• The Journal of the Korean bone and joint tumor society
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• v.14 no.1
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• pp.62-67
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• 2008

Malignant degeneration of fibrous dysplasia is rare and involves transformation into osteosarcoma, fibrosarcoma and chondrosarcoma. The most frequent sites involved in malignant transformation were craniofacial bones, proximal femur, humerus, pelvis, tibia and scapula in a decreasing order of frequency. An 41-year-old man with a history of polyostotic fibrous dysplasia presented with increasing left arm pain. Plain radiograph showed expansile destructive lesion along the humeral shaft. As initial biopsy report was low grade chondrosarcoma, he underwent marginal resection. However, he developed local recurrence 7 month later and subsequent pathologic finding was chondroblastic osteosarcoma. We report one case of secondary chondroblastic osteosarcoma from polyostotic fibrous dysplasia initially misdiagnosed as low grade chondrosarcoma that caused fallacy in treatment strategy.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.42
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• pp.31.1-31.7
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• 2020

Background: Fibrous dysplasia (FD) is a rare, sporadic, and benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. FD localized in the cranial and facial bones is called craniofacial fibrous dysplasia (CFD). Cystic degeneration in CFD cases is rare; cystic degeneration appearing in both the maxilla and the mandible FD lesion is even rarer. The aim of this article was to report a case of fibrous dysplasia of the mandible and maxilla complicated by nonspecific cystic degeneration. Case presentation: A 30-year-old woman presented with a rare case of non-specific cystic degeneration in a mandible and maxilla FD lesion that occurred 11 years after surgery. She was diagnosed with polyostotic CFD and underwent maxillary and mandibular bone contouring. Cyst enucleation under general anesthesia was performed in the mandibular region due to pain and discomfort. Conclusions: In cases involving non-aggressive and non-invasive FD cystic degeneration in focal areas, conservative treatment is recommended. However, if cystic degeneration of FD develops rapidly and causes discomfort, pain, or dysfunction, surgical treatment should be considered.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.12 no.2
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• pp.95-101
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• 1990

This is a case report of polyostotic fibrous dysplasia developed in the craniofacial region of 21 year old male patient, who had complained the buccolingual expansion of left mandibular body area, malocclusion and facial asymmetry. We could achieve satisfactory results by radical resection of the relatively well defined small lesion of mandible and by cosmetic bone shaving procedure on the widely dispersed and poorly defined lesions of cranium. But the persistent growth and recurrence of the lesions may produce loss of hearing, visual difficulties, facial paralysis and anosmia, and as it is a polyostotic type occured in the craniofacial region of male patient, the possibility of malignant degeneration should not be excluded completely and periodic recall and check up will be necessary.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.12 no.3
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• pp.68-73
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• 1990

Fibrous dysplasia is an idiopathic skeletal disorder in which medullary bone is replaced and disturbed by poorly organized, structually unsound fibroosseous tissue, which may produce cortical expansion. When facial bones are involed, 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 arises as a resujlt of trauma. It occurs predominantly in infant, adolescent females and runs a variable clinical course. When several bones are involed, it tends to be unilateral. Involements of alveolar bone may produce displacement of teeth with malocclusion, or loss of teeth, or both. Radiographycally, it shows an indistinctly delimited osteolytic defect with a bubble - like pattern, but without a sclerotic rim. The preferred treatment is almost always surgery. If the lesion is extensive, surgical intervention with use of recontouring procedures aimed at the correction of esthetic or funtional disturbances is preferred treatment. Now, we present a case of fibrous dysplasia on the left maxilla and the zygoma treated by bony contourign via hemicoronal flap and intraoral approach with good results.

• The Journal of the Korean dental association
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• v.19 no.3 s.142
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• pp.213-213
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• 1981

• The Journal of the Korean bone and joint tumor society
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• v.15 no.1
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• pp.87-91
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• 2009

Fibrous dysplasia is a developmental benign disorder in which normal bone is replaced by fibrous tissue. Malignant transformation of fibrous dysplasia is a rare complication and more commonly occur in the polyostotic form than monostotic form. We report a case in which osteosarcoma developed in area of fibrous dysplasia of proximal femur after treating with curettage, wedge osteotomy, internal fixation, and bone graft. A review of the literature is presented.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.237-240
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• 1999

The presence of polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and precocious sexual development in children is known as the McCune-Albright syndrome. In addition to the described in McCune-Albright syndrome, other endocrinopathies have been reported including hyperthyroidism, acromegaly, Cushing syndrome and vitamin D resistant rickets. The case describes a 6-year-old boy showing bony deformities due to polyostotic fibrous dysplasia, hyperpigmented skin macules, hyperthyroidism and vitamin D resistant rickets. The purpose of this report is to describe a patient of McCune-Albright syndrome with vitamin D resistant rickets which is very rare.

• 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.

• The Journal of the Korean bone and joint tumor society
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• v.11 no.2
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• pp.188-193
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• 2005

Fibrous dysplasia is a condition characterized by the replacement of the medullary component of predominantly long bone with fibrous tissues. The monostotic form of the fibrous dysplasia occurs more frequent than the polyostotic form. Only the twenty three previous cases of vertebral involvement in monostotic fibrous dysplasia have been reported. Authors experienced a case of monostotic fibrous dysplasia of the third lumbar vertebra, so we report this case. Report details the diagnosis and treatment of a 34 years old man with back pain from monostotic fibrous dysplasia of the third lumbar vertebra. We discuss the experience in the consideration of previous report to recommend the optimal management of this disease.

• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1346-1351
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• 2021

Mazabraud syndrome is a rare benign disease that is accompanied by polyostotic fibrous dysplasia and intramuscular myxoma. Malignant transformation of fibrous dysplasia occurs in approximately 1% of cases. To date, only eight cases of malignant transformation, of fibrous dysplasia to osteosarcoma, in Mazabraud syndrome have been reported worldwide. The authors report the first case of osteosarcomatous transformation in a patient with Mazabraud syndrome in the Republic of Korea, focusing on imaging findings.