IP Archives of Cytology and Histopathology Research

Print ISSN: 2581-5725

Online ISSN: 2456-9267

CODEN : IACHCL

IP Archives of Cytology and Histopathology Research (ACHR) open access, peer-reviewed quarterly journal publishing since 2016 and is published under the Khyati Education and Research Foundation (KERF), is registered as a non-profit society (under the society registration act, 1860), Government of India with the vision of various accredited vocational courses in healthcare, education, paramedical, yoga, publication, teaching and research activity, with the aim of faster and better dissemination of knowledge, we will be publishing the article more...

  • Article highlights
  • Article tables
  • Article images

Article statistics

Viewed: 311

PDF Downloaded: 139


Get Permission Sachdev, Chettiankandy, Sardar, Sarda, and Chhapane: The histological artistry of fibrous dysplasia: A case report


Introduction

The fibro-osseous lesions (FOLs) are the craniofacial bones are a group of lesions that comprise reactive, neoplastic, developmental, and dysplastic pathologic processes.1 The lesions included under the rubric of FOLs closely resemble each other in clinical, radiographic, and particularly, histopathological aspects, thereby making differentiation between them a challenging task. These include fibrous dysplasia (FD), ossifying fibroma (OF), Cemento-osseous dysplasia (COD), segmental odontomaxillary dysplasia (SOD), and familial gigantiform fibroma. 2

FD is characterized by the replacement of normal medullary bone by proliferative fibrous connective tissue. The resultant bone consists of underdeveloped, inadequately calcified irregular trabeculae. 3 The term was initially coined by Lichenstein and since then many descriptive terms have been used for the lesion. 4 These range from a developmental anomaly to hamartoma to a benign tumor-like process. 5, 6, 7 Considering the incidence rate, FD comprises only 2% of all benign tumors and tumor-like processes of the bone. 8

FD may affect a single bone (monostotic) or multiple bones (polyostotic). In the case of involvement of craniofacial bones, it is referred to as craniofacial FD, which is generally polyostotic (more than 75% cases) and seldom monostotic (10-25% cases). 7 Although much less alarming in terms of severity of symptoms, monostotic craniofacial FD is of great interest to dental professionals, owing to its relatively higher predilection to occur in the jawbones. 9

The present case report comprises one such case of monostotic craniofacial FD occurring in the mandible of an Indian adolescent male.

Case Report

A 13-year-old male complained of swelling in the left mandibular anterior region since one year. No remarkable findings were noted on extraoral examination. Intraorally, ill-defined intra-osseous swelling extending from the mandibular permanent left central incisor to the mandibular left deciduous second molar region was present.(Figure 1) The size of the lesion was approximately 5 x 4 cm. Buccoversion of the deciduous mandibular left first molar and permanent maxillary left canine was noted. The lesion was firm on palpation.

Orthopantomogram showed an ill-defined radiolucent lesion involving the mandible from the permanent left mandibular central incisor to the second molar region.(Figure 2) All the permanent teeth were present except for the mandibular left second premolar. Permanent maxillary canines of both sides and mandibular left first molar (distoangular) and second molars (mesioangular) were impacted. Dilaceration was noted apically with the roots of the impacted permanent mandibular first molar.

Figure 1

Ill-defined intra-osseous welling extending from the permanent mandibular left central incisor to the deciduous second molar region.

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/a36c5419-00ad-4964-bc46-c0ee80e4b97cimage1.png
Figure 2

Ill-defined radiolucent lesion extending from the permanent mandibular left central incisor to the deciduous second molar.

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/a36c5419-00ad-4964-bc46-c0ee80e4b97cimage2.png
Figure 3

A: Curvilinear trabeculae exhibiting typical ‘Chinese letter’ pattern; B: Absence of a definite transition zone between the trabeculae of the lesional area and the peripheral bone. (H and E, Original magnification x100)

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/a36c5419-00ad-4964-bc46-c0ee80e4b97cimage3.png
Figure 4

A: Oseeous trabeculae with osteocytes in the lacunae, and prominent resting and reversal lines. Note the clefting at the bone—stroma interface (yellow arrowheads). (H and E, Original magnification x100); B: High power view showing the classic ‘brush border’ of the trabeculae (black arrowheads). (H and E, Original magnification x400)

