Desmoplastic fibroma of the mandible: a rare gnathic bone tumor with a review of the literature.

Manoj Gopal Madakshira,Roshan Kumar Verma,Amanjit Bal

doi:10.4322/acr.2019.091

Manoj Gopal Madakshira, Roshan Kumar Verma + Show 1 more

Open Access

https://doi.org/10.4322/acr.2019.091

Copy DOI

Abstract

Desmoplastic fibroma (DF) is a rare bone tumor, which is known to involve mostly the gnathic bones. In this setting, the clinical presentation is usually represented by a bulging tumor of the face. Radiologically, the tumor is usually characterized by an expansile lytic bone lesion. The histopathology of the tumor shows a poorly circumscribed hypocellular lesion rich in collagen fibers with interspersed spindle cells having bland nuclear chromatin. Despite the lack of mitoses and nuclear pleomorphism, DF has an aggressive nature, presenting as a destructive growth causing entrapment of neuro-vascular bundles, sinusitis, or trismus. Some cases of DF show mutations in the adenomatous polyposis coli pathway shown by nuclear localization of the β-catenin protein. Few reports showed an association with tuberous sclerosis, though most of these cases were sporadic. We discuss a rare case of desmoplastic fibroma involving the mandible, and a review of the literature of the DF cases involving the gnathic bones.

Highlights

Desmoplastic fibroma (DF) is an unusual bone tumor having a myofibroblastic origin, akin to the desmoid tumor seen in the soft tissue.[1]
We discuss a case of DF affecting the mandible and review the available literature data on DF affecting the gnathic bones, in an attempt to better understand the clinical and pathological characteristics of this rare tumor
A total of 54 cases of DF involving the gnathic bones were found (Table 1). 42 cases (77%) involved the mandible, while the remaining showed the involvement of the maxilla

Summary

INTRODUCTION

Desmoplastic fibroma (DF) is an unusual bone tumor having a myofibroblastic origin, akin to the desmoid tumor seen in the soft tissue.[1]. A contrast-enhanced computed tomography (CT) showed a 6.5 × 6 × 4.5 cm lytic lesion involving the right ramus and body of the mandible (Figure 1). The excised a Post Graduate Institute and Medical Education and Research (PGIMER), Department of Histopathology. B Post Graduate Institute and Medical Education and Research (PGIMER), Department of Otolaryngology and Head & Neck Surgery. The spindle cells had bland nuclear chromatin and indistinct cell borders, which appeared to merge with the surrounding fibrous stroma (Figure 3A). These spindle cells showed a dense and diffuse cytoplasmic positivity for vimentin (Figure 3B) and cytoplasmic positivity for smooth muscle actin (SMA) (Figure 3C). At the end of 5 months of follow-up, the girl is asymptomatic without any evidence of recurrence

Findings

DISCUSSION

CONCLUSION