• Title/Summary/Keyword: Fibrous dysplasia

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A CLINICOPATHOLOGIC STUDY ON FIBROUS DYSPLASIA OF THE MAXILLOFACIAL REGIONS (악안면 부위에 발생한 섬유성 골이형성증에 대한 임상조직병리학적 연구)

  • PYO, Sung-Woon;Nam, Il-Woo
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.14 no.1_2
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    • pp.124-134
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    • 1992
  • Fibrous dysplasia is a benign pathologic condition of bone in which fibrous tissue gradually expands and replaces normal bone into fibro-osseous lesion. It is a primary developmental abnormality of bone-forming mesenchyme in origin. This study shows clinical history, radiological and histopathological feature of fibrous dysplasia with the intention of establishing correct diagnosis, treatment plan and evaluation of prognosis. This paper reviews and summarizes the materials from 57 fibrous dysplasias submitted to the Department of Oral and Maxillofacial Surgery in College of Dentistry, Seoul National University. Conclusions obtained were as following : 1. Fibrous dysplasia developed mainly in teenagers and shows female predeliction. 2. Fibrous dysplasia developed much on the maxilla 3. Monostotic fibrous dysplasia was most popular form. 4. Main symptom of fibrous dysplasia was painless swelling. 5. Radiological feature of fibrous dysplasia was ground-glass appearance, 6. Histopathological feature of fibrous dysplasia was irregular immature bony trabeculae(woven bone). 7. Treatment of fibrous dysplasia was mainly conservative contouring surgery.

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Expression of c-fos Protein in Fibrous Dysplasia and Osteofibrous Dysplasia (섬유성 이형성증 및 골섬유성 이형성증에서의 c-fos 단백 발현)

  • Park, Hye-Rim;Park, Yong-Koo;Kim, Duck-Whan
    • The Journal of the Korean bone and joint tumor society
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    • v.5 no.4
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    • pp.216-220
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    • 1999
  • The purpose of this study was to evaluate the role of c-fos oncogenes in the development of fibrous dysplasia and osteofibrous dysplasia. The immunohistochemical expression of c-fos protein was evaluated in 15 cases of fibrous dysplasia and 8 cases of osteofibrous dysplasia. Ten cases of fibrous dysplasia were weakly positive with c-fos. Six cases of osteofibrous dysplasia were weakly positive and the remaining two cases were strongly positive. The overall expression of c-fos protein is weaker than high-grade osteosarcoma, thus the implication of c-fos protein is little in the development of these tumors. Fibrous dysplasia and osteofibrous dysplasia share some features of characteristic histology and c-fos expression.

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A Radiographic study of Fibro-osseous lesions of the jaw bones (악골 섬유성 골병소의 방사선학적 연구)

  • Kwon Kyung-Yun;Choi Karp-Shik
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.28 no.1
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    • pp.27-36
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    • 1998
  • The purpose of this study was to obtain information on the clinical and radiographic features of the fibro-osseous lesions in the jaws. For this study, the author examined and analysed the clinical records and radiographs of 71 cases of 68 patients in fibrous dysplasia, 35 cases of ossifying fibroma and 30 cases of 16 patients of periapical cemental dysplasia diagnosed by clinical and radiographic or histopathological examinations. The obtained results were as followings: L Fibrous dysplasia occurred most frequently in the 2nd decade (30.0%), ossifying fibroma in the 3rd-4th decades, periapical cemental dysplasia in the 4th decade, and all of three lesions showed slight predilection in females. In most cases, chief complaints were painless facial swelling in fibrous dysplasia and ossifying fibroma, and periapical cemental dysplasia was found accidentally in radiographs. 2. Fibrous dysplasia was occurred more frequently in maxilla, ossifying fibroma in mandible and both lesions in premolar-molar area. Periapical cemental dysplasia was occurred most frequently in the mandibular anterior area. The size of fibrous dysplasia was larger than that of ossifying fibroma, and the shape of ossifying fibroma was more round and elliptical than fibrous dysplasia whose was fusiform. 3. Fibrous dysplasia was shown homogeneous radiopaque shadow of 57.6% and ossifying fibroma & periapical cemental dysplasia were shown mixed appearance of radiolucency and radiopacity shadows at 74.2%, 60.0%, respectively. 4. Fibrous dysplasia was entirely shown poorly defined at 87.7%, but ossifying fibroma & periapical cemental dysplasia were shown well outlined at 60.0%, 70.0%, respectively. 5. Cortical thinning and expansion were observed in fibrous dysplasia and ossifying fibroma, and severe in ossifying fibroma than fibrous dysplasia, and those signs were not seen in periapical cemental dysplasia. Loss of lamina dura was dominant in fibrous dysplasia and root resorption was dominant in ossifying fibroma. Displacement of mandibular canal and the degree of the increase of vertical dimension were alike in both lesions. Displacement of maxillary sinus or nasal cavity, thinning & expansion of the maxillary sinus were dominant in fibrous dysplasia. 6. Polyostotic fibrous dysplasia was occurred at 5.9%, Multiple periapical cemental dysplasia at 43.7%. Occurrence rate in the edentulous area of fibrous dysplasia and ossifying fibroma were 7.0%, 8.6%, respectively.

