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Table of Contents   
CASE REPORT  
Year : 2020  |  Volume : 31  |  Issue : 4  |  Page : 640-643
An unusual case of polyostotic fibrous dysplasia—A case report


1 Department of Oral Pathology and Microbiology, SMBT Institute of Dental Sciences and Reserach, Dhamangoan, Nashik, Maharashtra, India
2 Department of Oral Medicine of Dental Sciences and Research, SMBT Institute of Dental Sciences and Reserach, Dhamangoan, Nashik, Maharashtra, India
3 Department of Oral and Maxillofacial Surgery, SMBT Institute of Dental Sciences and Reserach, Dhamangoan, Nashik, Maharashtra, India

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Date of Submission10-May-2018
Date of Decision15-Aug-2019
Date of Acceptance28-Oct-2019
Date of Web Publication16-Oct-2020
 

   Abstract 


Fibrous dysplasia (FD) is a fibro-osseous lesion where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to expansion. It is classified according to the number of affected bones, and its association to endocrine alterations, that is, monostotic (80–85%), polyostotic forms (20–30%), and Albright's disease. Involvement of two or more bones is termed as polyostotic FD, a relatively uncommon condition. Here we report a rare case of polyostotic FD involving facial bones, orbital bones, sphenoid bone, and spine in a 21-year-old male patient.

Keywords: Fibro-osseous lesion, fibrous dysplasia, polyostotic

How to cite this article:
Jagtap KS, Patel NR, Hiray P, Wadhwa A. An unusual case of polyostotic fibrous dysplasia—A case report. Indian J Dent Res 2020;31:640-3

How to cite this URL:
Jagtap KS, Patel NR, Hiray P, Wadhwa A. An unusual case of polyostotic fibrous dysplasia—A case report. Indian J Dent Res [serial online] 2020 [cited 2020 Oct 31];31:640-3. Available from: https://www.ijdr.in/text.asp?2020/31/4/640/298406



   Introduction Top


Fibrous dysplasia (FD) is an uncommon non-hereditary, developmental anomaly of the bone that occurs due to defect in osteoblastic differentiation and maturation.[1] It was originally described by Lichtenstein in 1938 and by Lichtenstein and Jaffe in 1942.[2] It is a sporadic condition that occurs due to postzygotic mutation in GNAS1 gene. It can affect one bone (monostotic form) or multiple bones (polyostotic form), and the latter may form part of the McCune–Albright syndrome (MAS) or the Jaffe–Lichtenstein syndrome (JLS).[3] Polyostotic FD involves multiple bones such as femur, tibia, pelvis, ribs, skull and facial bones, clavicle, lumbar and cervical spine.

As polyostotic FD is a very rare condition occurring only in 20–25% of patients, therefore the aim of this article is to report such a rare case of polyostotic FD in a 21-year-old male patient and add it to the literature.


   Case Report Top


A 21 year-old male patient reported with the chief complaint of painless swelling over the face. Initially the swelling was small in size and gradually increased till present size. No medical history was associated. On clinical examination, bilaterally extraoral diffuse swelling of approximately 5 cm × 6 cm in dimension was seen. The swelling was seen increasing in size which extended superio-inferiorly from lower eyelid to corner of mouth and reached up to lower border of mandible of left side. Anterio-posteriorly swelling was seen extending from ala of the nose to approximately 1 cm in front of pinna of ear of left side and on right side it was seen extending from ala of nose to 3 cm in front of pinnae of ear lobe. Extraorally collapsed bridge of nose, frontal bossing, hypertelorism [Figure 1], protrusion of chest and clavicle was seen [Figure 2].
Figure 1: Clinical photograph showing enlargement of facial bones

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Figure 2: Showing involvement of chest and clavicle

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Intraorally, constricted maxillary arch with high palatal vault and posterior expansion [Figure 3]a and constricted mandibular arch with anterior crowding was seen. Swelling was seen in mandibular arch with expansion of buccal cortical plates in the anterior region extending superio-inferiorly from cervical region of anterior teeth to labial vestibule and anterio-posteriorly from the midline to second premolar on right side and up to first premolar on left side [Figure 3]b. Overlying mucosa appeared to be pigmented and stretched. Swelling was bony hard, non-tender and no paresthesia was associated.
Figure 3: (a) Intraoral photograph showing constricted upper arch and expansion of maxillary arch in posterior region. (b) Showing expansion of mandibular bone

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Based on clinical findings, provisional diagnosis of fibro-osseous lesion was made. Differential diagnosis such as Ossifying fibroma, Paget's disease, and focal cementosseous dysplasia were considered for bony hard swelling. Furthermore, the patient was advised investigations such as complete hemogram, serological investigations such as serum calcium and phosphorous, alkaline phosphatase level, orthopantomogram (OPG), CT scan, and incisional biopsy. Complete hemogram showed normal parameters and serological investigations were within normal limits.

