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 Table of Contents  
Year : 2019  |  Volume : 31  |  Issue : 1  |  Page : 74-78

Desmoplastic fibroma of the mandible – A rare benign tumor mimicking malignancy

Department of Oral Medicine and Radiology, Government Dental College, Thiruvananthapuram, Kerala, India

Date of Submission18-Dec-2018
Date of Acceptance11-Feb-2019
Date of Web Publication23-Apr-2019

Correspondence Address:
Dr. Saranya Konikoth
Edavanath, Avitanallur, Naduvannur, Calicut - 673 614, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaomr.jiaomr_213_18

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Desmoplastic fibroma (DF) is a rare benign intra-osseous tumor with provincially aggressive nature. The tumor constitutes <1% of bone tumors, and mandibular involvement is common with a reported frequency of approximately 40% of the various bony sites. It may progress to bone destruction and displays a tendency to invade the soft-tissues if untreated. Inadequate treatment accounts for the high propensity for local recurrence. We report a case in a 6-year-old female child with a swelling over the left body of the mandible, an expansile lytic lesion with the destruction of lingual and buccal cortex noted on computed tomography (CT) scan. The child underwent left hemimandibulectomy and reconstruction of the sick jawbone. Recognition of this entity is important because it may histologically and radiographically resemble benign fibrous lesions. The non-specific clinical appearance of the DF implies an extensive imaging study before in order to establish a proper therapeutic approach. We hope that our case report will shed new light on this special entity.

Keywords: Benign bone tumor, desmoplastic fibroma, mandible, recurrence

How to cite this article:
Konikoth S, Bose T, Anupama I V, Yogidas R. Desmoplastic fibroma of the mandible – A rare benign tumor mimicking malignancy. J Indian Acad Oral Med Radiol 2019;31:74-8

How to cite this URL:
Konikoth S, Bose T, Anupama I V, Yogidas R. Desmoplastic fibroma of the mandible – A rare benign tumor mimicking malignancy. J Indian Acad Oral Med Radiol [serial online] 2019 [cited 2022 Dec 2];31:74-8. Available from: http://www.jiaomr.in/text.asp?2019/31/1/74/256901

   Introduction Top

Desmoplastic fibroma (DF) was first described by Jaffe[1] in 1958. In 1965, the first report about a DF involving the mandible was presented by Griffith and Irby.[2] DF is recognized as the intra-osseous counterpart of soft tissue fibromatosis in both gnathic and extra-gnathic sites. Histologically and biologically, it simulates the extra-abdominal desmoid tumor of soft tissue. Desmoid tumors are well-known benign fibrous tissue tumors that are mainly localized in the abdominal wall in females of childbearing age (abdominal desmoids).[3] The most common localizations of extra-abdominal desmoid tumors are the shoulder and upper limb.[4]

DF is a benign locally destructive tumor without metastasis. It is notorious for the high incidence of recurrence after resection. On histopathologic examination, this tumor shows gradual progression with well-differentiated cells that produce collagen. Because of its locally infiltrative nature, some authors place this entity in a borderline position with regard to malignancy.[5] Recognition of this entity is important because it may histologically and radiographically resemble benign fibrous lesions that behave in a less aggressive fashion or, more significantly, with spindle cell sarcomas.

   Case History Top

A 6-year-old girl reported in oral medicine out-patient department complaining of painless swelling over left lower jaw for the past 6 months. The swelling was painless and showed a gradual increase in size, and there was no history of local trauma, paresthesia, pus/blood discharge, and tooth mobility. The patient gave a history of extraction of decayed left lower back tooth 2 weeks back.

Extraoral examination revealed a solitary, diffuse swelling of size approximately 5 × 4 cm over the left body of the mandible with normal overlying skin extending from the level of ala-tragus line to 1 cm inferior to lower border of the mandible. Mediolaterally, it extended from the left corner of the mouth to preauricular region [Figure 1].
Figure 1: Clinical image showing swelling of left side of lower jaw

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On palpation, the swelling was firm and non-tender, and there was no rise in local temperature. Multiple, soft, mobile, and non-tender submandibular lymph nodes were palpable bilaterally with the largest one on left side measuring 2 × 1.5 cm.

