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

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

• The Journal of the Korean bone and joint tumor society
• /
• v.11 no.2
• /
• pp.188-193
• /
• 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 Association of Oral and Maxillofacial Surgeons
• /
• v.35 no.5
• /
• pp.304-309
• /
• 2009

Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the $\alpha$-subunit of stimulatory G protein. FD may affect a single bone (called monostotic form) or multiple bones (called polyostotic form). The extent of lesions reflects the onset time of mutation. In this study, cells from monostotic FD in maxilla of a patient were isolated and cultured in vitro for characterization. Materials and Methods: The single cells were released from FD lesion which was surgical specimen from 15 years-old boy. These isolated cells were cultured in vitro and tested their proliferation activity with MTT assay. In osteogenic media, these cells underwent differentiation process comparing with its normal counterpart i.e. bone marrow stromal cells. The proliferated FD cells were detached and transplanted into the dordsal pocket of nude mouse and harvested in 6 weeks and 12 weeks. Results and Summary: FD cells have an increased proliferation rate and poor differentiation. As a result, cells isolated from FD lesion decreased differentiation into osteoblast and increased proliferation capacity. MTT assay presented that proliferation rate of FD cells were higher than control. However, the mineral induction capacity of FD was lesser than that of control. Monostotic FD cells make fewer amounts of bone ossicles and most of them are woven bone rather than lamellar bone in vivo transplantation. In transplanted FD cells, hematopoietic marrow were not seen in the marrow space and filled with the organized fibrous tissue. Therefore, they were recapitulated to the original histological features of FD lesion. Collectively, these results indicated that the FD cells were shown that the increased proliferation and decreased differentiation potential. These in vitro and in vivo system can be useful to test FD cell's fate and possible.

• The Journal of the Korean bone and joint tumor society
• /
• v.13 no.1
• /
• pp.22-30
• /
• 2007

Purpose: We describe clinical, radiographic, MRI and pathologic findings as well as final outcome after simple curettage and bone graft of cystic fibrous dysplasia (FD) in the long bone, which has been rarely documented in the literature. Materials and Methods: Clinical records, radiographs, MRI and histologic slides of 11 patients with cystic FD in the long bone were retrospectively analyzed. Results: Six patients complained pain for several months, 4 patients presented pain after trivial injury event, and 1 patient suffered pathologic fracture. The mode of involvement was monostotic in 10 patients and polyostotic in l patient. The femur was affected in 7 patients, the humerus in 3, and the radius in 1. Radiography showed prominent, expansive lysis associated with ground-glass density of FD. MRI revealed 2 different signals of FD and cyst. Microscopic examination revealed classic findings of FD and non-specific cystic degeneration. The final outcome was satisfactory in every patient. Local recurrence was not observed. Conclusion: Cystic FD in the long bone seems not as rare as the scarcity of reported cases would indicate. MRI features provide a basis for differential diagnosis between benign cystic change and malignant transformation. Cystic FD would be an indication for surgery and simple curettage with allo-chip-bone graft is effective.

• The Journal of the Korean bone and joint tumor society
• /
• v.11 no.2
• /
• pp.111-117
• /
• 2005

Purpose: We evaluate the results of treatment of pathologic femur fractures secondary to bone tumors in children. Materials and Methods: Between January 1995 and June 2004, 18 patients(20 cases) were evaluated. Their mean age of the first episode of fracture was 10.2 years and mean follow-up period is 42.5 months. Primary bone tumors, the location of fracture, time to union and complications were evauated. Results: Fractures occurred at proximal portion in 14 cases, shaft 3 cases and distal portion 3 cases. The bone tumors causing pathologic fracture were fibrous dysplasia(9 c ases), simple bone cyst(4 cases), aneurysmal bone cyst(4 cases), nonossifying fibroma(2 cases) and eosinophilic granuloma(1 case). In the treatment for fractures, cast was in 11 cases, internal fixation 8 cases and external fixation in 1 case. In the treatment for tumors, observation was in 11 cases, curettage & bone graft in 8 cases and resection in 1 case. In polyostotic fibrous dysplasia, all cases were treated by cast initially but deformity developed in all cases. Fracture prevention and deformity correction were obtained with intramedullary nailing. Conclusion: Adequate choice of treatment of bone tumor and fracture will result in good prognosis.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
• /
• v.28 no.1
• /
• pp.27-36
• /
• 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.