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Current concepts of craniofacial fibrous dysplasia: pathophysiology and treatment

  • Dong Yeon Kim
    • Archives of Craniofacial Surgery
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    • v.24 no.2
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    • pp.41-51
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    • 2023
  • Fibrous dysplasia is an uncommon genetic disorder in which bone is replaced by immature bone and fibrous tissue, manifesting as slow-growing lesions. Sporadic post-zygotic activating mutations in GNAS gene result in dysregulated GαS-protein signaling and elevation of cyclic adenosine monophosphate in affected tissues. This condition has a broad clinical spectrum, ranging from insignificant solitary lesions to severe disease. The craniofacial area is the most common site of fibrous dysplasia, and nine out of 10 patients with fibrous dysplasia affecting the craniofacial bones present before the age of 5. Surgery is the mainstay of treatment, but the technique varies according to the location and severity of the lesion and associated symptoms. The timing and indications of surgery should be carefully chosen with multidisciplinary consultations and a patient-specific approach.

Radiographic Differential Diagnosis between the Fibrous Dysplasia and the Ossifying Fibroma (섬유성이형성증과 골화섬유종의 방사선학적 감별진단)

  • Choi Karp-Shik
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.29 no.1
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    • pp.55-63
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    • 1999
  • The author observed and compared the radiographic features of 49 cases of the fibrous dysplasia and 14 cases of the ossifying fibroma in the osteoblastic or mature stage radiologically and histopathologically. The obtained results were as follows: 1. Fibrous dysplasia occurred most frequently in the 2nd decade, but ossifying fibroma in the 3rd and 4th decades, and both lesions occurred with slight predilection in females. 2. In most cases, chief complaints were painless facial swelling. And 61.1% of fibrous dysplasia occurred in the maxilla, 92.9% of ossifying fibroma in the mandible, and most of these lesions occurred in the premolar-molar region. 3. In the mandibular lesions, ossifying fibroma was shown more oval and round shape. but fibrous dysplasia was shown fusiform shape. 4. Fibrous dysplasia was shown homogeneously distributed. complete radiopaque shadow at 63%, and ossifying fibroma was shown concentric. mixed appearance of radiolucent and radiopaque shadow at 92.9%. 5. Fibrous dysplasia was entirely shown poorly outlined and blended to normal surrounding bone, but ossifying fibroma was shown well-defined border. 6. Cortical thinning and expansion were observed in these lesions. but degree of cortical expansion was more severe in ossifying fibroma than fibrous dysplasia. 7. Loss of lamina dura. tooth displacement. and displacement of mandibular canal were observed in both lesions. but root resorption was observed in ossifying fibroma only.

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Malignant Transformation of Fibrous Dysplasia on Anterior Skull Base

  • Lee, Chul-Jae;Kim, Seong-Min
    • Journal of Korean Neurosurgical Society
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    • v.37 no.5
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    • pp.383-385
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    • 2005
  • Malignant transformation of fibrous dysplasia is a rare condition and the incidence is estimated at 0.4% for fibrous dysplasia and 4% for Albright's syndrome. The authors did not find a reported case of malignant change at skull base around the orbit in the literature. We experienced a case of fibrous dysplasia, in which neurologic symptoms were aggravated due to malignant change around the orbit, and report its favorable outcome obtained with total surgical removal.

A RADIOGRAPHIC STUDY OF FIBRO-OSSEOUS LESIONS IN THE JAWS (악골의 섬유성골병소에 관한 X선학적 연구)