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/a36c5419-00ad-4964-bc46-c0ee80e4b97cimage4.png

An incisional biopsy from the lesional area along with a rim of peripheral tissue was obtained. The tissue was decalcified in 2% nitric acid for three days and subjected to routine histological processing. Microscopically, thin trabeculae exhibiting a typical ‘Chinese-letter’ pattern were noted without any demarcating zone from the peripheral trabeculae. (Figure 3) The curvilinear trabeculae were in a background of fibrocellular connective tissue stroma. Most of the trabeculae consisted of osteocytes within the lacunae but were devoid of osteoblastic rimming. Prominent resting and reversal lines could be noted. The interface between the trabeculae and stroma exhibited ‘brush borders’ and also clefting in some areas. (Figure 4)

A final diagnosis of Fibrous dysplasia was imparted. Surgical re-contouring was performed and the patient is free of symptoms after a one-year follow-up.

Discussion

FD is relatively rare in the craniofacial region (20% cases) and generally exhibits a female predilection. 10 Monostotic FD more frequently affects the maxilla than the mandible.9 Most of the lesions affect the premolar region or areas posterior to it. The anterior mandible is seldom affected. This makes the present case quite unusual in terms of clinicodemographic profile, wherein the lesion occurred in the anterior mandible without a noticeable involvement of the maxilla, in an adolescent male.

Radiographically, FD exhibits a characteristic ‘ground glass’ matrix, which is smooth, homogenous, and eccentric in the alveolar bone. While the radiological appearance of typical FD is mixed, the spectrum also includes a percentage of homogenous dense sclerotic (23%), and radiolucent (21%).11 The poorly defined margins along with histopathological confirmation are currently considered the gold standard for the diagnosis of FD.12

Histopathologically, the differential diagnosis of FD comprises the other FOLs that closely resemble each other which include ossifying fibroma and cemento-osseous dysplasia. These FOLs can only be differentiated from one another by taking into account the clinicodemographic and radiological findings. 2

While both FD and OF affect adolescents and young adults, differentiating between the two is important because while the former is a reactive phenomenon, the latter is a true neoplastic phenomenon warranting surgical removal.13 Radiographically, OF is well-defined with a sclerotic rim, indicating a confined intra-osseous benign mass.11 Histopathologically, OF shows a distinct ‘transitional zone’ between the lesional area and the normal bone.14 In the present case, the lesion was ill-defined without a sclerotic border. Furthermore, the osseous trabeculae of the lesional area and peripheral normal bone blended indistinctly without a definite zone of transition.

COD tends to affect patients of relatively older age groups, usually 30-40 years. 14 Histopathologically, spherules of cementum-like deposits are noted, that fuse to form ginger root-like trabecular masses. 13 The present case depicted classic thin curvilinear trabeculae without evidence of any cementum-like material. The trabeculae of COD are also typically devoid of the ‘brush border’ at the bone-stroma interface. SOD was only recently included in the official classification of head and neck tumors.2 It characteristically involves the maxilla and generally presents with additional extragnathic dermal features.

Generally, conservative treatment or correction of the deformity is deemed sufficient for controlling FD.5, 6, 7 Surgical resection is considered for lesions with aggressive behavior or in case of the presence of symptoms. The pathogenesis of FD involved somatic activating mutations of the GNAS-1 gene.15 The protein product, stimulatory G protein – α amplifies Interleukin-6 production, which results in an imbalance in the osteoblastic progenitor cell and osteoclastic cell activity.16, 17 It is believed that this imbalance wanes or even completely ceases after puberty when bone maturation is completed. Only rarely do the lesions continue progressing in old age; in such cases, surgical excision of the demineralized bone followed by osteoplasty may be necessary. 18

The selection of surgical modality for treatment depends on the age, willingness, and financial ability of the patients. The treatment approach should be tailored considering these factors for each individual case subjective to the clinician’s judgment and expertise. In the present case, the patient being in the pubertal age group, with the absence of symptoms and monostotic form of the disease, a conservative treatment plan was adopted.

