Iranian Journal of Otorhinolaryngology

Iranian Journal of Otorhinolaryngology

Intraosseous Desmoplastic Fibroblastoma of the Mandible: Report of a Rare Case Mimicking Aggressive Jaw Lesions

Document Type : Case Report

Authors
Shruti Singh
Anju Devi
Mala Kamboj
Anjali Narwal
Virendra Singh
Department of Oral & Maxillofacial Pathology, Pandit Bhagwat Dayal Sharma University of Health Sciences (Postgraduate Institute of Dental Sciences), Rohtak, Haryana-124001, India.
10.22038/ijorl.2026.97097.4195
Abstract
Introduction:
Desmoplastic fibroblastoma is an exceptionally rare benign fibroblastic neoplasm that predominantly affects soft tissues, whereas primary intraosseous occurrence is exceedingly uncommon. Its variable clinicoradiological presentation often mimics aggressive odontogenic and mesenchymal tumors, rendering preoperative diagnosis particularly challenging.

Case Report:
A 20-year-old male presented with a progressively enlarging swelling of the left mandible. Computed tomography revealed a well-defined, expansile, mixed radiolucent-radiopaque intraosseous lesion with cortical expansion and focal breach. Although the incisional biopsy favoured a benign spindle-cell mesenchymal lesion, the extensive osseous involvement warranted hemimandibulectomy. Histopathological examination demonstrated a well-circumscribed, hypocellular fibroblastic neoplasm composed of bland spindle- and stellate-shaped cells with eosinophilic cytoplasm dispersed within an abundant, densely collagenised stroma. Immunohistochemical analysis showed diffuse vimentin positivity, supporting the diagnosis of primary intraosseous desmoplastic fibroblastoma. At 8-month follow-up, the patient remains free of recurrence.

Conclusion:
As the fifth reported primary intraosseous case and the fourth involving the jaws, this case expands the limited literature on this rare entity. It highlights the indispensable value of meticulous clinicoradiological, histopathological, and immunohistochemical correlation in distinguishing this benign neoplasm from more aggressive jaw lesions, thereby facilitating accurate diagnosis and appropriate surgical management.
Keywords
Desmoplastic fibroblastoma
Collagenous fibroma
Mandible
Subjects
1.                   Evans HL. Desmoplastic fibroblastoma: a report of seven cases. The American journal of surgical pathology. 1995 Sep 1;19(9):1077-81.
2.                   Nielsen GP, O'Connell JX, Dickersin GR, Rosenberg AE. Collagenous fibroma (desmoplastic fibroblastoma): a report of seven cases. Modern pathology: an official journal of the United States and Canadian Academy of Pathology, Inc. 1996 Jul 1;9 (7):781-5.
3.                   Miettinen M, Fetsch JF. Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. Human pathology. 1998 Jul 1;29(7):676-82.
4.                   Watanabe H, et al. Clinical features of desmo- plastic fibroblastoma.J Dermatol.2008;35: 561–7.
5.                   Nagao T, et al. Collagenous fibroma of oral cavity. Oral Oncol. 2002;38:278–282.
6.                   Yamamoto A, et al. Radiologic findings of desmoplastic fibroblastoma. Radiol Case Rep. 2017; 12:1234–1240.
7.                   Murphey MD, et al. Imaging of benign fibrous tumors. Radiographics. 2009;29:203–219.
8.                   Bernal K, Nelson M, Neff JR, Nielsen SM, Bridge JA. Translocation (2;11)(q31;q12) is recurrent in collagenous fibroma (desmoplastic fibroblastoma). Cancer genetics and cytogenetics. 2004 Mar 1;149(2):161-3.
9.                   Al-Zaidi R, AL-Zaidi RS. An unusual case of intraosseous desmoplastic fibroblastoma (collagenous fibroma) of the mandible. Cureus. 2024 Dec 17;16(12).
10.                Mesquita RA, Okuda E, Jorge WA, de Araújo VC. Collagenous fibroma (desmoplastic fibro- blastoma) of the palate: A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001; 91(1):80-84.
11.                Garcia NG, De Freitas Filho SA, Soares CT, Figueiredo CM, Oliveira DT. Intraosseous collagenous fibroma (Desmoplastic fibroblastoma) involving maxillary bone. J Clin Diagn Res. 2018 Feb 1;12:6.
12.                Jaafari-Ashkavandi Z, Shirazi MY, Assar S. Desmoplastic fibroblastoma in maxillary alveolar bone mimicking an odontogenic lesion: a novel case report with review of literature. Turk Patoloji Derg. 2018 Sep 1;34(3):247-50.
13.                Fong F, Odell E, Simo R: Collagenous fibroma (desmoplastic fibroblastoma) of the neck presenting with neurological symptoms. Head Neck Pathol. 2009, 3:47-50.
14.                Hornick JL, Miettinen M, Bridge JA: WHO classification of Soft tissue and bone tumors. WHO Classification of Tumors Series, 5th Edition. WHO Classification of Tumors Editorial Board (ed): International Agency for Research on Cancer, Lyon, France; 2020. 3:
15.                Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY. Diagnosis and differential diagnosis of desmoplastic fibroblastoma by clinical, radiological, and histopathological analyses. Chinese Medical Journal. 2018 Jan 5;131(01):32-6.
16.                Nielsen GP, Rosenberg AE. Desmoplastic fibroma of bone. In: WHO Classification of Tumors Editorial Board. Soft Tissue and Bone Tumors. WHO Classification of Tumors. 5th ed. Lyon: International Agency for Research on Cancer; 2020.
17.                WHO Classification of Tumors Editorial Board. Soft Tissue and Bone Tumors. WHO Classification of Tumors. 5th ed. Lyon: International Agency for Research on Cancer; 2020.
18.                WHO Classification of Tumors Editorial Board. Head and Neck Tumors. WHO Classification of Tumors. 5th ed. Lyon: International Agency for Research on Cancer; 2022.
19.                Mentzel T, Calonje E, Nascimento AG, Fletcher CDM. Myofibroma in adults: Clinicopathological analysis of a series of cases. Am J Surg Pathol. 1995;19:306–315.
20.                Doyle LA, Möller E, Dal Cin P, Fletcher CDM, Mertens F, Hornick JL. MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. Am J Surg Pathol. 2011;35:733–741.
Iranian Journal of Otorhinolaryngology
Volume 38, Issue 6
November and December 2026
Pages 421-426