  • Lee Mee Kyung;Lee Sang Rae
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.21 no.2
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    • pp.261-273
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    • 1991
  • This study was undertaken to document and better defined this condition to help clarify this clinical and radiographical appearances by the analysis of clinical and radiographical features of fibro-osseous lesions in the jaws. A study was made of a series of 128 cases with fibro-osseous lesions. The obtained results were as follows. 1. Fibrous dysplasia of the jaws occurred with equal predilection for males and females. But the females occurred in 68% of cemento-ossifying fibroma and 75% of periapical cemental dysplasia. 2. 43% of fibrous dysplasia and 32% of cemento-ossifying fibroma occurred in the 2nd decades and 33% of periapical cemental dysplasia in 5th decades. 3. 62% of fibrous dysplasia occurred in the maxilla, 73% of cemento-ossifying fibroma in mandible, 90% of periapical cemental dysplasia in mandible. 4. 98% of fibrous dysplasia occurred in premolar-molar region, 77% of cemento-ossifying fibroma in molar region, 68% of periapical cemental dysplasia in incisor region. 5. In serial radiographic features, mature stage were 55% of fibrous dysplasia, 45% of cemento-ossifying fibroma, 59% of periapical cemental dysplasia. 6. 87% of fibrous dysplasia had monostotic lesion, 67% of periapical cemental dysplasia had multiple lesions. 7. In fibrous dysplasia and cemento-ossifying fibroma, migration of tooth occurred in 61.7% and 36.4%, retention of tooth occurred in 4.3% and 9.1%, loss of lamina dura occurred in 6.4% and 9.1%, and root resorption had not occurred in fibrous dysplasia, but occurred in 18% of cemento-ossifying fibroma, displacement of mandibular canal occurred in 14.9% and 31.8%.

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Pediculated Fibrous Dysplasia in Maxillary Sinus: A Case Report

  • Kim, Jung Ho;Lee, Baek Soo;Kwon, Yong Dae;Choi, Byung Joon;Lee, Jung Woo;Lee, Hyun Woo;Kim, Do Seop;Ohe, Joo Young
    • Journal of Korean Dental Science
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    • v.8 no.1
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    • pp.36-40
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    • 2015
  • Fibrous dysplasia is a bone disorder characterized by progressive replacement of normal bone by fibrous bone tissue. Common involving sites of fibrous dysplasia are the skeletal system including long bones, ribs, craniofacial bones and the pelvis. If maxilla were affected by fibrous dysplasia, antrum is almost always involved. And fibrous dyplasia in maxillary sinus were followed the shape of bone. In our case, the lesion involves antrum but, its shape was different from typical fibrous dysplasia pattern of maxillary sinus. Therefore we report a case of monostotic pediculated fibrous dysplasia in the maxillary sinus with a review of literature.

FIBROUS DYSPLASIA ON LEFT MAXILLOFACIAL REGION (좌측 악안면부위에 발생한 섬유성 골이형성증 치험례)

  • Lim, Seok-kyun;Yeo, Hwan-Ho;Kim, Young-Kyun;Kim, Su-Gwan
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.20 no.3
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    • pp.232-236
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    • 1998
  • Fibrous dysplasia is a benign pathologic condition of bone which medullary bone is replaced and disturbed by poorly organized, structually unsound fibro-osseous tissue. When facial bones are involved, considerable esthetic deformity may result. The term monostotic fibrous dysplasia has been applied when one bone is involved : when more than one bone is affected, the term polyostotic used. The polyostotic form may be accomplished by pigmented skin lesion (Jaffe type), or by pigmented skin lesions with endocrine disturbance (Albright syndrome). No general agreement exists on the cause of fibrous dysplasia. A few authors have suggested that fibrous dysplasia as a result of trauma. It occurs predominantly in infant, adolescent females and runs a variable clinical coures. When several bones are involved, it tends to be unilateral. Involvements of alveolar bone may produce displacement of teeth with malocclusion, or loss of teeth, or both. Now, we will present a case of fibrous dysplasia on the left facial region treated by conservative contouring surgery.

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Computed tomographic features of fibrous dysplasia of maxillofacial region

  • Sontakke, Subodh Arun;Karjodkar, Freny R.;Umarji, Hemant R.
    • Imaging Science in Dentistry
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    • v.41 no.1
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    • pp.23-28
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    • 2011
  • Purpose : This study was to find the computed tomographic features of fibrous dysplasia of the maxillofacial region. Materials and Methods : All eight cases included in the study reported either to Government Dental College and Hospital or Nair Hospital Dental College, Mumbai between 2003 and 2009. The patients were prescribed computed tomogram in addition to conventional radiographs of maxillofacial region which were studied for characteristic features of fibrous dysplasia. The diagnosis of fibrous dysplasia was confirmed by histopathological report. Results : All cases showed the ill-defined margins of lesions except in the region where the lesions were extending to cortex of the involved bone. Internal structure of all cases showed ground glass appearance. Four cases of maxillary lesion showed the displacement of maxillary sinus maintaining the shape of maxillary sinus. Two cases showed complete obliteration of maxillary sinus. Displacement of inferior alveolar canal did not follow any typical pattern in any of the cases but was displaced in different directions. Conclusion : The craniofacial type of fibrous dysplasia is as common as fibrous dysplasia of jaw. The margins, extent, internal structure and effect on surrounding structure are well detected on computed tomographic images.