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 Table of Contents  
Year : 2014  |  Volume : 26  |  Issue : 2  |  Page : 222-224

Desmoplastic fibroma of the mandible: A rare case report

1 Department of Oral Medicine and Radiology, Meghna Institute of Dental Sciences, Nizamabad, Telangana, India
2 Department of Oral Medicine and Radiology, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India

Date of Submission15-Jun-2014
Date of Acceptance24-Aug-2014
Date of Web Publication30-Oct-2014

Correspondence Address:
Harsha Vardhan Talla
House No 1-2-133, 3rd Lane, JKC Nagar, Guntur - 522 006, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-1363.143711

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Desmoplastic fibroma (DF) is a rare, locally aggressive benign fibrous tumor of the bone that has been reported in the maxillofacial skeleton. In this case report, we present imaging findings of a histopathologically proven desmoplastic fibroma involving the left mandibular body region in a 27-year-old male patient. We have also discussed epidemiological data, clinical, radiographical, and histopathological features, along with the prognosis and treatment of DF.

Keywords: Benign fibrous tumor, desmoplastic fibroma, maxillofacial skeleton

How to cite this article:
Talla HV, Alaparthi RK, Yelamanchili S, Naik PR. Desmoplastic fibroma of the mandible: A rare case report . J Indian Acad Oral Med Radiol 2014;26:222-4

How to cite this URL:
Talla HV, Alaparthi RK, Yelamanchili S, Naik PR. Desmoplastic fibroma of the mandible: A rare case report . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2020 Aug 14];26:222-4. Available from: http://www.jiaomr.in/text.asp?2014/26/2/222/143711

   Introduction Top

Desmoplastic fibroma (DF) of the bone is a rare, locally aggressive benign tumor that histologically resembles a desmoid tumor of the soft tissues. [1] It is a non-metastasizing tumor that was first described by Jaffe, in 1958. [2] The term desmoplastic is derived from the Greek word 'desmos', which means band or ligament. [3] This tumor constitutes less than 1% of the bone tumors and about 0.3% of all benign osseous tumors, which usually involve the tibia, scapula, and femur. In the head and neck region, the mandible is the most commonly affected site. [4] In rare instances, multifocal desmoplastic fibromas have been reported in the extremities and the pelvis. [5] There is no sex predilection and 92% of all the patients affected are younger than 30 years old. [6]

Desmoplastic fibroma has an insidious onset and lacks pathognomic clinical symptoms; however, they have been occasionally accompanied with pain and swelling. [3] Radiographically, the tumor presents as an expanding, well-delineated radiolucency, either unilocular or multilocular, and the cortex itself may be destroyed in some areas, with an associated soft tissue mass. This pattern of bone destruction may sometimes mimic a malignancy. [6] The histological appearance of a desmoplastic fibroma is identical to that of the extraosseous desmoid. Although the fibroma is infiltrative, there are no mitoses or nuclear atypia. [2]

   Case Report Top

A 27-year-old male patient, presented with a nine-month history of painless, progressive enlargement in relation to left mandibular body region. The swelling had gradually increased in size. His medical history was insignificant and no related family history was reported.

Clinical examination demonstrated a nontender, firm-to-hard swelling, with an obvious expansion of the cortical plate with soft tissue fullness on the left body of the mandible [Figure 1]. The overlying skin was normal and there was no associated cervical lymphadenopathy, paresthesia or anesthesia of the area under the left inferior alveolar nerve distribution. Intraorally, the lingual plate of the mandible was normal. Dental examination showed all normally erupted teeth, a root canal-treated 36, dental caries in 17, 46, and 47. Considering its slow growth, asymptomatic nature, and consistency, a provisional diagnosis of osteoma and a differential diagnosis of aneurysmal bone cyst, fibrous dysplasia, and Garre's osteomyelitis were considered.
Figure 1: Clinical photograph of the patient showing swelling on the left side of the face

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Radiographical evaluation revealed a well-defined, unilocular radiolucency in the inferior border of the body of mandible on the left side, measuring 1.5 × 1.5 cm, spherical in shape, with irregular sclerotic borders beneath the inferior alveolar canal [Figure 2]. The internal structure of the radiolucency was homogenous and there was no evidence of the effect on the surrounding structures. The cortical plates were intact and the apices of the teeth were normal. Presence of root canal treatment done in relation to 36, was without any evident periapical pathology.
Figure 2: Orthopantomograph of the patient showing the lesion

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Fine needle aspiration (FNA) was negative and the patient was taken up for surgery and excisional biopsy was done. On histopathological examination the specimen showed short, streaming fascicles with elongated fibroblasts [Figure 3] and bundles of collagen fibers [Figure 4], which revealed that it was a desmoplastic fibroma of the mandible. Following surgery, the patient had improved cosmetically and was followed up for a period of one year, wherein the patient was recalled every three months and no recurrence of the lesion was noted.
Figure 3: Photomicrograph (10x) of the lesion showing bundles of collagen fibers

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Figure 4: Photomicrograph (40x) of the lesion showing bundles of collagen fibers

