• Maxillofacial Plastic and Reconstructive Surgery
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• 제14권1_2호
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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.

• 대한두개안면성형외과학회지
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• 제24권2호
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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.

• Journal of Korean Neurosurgical Society
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• 제48권6호
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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.

• Journal of Korean Dental Science
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• 제8권1호
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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.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제20권3호
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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.

• 대한두경부종양학회지
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• 제37권1호
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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.

• Archives of Plastic Surgery
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• 제34권3호
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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.

• 치과방사선
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• 제24권1호
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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.

• Investigative Magnetic Resonance Imaging
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• 제13권2호
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• pp.203-206
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• 2009

섬유성 이형성증은 원인불명의 섬유조직의 양성질환으로 정상적인 해면골이 섬유조직과 불규칙적인 골소주의 증식에 의해 점진적으로 대체되는 특징을 가지며 단골성 또는 다골성으로 발생한다. 단골성의 경우 주로 늑골, 대퇴골, 경골 등에서 흔히 발생하지만 손에 발생하는 경우는 매우 드문 것으로 알려져 있다. 저자들은 13세 남아의 중수골에 발생한 단골성 섬유성 이형성증의 단순X선촬영과 자기공명영상의 소견을 중심으로 증례보고 하고자 한다.

• 대한골관절종양학회지
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• 제5권4호
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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.