Fibrous dysplasia of the anterior mandible: A rare case

report

Abstract
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INTRODUCTION
In 1938, Lichtenstein first coined the term “fibrous dysplasia (FD)” [1]. FD is a rare,
nonmalignant condition in which normal bone and marrow are replaced by fibrous tissue and
randomly distributed woven bone, usually with pain, bony deformity, and pathologic
fractures [2]. In general, FD presents in three forms – monostotic, polyostotic, and
polyostotic with endocrinopathies, which can be associated with hyperpigmentation and
endocrinological disorders and is called McCune–Albright syndrome [3]. Common sites of
skeletal involvement are the long bones, craniofacial bones, ribs, and pelvis [2]. Monostotic
FD (MFD), although less serious than polyostotic FD (PFD), is of greater concern to the
dentist because of the relatively high frequency of occurrence in the jaws [4]. FD is caused by
somatic activating mutations of the gene GNAS in a subunit of the stimulatory G protein,
located at 20q13.2-13.3 [2]. The diagnosis of FD is based on physical, radiological, and
histopathological examination. There are different treatment approaches including
observation, medical treatment, and surgical treatment. This article presents a case of MFD in
the anterior mandible in a 43-year-old female with the clinical, radiographical, and
histopathological features.
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CASE REPORT
A 43-year-old female presented at the Dentistry Department of Hualien Buddhist Tzu Chi
Hospital with pain over the left posterior teeth for a week. Extraoral examination showed no
obvious facial asymmetry. However, intraoral examination showed both labial and lingual
bone expansion in the anterior mandible. The labial expansion was located at teeth 22 and 23,
and the lingual expansion was from tooth 22 to tooth 27 [Figure 1]. Radiographic
examination revealed a lesion with both radiopaque and radiolucent features showing a
“ground-glass” appearance from the mesial side of the tooth 21 root apex to the distal side of
the tooth 27 root apex, and the two other small solitary apical lesions of teeth 18 and 31
[Figures [Figures22 and and3].3]. All nine teeth mentioned above responded normally to the
electric pulp test. The cone-beam computed tomography (CS 9300, Carestream Health,
Rochester, NY, USA) revealed a “ground-glass” appearance in the buccal and lingual bony
expansion regions with relatively well-defined borders [Figure 4]. Endosteal scalloping was
also noted. Thinning of the cortical bone was observed in the sagittal view labially and
lingually [Figure 4a], while the homogeneous sclerotic bone was shown in the transverse
view labially [Figure 4c]. A three-dimensional rendered image revealed labial bony
expansion [Figure 4d]. A confirmatory intrabony biopsy was done [Figure 5]. Grossly, the
specimens of lesion were grayish and elastic. Histologically, there was FD characterized by
irregularly shaped trabeculae of woven bone and lamellar bone in a cellular, loosely arranged
fibrous stroma [Figure 6]. After pain relief, we explained the possible risks and recommended
suitable treatment plans for the patient. The patient preferred noninvasive medication for pain
relief and regular follow-up.

Figure 1
Clinical photograph showing expansion of both the labial and lingual cortical plates of the anterior
mandible

Figure 2 Panoramic radiography reveals a mixed radiolucent and radiopaque lesion with a ground
glass appearance in the anterior mandible which is crossing the midline
Figure 3
Periapical radiograpy demonstrates details of the mixed radiolucent and radiopaque lesion extending
from the mesial side of the tooth 21 root apex to the distal side of the tooth 27 root apex and the two
other apical lesions of teeth 18 and 31
Figure 4
Cone-beam computed tomography reveals a “ground glass” appearance in the buccal and lingual bone
expansion regions and relatively well-defined borders. (a) Sagittal view. (b) Coronal view. (c)
Transverse view. (d) Three dimensional rendered image of the jaw bone
Figure 5
Biopsy performed at the most protruding site of the labial cortical plate
Figure 6
Irregularly shaped trabeculae of woven bone and lamellar bone in a cellular, loosely arranged fibrous
stroma

DISCUSSION
FD is a congenital, metabolic, nongenetic disturbance that represents 2.5% of all bone tumors
and over 7% of all benign bone tumors [5]. FD involves the facial and cranial bones in nearly
50% of PFD patients and in 10%–27% of MFD patients [6]. The maxilla is more commonly
involved than the mandible in MFD jawbone lesions. Our case involved the mandibular bone,
this is a rare case in the FD. The lesion was in the anterior mandible crossing the midline with
two other small lesions in the posterior region of the mandible. FD can occur at any age.
However, it is usually observed in children and young adults with 75% of patients presenting
before the age of 30 [2]. In one study reported that, FD has a female predilection and is
observed more frequently in older decades [7]. In another study, no gender predilection is
found [2]. After puberty or bone maturation, FD progresses slowly or ceases, whereas in
another report, FD continued to progress into old age [8].
The differential diagnosis of FD includes simple bone cyst, nonossifying fibroma,
osteofibrous dysplasia, adamantinoma, low-grade intramedullary osteosarcoma, and Paget's
disease [2]. The current gold standard for the diagnosis of FD is a histologically-proven fibro-
osseous lesion with poorly defined margins which are confirmed by radiographic findings
[3]. Treatment protocols for FD include observation, medical treatment, and surgery. Clinical
observation is suggested for FD lesions that have no risk of pathologic fracture or deformity
[2]. Medical treatment with bisphosphonates may have benefits including improvement of
function, pain relief, and lower fracture risk in appropriately selected FD patients [2]. One
study reported clinical improvement in children and adults treated with bisphosphonates [9].
Surgery is indicated for confirmatory biopsy, correction of deformity, prevention of
pathologic fracture, and/or elimination of symptomatic lesions [2]. Conservative management
has been the standard of care, which involves removing the diseased bone via an intraoral
approach. Cortical bone grafts are superior to cancellous bone grafts or bone-graft substitutes
because of the excellent quality of the remodeled cortical bone [2]. After a confirmatory
biopsy, our case was diagnosed with symptomatic MFD. After discussion with the patient,
she refused invasive surgical treatment and chose conservative management with regular
recall and clinical observation. The reported prevalence of malignant transformation of FD is
0.4%–4% [2]. Therefore, periodic follow-up, for example, every 6 months, and radiographic
examination should be carried out to verify that there is no progression or malignancy [2].
MFD of the anterior mandible in the middle-aged female is rare. Nevertheless, the general
dental clinician can be the first to detect this disease in daily practice using panoramic
radiography. Therefore, sufficient knowledge of FD is essential to make an appropriate
diagnosis and reduce the complications of the disease. With improvements in medicine and
molecular technology, better therapy for FD, such as genetic therapy, may be possible in the
near future.

Declaration of patient consent

The authors certify that the patient has obtained an appropriate patient consent form. In the
form, the patient has given her consent for her images and other clinical information to be
reported in the journal. The patient understands that her name and initials will not be
published, and due efforts will be made to conceal her identity, but anonymity cannot be
guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest
There are no conflicts of interest.
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