Intraorally diffuse non-tender firm swelling with normal overlying mucosa obliterating left mandibular buccal vestibule noted in relation to 36 region. Healing extraction socket of 75 was present mesial to swelling. Vitality testing showed a positive response in 74,36. A provisional diagnosis of odontogenic tumor/fibro-osseous lesion was made [Figure 2].
Figure 2: Intra-oral photograph showing obliteration of the left buccal vestibule in relation to 36

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In the panoramic radiograph, a destructive radiolucent lesion measuring 1.5 × 1.5 cm was noted on left body of the mandible just below inferior alveolar nerve extending from 75 to 37 region. Superior border of the lesion appeared irregular and inferior destruction of mandibular cortical border from 35 to 37 region was present [Figure 3].
Figure 3: Panoramic view of radiograph showing destruction of mandibular inferior cortical border

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A break in the buccal cortex in relation to 36 region was visible in the mandibular occlusal view, and there was no considerable cortical expansion [Figure 4].
Figure 4: Mandibular occlusal view showing a break in the buccal cortex in relation to 36 region

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Ultrasonographic (USG) examination done with a 12 MHz transducer showed a well-defined, heterogeneous predominantly hypoechoic lesion of size 2.7 × 1.7 cm with posterior echo enhancement noted over the left submandibular region. Multiple bilateral reactive Level I, II, III, and IV lymph nodes were also noted [Figure 5].
Figure 5: USG showing heterogeneous predominantly hypoechoic lesion with posterior echo enhancement noted over left submandibular region

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Computed tomography (CT) images of the mandible demonstrated well-defined expansile lytic lesion involving the angle of left mandible with the destruction of lingual and buccal cortex. Minimally enhancing soft tissue was present adjacent to the lesion extending to the masseter, digastric, and mylohyoid muscle near the mandibular attachment. There was no significant periosteal reaction or infiltration of the submandibular gland. Margins of the lesion were well defined. Lesion measured 20 × 19 × 21 mm. A single round submandibular node measuring 9.5 × 9.5 mm with loss of hilum and multiple subcentimeter nodes in the Level II, III, and IV lymph node stations bilaterally were present [Figure 6]a and [Figure 6]b.
Figure 6: (a) Computed tomography (CT) axial view of mandible demonstrating a well-defined expansile lytic lesion involving the angle of left mandible with destruction of lingual and buccal cortex. (b) Three-dimensional computed tomography view showing destruction of inferior cortical border

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An abnormal increased localization of radiopharmaceuticals (99m-Tc-MDP) was seen at the mandible in the single photon emission computed tomography (SPECT) scan. Rest of the skeleton appeared normal [Figure 7]. CT thorax was with in normal limit. Bone marrow imprint showed normal hematopoietic element with mild eosinophilia.
Figure 7: SPECT scan showing an abnormal increased localization of radiopharmaceuticals (99m-Tc-MDP) at mandible

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Biochemical and routine blood investigation results were within normal limits. Fine needle aspiration from the lesion was done, and cytological examination of the smear showed few clusters of cells with high N:C ratio and blood.

An incisional biopsy was done under local anesthesia, and the greyish brown tissue mass obtained was sent for histopathological examination. Microscopically, small fragment of neoplasm composed of sheets of bland spindle cells with vesicular elongated nuclei separated by collagenous connective tissue stroma was noted. Mitotic activity was low and inflammatory cells were minimal. The neoplasm was infiltrating the bone [Figure 8]. Immunohistochemical labeling was performed, and lesional cells were positive for SMA and negative for S-100. MIB-1 labeling index was low (1%-2%). Histopathological diagnosis was low-grade spindle cell proliferating lesion, possibly DF like a tumor.
Figure 8: Photomicrograph from the incisional biopsy demonstrating small fragment of neoplasm composed of sheets of bland spindle cells with vesicular elongated nuclei separated by collagenous connective tissue stroma. The neoplasm was infiltrating the bone

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The child underwent left hemimandibulectomy and reconstruction of the sick jawbone [Figure 9]. The excised specimen measured 5.5 × 3 cm which consisted of a piece of mandibular bone with one tooth attached to it and underlying ulceroproliferated area attached to submandibular gland. A final histopathological examination of the specimen confirmed the diagnosis of DF infiltrating adjacent fat and muscle.
Figure 9: Post-operative panoramic view showing left hemimandibulectomy and reconstruction plate