OPG showed expansion of bone and thinning of outer cortex. Abnormal trabecular pattern with irregular shape, displacement of teeth, mixed radiolucent and radiopaque pattern, and ground glass to orange peel appearance and cystoid appearance was also seen [Figure 4].
Figure 4: OPG showing radiographic pattern of cystoid and ground-glass to orange peel appearance

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Occlusal radiograph showed expansion of maxillary arch in posterior region [Figure 5]. CT scan revealed expansion of mandible and bulging out of lesions. Mandibular lesions were seen encroaching the tooth roots and maxillary lesions were seen to be occupying maxillary sinus. Generalized calvarial and facial bone thickening was noted [Figure 6]. Furthermore, incisional biopsy was done and tissue specimen was sent to the department of oral pathology for histological examination.
Figure 5: Occlusal radiograph showing maxillary expansion

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Figure 6: CT-image showing extensive involvement of facial bones

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Histopathology of the tissue showed irregularly shaped bony trabeculae embedded within moderately cellular fibrous stroma [Figure 7]a and [Figure 7]b. Immature bone with large osteocytes in lacunae resembling Chinese letter configuration was seen. Osteoblastic rimming was absent [Figure 7]a. Basophilic reversal lines were not prominent. Mature lesion showed broad bony trabeculae with lamellar maturation [Figure 7]c. Based on histopathological findings, diagnosis of fibro-osseous lesion was made. After the clinical and radiological features were correlated, final diagnosis of polyostotic FD was made.
Figure 7: (a) and (b) Histopathological image showing irregularly shaped trabeculae of bone in a fibrous stroma with absence of osteoblastic rimming. (c) High power view showing mature lesion of fibrous dysplasia with broad bony trabeculae, lamellar maturation of bone, and large osteocytes in lacunae

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   Discussion Top


Fibrous dysplasia (FD) is a benign disease of bone that was originally described by Lichtenstein more than 60 years ago.[4] The disease was labeled polyostotic FD by Lichtenstein in 1938, and it was Lichtenstein and Jaffe who initially described the spectrum of the clinical, radiological, and histological features. Additionally, they introduced the idea that FD results from the “perverted activity of the specific bone-forming mesenchyme.” [3] FD is classified as follows:

Classification[5]

  1. Monostotic FD
  2. Polyostotic FD


    1. Jaffe's type
    2. Albright syndrome


  3. Craniofacial form
  4. Cherubism


Patients with FD show bone deformity and facial asymmetry more commonly as compared to normal patients. Polyostotic FD has its onset mainly in children younger than 10 years of age and the lesions grow with the child and stabilize after puberty. The ratio of occurrence of polyostotic to monostotic FD is 3:7 and gender prevalence in monostotic and polyostotic fibrous dysplasia is equal.[6] Polyostotic form involves more than one bone ranging from group of few to most of the bones in the skeletal system. The sites of involvement are femur, tibia, pelvis, ribs, skull, facial bones, upper extremities, spine, clavicle.[1] Here we report a case of polyostotic FD in a 21-year-old male patient showing involvement of facial bones, frontal bone, chest, ribs, clavicle. The oral manifestations in the form of polyostotic FD includes painless swelling, bulging of the jaw involving commonly buccal plate, in the mandible sometimes it causes protruberent excrescence of the inferior border.[1] Similar findings too were seen in our case. Radiographically, the borders of FD are known to be ill defined with loss of lamina dura around teeth due to replacement of normal bone.[7] As the lesion matures, they tend to become more radiopaque with marked homogeneous increase in bone density associated with bone expansion. This is sometimes referred to as a ground-glass appearance or orange peel.[8] Radiographically, similar findings were seen in our case. Irregular bony trabeculature along with variations in cortical thickness are seen, such as pseudo type (ground-glass or orange peel appearance), whorled—plaque like, diffuse—sclerotic type, cyst-like, pagetoid-like.[9] In the present case, ground-glass and cystoid-like appearance was seen on OPG.[1] Histologically, fibrous dysplasia shows irregular bony trabeculae of immature bone in a cellular loosely arranged fibrous stroma. Bony trabeculaeassuming curvilinear shape like Chinese script writing are seen. There is absence of osteoblastic rimming around the bony trabeculae and they are seen to be not connected to each other.[1],[10] Similar findings were also seen in our case. Laboratory findings in the FD patients show no consistent significant changes in the serum calcium or phosphorous, although serum alkaline phosphatase level is sometimes elevated.[1] In our case, laboratory findings were found to be within normal limits. Differential diagnosis of FD includes ossifying fibroma, focal cemento-osseous dysplasia, and Paget's disease. Ossifying fibroma occurs in third and fourth decade of life, females are more commonly affected, whereas FD often occurs in second decade and males and females are equally affected.[11] The age of the patient in the present case is 21 years and is a male. Focal cemento-osseous dysplasia shows single site of involvement, it occurs in 90% of females at the age of 38 years. Radiographically, focal cemento-osseous dysplasia shows mixed radiolucent and radiopaque pattern most commonly in the extraction site.[11] No such findings were seen in this case and hence this diagnosis was ruled out. Other differential diagnosis was Paget's disease which occurs in older population. Radiographically, it shows cotton-wool appearance and hypercementosis. There is increased levels of serum alkaline phosphatase level in Paget's disease. Histologically, there is presence of prominent reversal lines.[8],[11] However, these findings were not similar with the present case, so Paget's disease was also ruled out.

