is rarely affected. The posterior mandible is most frequently involved (the ramus, angle and molar area). The premolar area and the anterior segments are less commonly affected. The initial symptoms include painful swelling of the jaw and occasionally loss of teeth. Radiographically, a well demarcated lytic lesion is seen. It is usually multilocular and often expands the bone. The radiographic differential diagnosis includes ameloblastoma, odontogenic fibroma, aneurysmal bone cyst and hemangioma. [5] Only rarely will primary malignant lesions such as fibrosarcoma or malignant fibrous histiocytoma be suspected on the basis of radiographic evidence. The histological features of desmoplastic fibroma and the extra-abdominal desmoid tumor are essentially identical. They are characterized by uniform-appearing fibroblastic cells in a stroma containing various amounts of collagen fibers. The morphologic differential diagnosis includes benign and malignant spindle cell tumors of bone. Fibrous dysplasia can stimulate desmoplastic fibroma in areas where fibrous tissue predominates and osteoid production is not apparent. The distinction can be made by recognizing areas of bone formation by additional sampling. Also the nuclei in fibrous dysplasia are shorter and more compact-looking than the elongated, slender nuclei seen in desmoplastic fibroma. Low grade intraosseous osteosarcoma, another tumor that can mimic desmoplastic fibroma, can also be excluded by identification of bone formation. Nonossifying fibroma and solitary congenital fibromatosis of bone can be confused with desmoplastic fibroma. Low grade fibrosarcoma poses the most difficult problem in the histologic differential diagnosis; in fact, the distinction may not always be possible and can only be detected when it recurs and metastasizes. However, fibrosarcoma is more cellular, with a recognizable herringbone pattern and plumper, larger cells than those in desmoplastic fibroma. [5],[6]
CT or magnetic resonance imaging is suggested for detection of the exact soft-tissue extension of the lesion. The radiographic appearance may not be effective in differentiation of this lesion from malignant lesion such as fibrosarcoma, malignant histiocytoma and osteosarcoma. The most common treatment for desmoplastic fibroma being a block resection or wide local excision, with enucleation rarely used. [6],[7] The infrequent use of enucleation is due to the concern that recurrences rates are higher, approximately 20-40% compared to a minimal recurrence rate in block resection or wide local excision. Jaffe, in his discussion of the treatment of desmoplastic fibroma of bone, recommended segmental resection as the treatment of choice and noted that if the lesion is curetted and recurs, segmental resection or a more thorough curettage should be performed. [8] Wide resection or a thorough "marginal" curettage was the preferred method of treatment while local or limited curettage often led to continued growth of the tumor. It has been observed by Tandon et al., that curettage or peripheral ostectomy achieved with a burr drill achieves better local tumor control. [8]
| Conclusion | | |
Desmoplastic fibroma is a rare, well-differentiated fibrous tumor with a slow but aggressive potential for growth. This lesion, while incapable of metastasizing, may recur locally when incompletely excised and thorough curettage with possible widening of margin with a bur (peripheral osteotomy) is the treatment of choice in early lesions, lesion growing outside the reactive bony rim requires wide excision.
| References | | |
| 1. | Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW. Desmoplastic fibroma of bone: Radiographic analysis. Radiology 1989;172:827-32.  |
| 2. | Griffith JG, Irby WB. Desmoplastic fibroma. report of a rare tumor of the oral structures. Oral Surg Oral Med Oral Pathol 1965;20:269-75. |
| 3. | Batsakis JG, Raslan W. Extra-abdominal desmoid fibromatosis. Ann Otol Rhinol Laryngol 1994;103:331-4. |
| 4. | Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G. Desmoid fibromatosis: A clinicopathologic study of 25 children. Semin Diagn Pathol 1986;3:138-50. |
| 5. | Inwards CY, Unni KK, Beabout JW, Sim FH. Desmoplastic fibroma of bone. Cancer 1991;68:1978-83. |
| 6. | Reid EN, Lawoyin DO, Suresh L, Longwe E. Desmoplastic fibroma of the anterior mandible. Case report and review of literature. N Y State Dent J 2009;75:32-3. |
| 7. | Summa A, Cerasti D, Crisi G, Ormitti F, Ventura E, Sabato M. Desmoplastic fibroma of the mandible: Usefulness of CT and MR imaging in diagnosis and treatment. A case report. Neuroradiol J 2010;23:109-13. |
| 8. | Tandon S, Garg RK. Intraoral desmoplastic fibroma: A manifestation of tuberous sclerosis. Fetal Pediatr Pathol 2012;31:195-201. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]
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