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Post-operative orthopantomography (OPG) was taken after 6 months which showed normal bony margins without any signs of recurrence [Figure 10]. After surgery, the patient had an acceptable aesthetic facial profile and was put on periodic recall [Figure 11].
Figure 10: Panoramic radiography (6 months after resection) showing normal bony margins and evidence of bone

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Figure 11: Follow-up photograph of the patient after 6 months

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   Discussion Top

Fibromas are benign connective tissue neoplasms which are fairly common on oral mucosa. Rarely, they may involve the jaw bone (intra-osseous fibroma). They are categorized as odontogenic and non-odontogenic (desmoplastic) fibromas based on the presence or absence of odontogenic epithelium.[6] Up to new, fewer than 100 cases of DF in the mandible have been published to date, which attest the rarity of these tumors and the low incidence.

In 1958, Jaffe[1] distinguished DF from other intra-osseous fibromas for the first time. Regezi et al.[7] described DF as a benign locally aggressive neoplasm of bone which shares several characteristics with soft tissue fibromatoses and desmoid tumors. In 1965, the first report about a DF of the jaw was presented by Griffith and Irby.[2] This is one of the rarest bone tumors constituting <1% of bone tumors and about 0.3% of all benign osseous tumors.[8]

As an intra-osseous lesion, it commonly affects the metaphysis of long bones with plenty of cases involving children. The site of predilection within the jawbone is the mandible (the ramus, angle, and molar area) whereas the maxilla is infrequently affected.

It can affect any age group, occurs most often in the first 3 decades, and is found equally in men and women. Mandibular involvement is reported to be approximately 40% of the various bony sites.

DF of jawbone usually manifests as a painless, slow-growing, firm swelling, barely associated with loose teeth, chewing/swallowing difficulty, and recurrent sinusitis. Facial asymmetry and abnormal teeth alignment may be present. Common radiographic appearance is a radiolucent, multilocular lytic lesion expanding bone without any periosteal reaction sign.[9]

CT and magnetic resonance imaging (MRI) may be helpful in assessing the degree of bone destruction and cortical continuity and identifying soft tissue extension. The “internal explosion” effect of the lytic lesion could be visualized in CT scan.[10] In MRI, a majority of reported cases have shown low to intermediate signal intensity foci on T2 weighted images and no restriction of the apparent diffusion coefficient, which confirms a benign but locally aggressive neoplasm without an inclination to malignancy.[9]

Owing to the remarkable resemblance to other lytic bone lesions, a wide range of radiographic differential diagnoses are discussed in the literature. They include fibrous dysplasia, giant cell tumor, aneurysmal bone cyst, odontogenic fibroma, eosinophilic granuloma, fibro-sarcoma, intraosseous osteosarcoma, adamantinoma, and metastases.[8],[11],[12],[13]

Only two case reports with sonographic imaging findings were found in the literature.[9],[14] In this case, the sonographic evaluation revealed an ill-defined heterogeneous predominantly hypoechoic lesion of size 2.7 × 1.7 cm over the left submandibular region with moderate vascularity in color flow Doppler. Multiple Level I, II, III, and IV reactive lymph nodes were noted bilaterally.

The histologic criteria for DF as defined by the World Health Organization is “a rare benign bone tumor composed of spindle cells with minimal cytological atypia and abundant collagen production” (ICD-O code 8823/0).[15] It is characterized by low cellularity with some possible foci of hypercellularity. Lack of a capsule and infiltrative nature of the lesion are hallmarks of DF.[16],[17] In our case, infiltration of the tumor into bone was evident.

Low-grade fibrosarcoma and low-grade intraosseous osteosarcoma are the most difficult differential diagnoses. The typical fibrosarcoma is more cellular with a herringbone pattern showing more polymorphism and higher mitotic activity. However, in some cases of low-grade fibrosarcoma, mitoses are not manifested and areas with predominant collagen tissue may be found, which makes the distinction extremely difficult. Recurrence and metastasis after therapy might indicate a low-grade fibrosarcoma, as DF is more aggressive locally and does not include metastasis. Low-grade intraosseous sarcoma and fibrous dysplasia can be distinguished by osteoid production in them.[13],[18]

