• Title/Summary/Keyword: Fibrous Dysplasia of Bone

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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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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.

Fibrous Dysplasia with Aneurysmal Bone Cyst Presenting as Painful Solitary Skull lesion

  • Lee, Jung-Won;Kim, Jae-Hoon;Han, Seung-Hoon;Kang, Hee-In
    • Journal of Korean Neurosurgical Society
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    • v.48 no.6
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    • pp.551-554
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    • 2010
  • We report a rare case of fibrous dysplasia with the development of a secondary aneurysmal bone cyst presenting as solitary tumor of calvarium. Although fibrous dysplasia with aneurysmal bone cyst is rare, it should be taken into account in differential diagnosis of the osteolytic solitary skull lesion.

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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Treatment of Fibrous Dysplasia of the Fronto-Orbital Area with Radical Resection and Autogenous Reconstruction Using Split Calvarial Bone Graft: A Case Report (전두-안와 구역에 발생한 섬유성 이형성증의 근치적 절제술 및 자가두개골 이식을 이용한 재건을 통한 치료: 증례보고)

  • Choi, Ji-An;Kwak, Jung-Ha;Yoon, Chung-Min
    • Korean Journal of Head & Neck Oncology
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    • v.37 no.1
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    • pp.57-61
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    • 2021
  • Fibrous dysplasia is a bone condition characterized by the replacement of normal bone tissue and the medullary cavity by abnormal fibrous tissues. Craniofacial fibrous dysplasia causes facial asymmetry compromising the aesthetics as well as vision and hearing. A 21-year-old male visited the clinic due to vertical orbital dystopia and exophthalmos that had developed over the previous 2 months. The patient was diagnosed with a fibrous dysplasia of the frontal, ethmoid bones and superior orbital wall. By a bicoronal incision on the scalp, the radical resection of the lesions was done. After harvesting the remaining frontal bone, we did the autogenous reconstruction using split calvarial bone graft. Postoperatively, the vertical orbital dystopia and exophthalmos significantly improved. The patient is satisfied with the surgical outcomes and has not reported any recurrence.

Malignant Transformation of Fibrous Dysplasia: A Case Report of Malignant Fibrous Histiocytoma of Facial Bone (안면골 섬유 이형성증이 악성 섬유성 조직구증으로 악성화된 환자의 치험례)

  • Lee, Sang Joon;Lim, So Young;Oh, Kap Sung;Bang, Sa Ik;Hyon, Won Sok;Mun, Goo Hyun
    • Archives of Plastic Surgery
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    • v.34 no.3
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    • pp.403-405
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    • 2007
  • Purpose: Malignant degeneration of fibrous dysplasia is an uncommon recognized complication of this disease. Especially, degeneration of fibrous dysplasia to malignant fibrous histiocytoma(MFH) in facial bone is rare and the publications had been limited. The purpose of this report is to share our experience. Methods: A 46-year-old patient with facial fibrous dysplasia visited our clinic for recent facial tingling and swelling. Malignant degeneration of fibrous dysplasia was suspected. Results: Total excision of the mass and adjacent facial bone was performed. Defect was immediately reconstructed with bone graft and bone cement. At a month follow up, metastasis was detected at ipsilateral parotid gland. Superficial parotidectomy and neck dissection was performed. The patient is currently taking chemotherapy. Conclusion: Because of the uncommon presentation of this entity, clinical course of treatment was dependent on other histological types of malignant degeneration. We report this case to share our experience.

POLYOSTOTIC FIBROUS DYSPLASIA (다골성 섬유성 이형성증)

  • Lee Kang-Sook;Park Sang-Eok;Choi Karp-Shik
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.24 no.1
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    • pp.189-193
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    • 1994
  • Fibrous dysplasia is a benign fibro-osseous condition that is replacement of normal bone and marrow tissues by cellular fibrous tissue and immature bone, and it is divided into monostotic type and polyostotic type. Polyostotic fibrous dysplasia involves multiple bones, such as skull, jaw bones, femur and tibia. And it is also divided into two forms: the less severe Jaffe's type and the more severe Albright's syndrome. Clinically, it frequently occurs in the 2nd decade, and occurs more frequently in maxilla than in mandible. And the lesions of fibrous dysplasia tend to become static as skeletal maturity is reached. The authors experienced three cases of polyostosic fibrous dysplasia in the craniofacial area with the complaints of facial asymmetry due to painless swelling. And we discussed the clinical, radiological, and histopathological features of these cases with a brief review of the literatures.

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Monostotic Fibrous Dysplasia in the Metacarpal Bone: A Case Report (중수골에 발생한 단골성 섬유성 이형성증: 1예 보고)

  • Cho, Han-Il;Park, Sung-Hoon;Juhng, Seon-Kwan
    • Investigative Magnetic Resonance Imaging
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    • v.13 no.2
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    • pp.203-206
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    • 2009
  • Fibrous dysplasia is a slowly progressive, benign disorder characterized by fibrous tissue replacement of skeleton and may affect solitary or multiple bones. Monostotic fibrous dysplasia mainly occurrs in the rib, femur and tibia, however, rarely in the hand. We report a case of monostotic fibrous dysplasia confined to the 2nd metacarpal bone with findings of plain radiographs and MR imaging.

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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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