Treatment of FD in case of asymptomatic and stable lesions includes just monitoring of the patient.[6] Management of FD usually involves osseous recontouring at the affected site to improve esthetics and function after the active growth cessation.[8] In polyostotic form, bisphosphonate therapy may be used for the management and it leads to increase in the bone density and reduces the pain.[6] In the present case, patient was kept under follow-up and was planned for further treatment.


   Conclusion Top


FD is a benign bone lesion characterized by progressive replacement of normal bone by fibrous tissues. The main complications of FD are pathologic fracture, secondary aneurysmal bone cyst formation, and rarely malignant change. So proper understanding, investigations, and management to diagnose such types of cases in initial stages is of prime importance to prevent any kind of complications. We report this case, of polyostotic FD as it is the rarest type and has very less percentage of occurrence of just about 20–25%, it is our attempt to add further knowledge to the literature.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Sivapathasundharam N. Bone and Joint Diseases. Shafer's Textbook of Oral Pathology. 8th ed. Elsevier; 2016. p. 462-6.  Back to cited text no. 1
    
2.
Dua N, Singla N, Arora S, Garg S. Fibrous dysplasia of maxilla: Report of two cases. J Indian Acad Oral Med Radiol 2015;27:472-5.  Back to cited text no. 2
  [Full text]  
3.
Agarwal M, Balaji N, Sumathi MK, Sunitha JD, Dawar G, Rallan NS. Fibrous dysplasia: A review. TMU J Dent 2014;1:25-9.  Back to cited text no. 3
    
4.
Chen YR, Chang CN, Tan YC. Craniofacial fibrous dysplasia. Chang Gung Med J 2006;29:543-9.  Back to cited text no. 4
    
5.
Dumitrescu CE, Collins MT. McCune-Albright syndrome. Orphanet J Rare Dis 2008;3:1-12.  Back to cited text no. 5
    
6.
Vikram K, Akhil S. Polyostotic fibrous dysplasia: A case report. JOJCS 2017;3:555601.  Back to cited text no. 6
    
7.
White SC, Pharoah MJ. editors. Oral radiology. Principles and Interpretation. 5th ed. St. Louis: Mosby Inc; 2004.  Back to cited text no. 7
    
8.
Sachdeva SK. Craniofacial fibrous dysplasia in an elderly patient: A case report with a review of literature. Acta Stomatol Croat 2015;49:60-4.  Back to cited text no. 8
    
9.
Raju R. Polyostotic fibrous dysplasia: A case report. Ann Essences Dent 2012;1:22-5.  Back to cited text no. 9
    
10.
Neville BW, Damn DD, Allen CM. Bone pathology. Oral and Maxillofacial Pathology. 3rd ed. Elsesvier; 2009. p. 624-5.  Back to cited text no. 10
    
11.
Michael G. Benign lesions of the oral cavity and the Jaws. Burket's Oral Medicine. 12th ed. People's Medical Publishing House-USA; 2015. p. 162.  Back to cited text no. 11
    

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Correspondence Address:
Dr. Nikita Rajendrakumar Patel
Department of Oral Pathology, SMBT Institute of Dental Sciences and Research, Dhamangoan, Nashik, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdr.IJDR_409_18

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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