Surgical management ranging from aggressive curettage with peripheral ostectomy in early lesions to segmented resection/wide excision in lesions with extra-osseous extension has been recommended for DF. Segmental resection is preferred when a lesion displays signs of aggressive behavior and extension into surrounding soft tissues.[19] Significant rates of recurrence (up to 67%) have been associated with DF, depending on the completeness of surgical removal.[20] Patients undergoing intra-lesional or marginal resection should be conversant with the need for long-term surveillance owing to the tendency for local recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Jaffe HL. Tumors and Tumorous Conditions of the Bones and Joints. Philadelphia: Lea and Febiger; 1958. p. 298-303.  Back to cited text no. 1
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.  Back to cited text no. 2
Böhm P, Kröber S, Greschniok A, Laniado M, Kaiserling E. Desmoplastic fibroma of the bone: A report of two patients, review of the literature, and therapeutic implications. Cancer 1996;78:101123.  Back to cited text no. 3
Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. St. Louis: Mosby-Year Book lnc; 1995.  Back to cited text no. 4
Dahlin DC, Unni KK. Bone Tumors. 4th ed. Springfield, Illinois: Thomas; 1986. p. 375-8.  Back to cited text no. 5
Langlais RP, Langland OE, Nortje CJ. Multilocularradiolucencies. In Diagnostic Imaging of the Jaws. Baltimore: Williams & Wilkins; 1995. p. 370-6.  Back to cited text no. 6
Regezi JA, Sciubba JJ. Odontogenictumors, benign nonodontogenictumors. In Oral Pathology: Clinical Pathologic Correlations 3rd ed. Philadelphia: W.B. Saunders; 1999.  Back to cited text no. 7
Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW. Desmoplastic fibroma of bone: Radiographic analysis. Radiology 1989;172:827-32.  Back to cited text no. 8
Averna R, De Filippo M, Ferrari S, Bacchini E, Rossi C. Desmoplastic fibroma of the mandible. Acta Biomed 2011;82:69-73.  Back to cited text no. 9
Ikeshima A, Utsunomiya T. Case report of intra-osseous fibroma: A study on odontogenic and desmoplastic fibromas with a review of the literature. J Oral Sci 2005;47:14957.  Back to cited text no. 10
Gebhardt MC, Canipbcll CJ, Schiller AL. Mankin HI. Des-moplastic fibroma of hone. A report of eight cases and re-view of the literature. J Rorie Joinf Surg Am 1985;67:732-47.  Back to cited text no. 11
Hufnagel TJ, Artiles C, Piepmeier J, Kier L, Kim JH. Desmoplastic fibroma of parietal bone simulating eosinphilic granuloma. J Neurosurg 1987;67:449-51.  Back to cited text no. 12
Inwards CY, Unni KK, Beahout JW, Sim FH. Desmoplastic fibroma of bone. Cancer 1991;68:1978-83.  Back to cited text no. 13
Gersak MM, Lenghel LM, Baciut G, Moldovan I, Mitea H, Botar-Jid C. Ultrasonography of desmoplatic fibroma of the mandible; A case report. Med Ultrason 2015:17:407-10.  Back to cited text no. 14
Fletcher CDM, Uni KK, Mertens F, WHO Classification of Tumours. Pathology and Genetics of Tumors and soft Tissue and Bone. Lyon: IARC Press; 2002. p. 288.  Back to cited text no. 15
Hopkins KM, Huttula CS, Kahn MA, Albright JE. Desmoplastic fibroma of the mandible: Review and report of two cases. J Oral Maxillofac Surg 1996;54:1249-54.  Back to cited text no. 16
Nedopil A, Raab P, Rudert M. Desmoplastic fibroma: A case report with three years of clinical and radiographic observation and review of the literature. Open Orthop J 2013;8:4046.  Back to cited text no. 17
Dahlin DC, Hoover NW. Desmoplastic fibroma of bone: Report of two cases. JAMA 1964;188:685-7.  Back to cited text no. 18
Nishida J, Tajima K, Abe M, Honda M, Inomata Y, Shimamura 'T, et al. Desmoplastic fibroma. Aggressive curettage as a surgical alternative for treatment. Clin Orthop Relat Res 1995;142-8.  Back to cited text no. 19
Said-Al-Naief N, Fernandes R, Louis P, Bell W, Siegal GP. Desmoplastic fibroma of the jaw: A case report and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:82-94.  Back to cited text no. 20


  [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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