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   Discussion Top

Desmoplastic fibroma is a rare, benign intraosseous tumor with a locally aggressive behavior. [4] With a propensity for recurrence, this is most commonly observed in the long bones. It comprises 0.06% of all tumors of the bone and 0.3% of all benign osseous tumors. [5] The age incidence is usually in the first, second or third decade. There is no specific gender predilection. The mandible is affected more than the maxilla. In the mandible, the posterior region of the mandibular body, angle, and the ascending ramus are the most common sites affected. The premolar area and the anterior segments are less commonly affected. [2]

The most common complaints of patients with a desmoplastic fibroma are facial swelling and sometimes an associated dysfunction. Pain has been rarely reported. In some cases these lesions are associated with pain and loss of teeth, both of which have not been observed in our case. Desmoplastic fibroma may also occur as part of the Gardner's syndrome. [2] Although some tumors are associated with limited mouth opening, with or without malocclusion, a painless swelling of the affected area is the most common initial complaint. Tooth mobility, proptosis, concurrent infection and dysesthesia have been reported infrequently. Most of the lesions measure 3-6 cm when detected, the majority of which are well-circumscribed, but not encapsulated. [4]

Radiographically, desmoplastic fibroma of the bone is characterized by the presence of an expanding and osteolytic lesion, unilocular or multilocular, with irregular cortical margins. A tendency to perforate the cortex in one or more places with subsequent adjacent soft tissue infiltration is common. [6] Our patient similarly had a unilocular osteolytic lesion with irregular corticated borders without any cortical perforation. A desmoplastic fibroma can also present with a geographic pattern of bone destruction with a narrow transition zone and non-sclerotic margins, internal pseudo-trabeculation (coarse thick septa or thin lace-like pattern), and cortical expansion. Perforation of the cortex may also be present. New periosteal bone formation has been seen in a limited number of reported patients. [7]

The differential diagnosis of an osteolytic lesion in the mandible includes giant cell reparative granuloma, aneurysmal bone cyst, odontogenic keratocyst, and fibrous dysplasia. Giant cell reparative granuloma, similar to desmoplastic fibroma, may extend to the subchondral bone, but it is round rather than oval. An aneurysmal bone cyst tends to have an eccentric, 'blow-out' appearance as opposed to the fusiform expansion usually seen with DF. An odontogenic keratocyst is usually centrally located, with a scalloped border and thin marginal sclerosis. Fibrous dysplasia contains a mineralized matrix and often has a sclerotic rim. [2]

At the microscopic level, DF consists of a mild-to-moderate cellular matrix of fibrocollageous stromal tissue, lacking cellular pleomorphism, nuclear hyperchromatism or mitosis. Increased cellularity seems to be associated with an increased tendency for recurrence. Both the lack of a capsule and the infiltrative nature of this lesion are hallmarks of DF; these features can pose as a diagnostic challenge. Immunohistochemistry is generally not helpful in establishing the diagnosis of DF. [5] The histopathological picture of our case showed short, streaming fascicles, with enlarged fibroblasts and bundles of collagen fibers; other areas showed plump proliferating fibroblasts with vesicular nuclei and prominent nucleoli.

Various medical and surgical treatment options have been suggested for desmoplastic fibroma. These include simple curettage, segmental resection, en block resection, radiotherapy, and chemotherapy, with or without additional surgical procedures. [4] Jaffe recommended segmental resection as the treatment of choice and noted that if the lesion is curetted and showed a recurrence, the second treatment should be segmental resection or a more thorough curettage. Generally radiotherapy is not considered, because of the documented hazards of such a therapy for benign disease, especially in younger children. [6]

The prognosis of the DF is dependent on the completeness of surgical removal. A three-year recurrence-free period can be considered as an indication of successful treatment. [6]

   Conclusion Top

Desmoplastic fibroma (DF) is a rare, benign, locally aggressive, intraosseous lesion, with a high local recurrence rate. The tumor should be carefully resected with narrow safety margins, considering the patient's postoperative well being, with guaranteed periodic follow-ups.

   References Top

Vargas-Gonzalez R, San Martin-Brieke W, Gil-Orduña C, Lara-Hernandez F. Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis. Pathol Oncol Res 2004;10:237-9.  Back to cited text no. 1
Shi H, Wang P, Wang S, Yu Q. Desmoplastic fibroma of the mandible. Dentomaxillofac Radiol 2008;37:408-11.  Back to cited text no. 2
Schneider M, Zimmermann AC, Depprich RA, Kübler NR, Engers R, Naujoks CD, et al. Desmoplastic fibroma of the mandible - review of the literature and presentation of a rare case. Head Face Med 2009;5:25.  Back to cited text no. 3
Dhaif G, Satir AA, Sharif S. Desmoplastic fibroma of the mandible: A 5 year follow-up. Bahrain Med Bull 2008;30:251-4.  Back to cited text no. 4
Herford AS, Reder P, Ducic Y. Multifocal desmoplastic fibromas of the mandible. J Oral Maxillofac Surg 2001;59:1078-81.  Back to cited text no. 5
Chang JJ, Hu C, Chang PC, Liu HY, Tu CN. Juvenile desmoplastic fibroma of mandible: A case report. Zhonghua Ya Yi Xue Hui Za Zhi 1988;7:35-40.  Back to cited text no. 6
Dalili-Kajan Z, Adham-Fumani G, Dalili-Kajan H. Desmoplastic fibroma with periosteal reaction. Acta Medica Iranica 2007;45:325-8.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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