About 1% of cases of FD may transform into a malignant lesion. Such cases generally have associated symptoms such as pain, rapid growth, or ulceration. Elevated serum alkaline phosphatase levels (AP) levels are considered a reliable indicator marker for the malignant transformation of FD.5 In the present case, the patient did not experience any symptoms and their serum calcium and AP levels were also within the normal range. Nevertheless, long-term follow-up is crucial for patients with FD even in the absence of these ‘red flags.’ A re-assessment of the serum AP levels and periodic six-monthly follow-ups were advised to the patient.

Source of Funding

None.

Conflicts of Interest

There is no conflict of interest.

References

1 

SK El-Mofty Fibro-osseous lesions of the craniofacial skeleton: an updateHead Neck Pathol20148443244

2 

M Vered J M Wright Update from the 5th Edition of the World Health Organization classification of head and neck tumors: odontogenic and maxillofacial bone tumoursHead Neck Pathol20221616375

3 

S Menon S Venkatswamy V Ramu K Banu S Ehtaih VM Kashyap Craniofacial fibrous dysplasia: Surgery and literature reviewAnn Maxillofac Surg2013316671

4 

L Lichtenstein Polyostotic fibrous dysplasiaArch Surg193836187498

5 

K I Assiri Monostotic Fibrous Dysplasia Involving the Mandible: A Case ReportSAGE Open Med Case Rep2020812050313X2093695410.1177/2050313X20936954

6 

A Berberi G Aoun E Khalaf G Aad Monostotic Fibrous Dysplasia of the Mandible in a 9-Year-Old Male Patient Treated with a Conservative Surgical Treatment: A Case Report and 15-Year Follow-UpCase Rep Dent2021996347810.1155/2021/9963478

7 

V Chandavarkar PM Patil D Bhargava MN Mishra A rare case report of craniofacial fibrous dysplasiaJ Oral Maxillofac Pathol20182234069

8 

SG Parekh R Donthineni E Ricchetti Fibrous dysplasiaJ Am Acad Orthop Surg200412530513

9 

C Ogunsalu NJ Smith A Lewis Fibrous dysplasia of the jaw bone: A review of 15 new cases and two cases of recurrence in Jamaica together with a case reportAust Dent J19984333904

10 

S Ziadi M Trimeche M Mokni B Sriha H Khochtali S Korbi Eighteen cases of craniofacial fibrous dysplasiaRev Stomatol Chir Maxillofac200911063182210.1016/j.stomax.2008.11.005

11 

YS Kushchayeva SV Kushchayev TY Glushko SH Tella OM Teytelboym MT Collins Fibrous dysplasia for radiologists: beyond ground glass bone matrixInsights Imaging20189610355610.1007/s13244-018-0666-6

12 

A Abdelkarim R Green J Startzell J Preece Craniofacial polyostotic fibrous dysplasia: a case report and review of the literatureOral Surg Oral Med Oral Pathol Oral Radiol Endod20081061495510.1016/j.tripleo.2008.03.023

13 

R Eversole L Su S Elmofty Benign fibro-osseous lesions of the craniofacial complex a reviewHead Neck Pathol200823177202

14 

BL Nelson BJ Phillips Benign fibro-osseous lesions of the head and neckHead Neck Pathol201913346675

15 

MR Dicaprio WF Enneking Fibrous dysplasia. Pathophysiology, evaluation, and treatmentJ Bone Joint Surg Am2005872184864

16 

H Youssoufian RE Pyeritz Mechanisms and consequences of somatic mosaicism in humansNat Rev Genet200231074858

17 

M Riminucci SA Kuznetsov N Cherman Osteoclastogenesis in fibrous dysplasia of bone: in situ and in vitro analysis of IL-6 expressionBone200333343442

18 

JS Lee EJ Fitzgibbon YR Chen HJ Kim LR Lustig SO Akintoye Clinical guidelines for the management of craniofacial fibrous dysplasiaOrphanet J Rare Dis201271119



jats-html.xsl


This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

Article type

Case Report


Article page

275-278


Authors Details

Sanpreet Singh Sachdev, Tabita Joy Chettiankandy, Manisha Ahire Sardar, Ashish Sarda, Ashwini Chhapane


Article History

Received : 20-08-2022

Accepted : 25-10-2022


Article Metrics


View Article As

 


Downlaod Files