• 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.

• The Korean Journal of Nuclear Medicine
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• v.25 no.2
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• pp.219-226
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• 1991

골 신티 그라피는 섬유성 골 이형성증의 초기 병소와 다골성 형 섬유성 골 이형성증의 진단에 예민한 검사 방법이나, 양성 또는 악성 골 병변과의 감별진단에 어려움이 있었다. 이에 저자들은 조직학적으로 확진된 섬유성 골 이형성증 환자 17명에서 (단골성 형 12명, 다골성 형 : 5명) $^{99m}Tc-MDP$ (20 mCi 또는 7,400 MBq) 주사후 $2\sim4$시간에 전면, 후면 전신상과 부분상을 얻은 30부위 병소를 X선 사진과 비교분석 하였으며 CT, MRI 영상과 병리조직 소견을 얻었다. 병소 침습 부위별로는 대퇴골-10예, 경골-4, 요골-2, 늑골-2, 척추골-2, 후두골-2, 장골-2, 두정골-1, 하악골-1, 접형골-1, 견갑골-1, 쇄골-1, 척골-1예 였으며 5명의 다골성 형에서는 :우 대퇴골과 우 비골 : 양측 말단 대퇴골 :좌 경골과 좌 대퇴골 :우 요골과 우 장골 양측 대퇴골, 양측 경골, 접형골, 두정골, 척추골, 늑골과 장골이었다. 골 신티 그라피 소견상 30예중 28예에서(28/30, 93.3%) 방사능 섭취 증가를 보였으며 2예에서(2/30, 6.7%)는 정상 방사능 섭취 소견을 보였으나 각각은 X선상 불투명 유리상과 골흡수 병변을 보였다. 하악골의 골 3상 스캔상 병소에 현저한 혈류 증가가 관찰되었다. X선 소견상 30예중 11예에서 (l1/30, 36.7%) 불투명 유리상의 병변을, 골 변형을 동반한 1예를 포함한 18예에서는 (18/30, 60.0%) 골흡수 병변을 보였으며, 1예의 늑골 병변은 정상소견을 보였다(1/30, 3.3%). 이상에서 골 신티 그라피 만으로 섬유성 골 이형성증을 진단하는데는 주의를 요하나 골 대사의 동적 측면인 혈류와 골 재형성 양상 특히, 초기 병변과 단골성 형 침습에서 다골성 형을 진단하는데 필수적이며 결론적으로 골 신티 그라피와 X선 촬영등은 섬유성 골 이형성증 진단에 상호보완적인 검사 방법으로 사료된다.

• 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.

• 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.

• 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.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.1
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• pp.22-30
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• 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
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• v.10 no.1
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• pp.50-55
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• 2004

Fibrodysplasia ossificans progressiva is a very rare genetic disorder, but is possible to diagnose with mass on neck or scalp in early neonate or child and accompanying characteristic congenital malformation of great toe. But because inappropriate treatment and complications from misdiagnosis may aggravate the progress of the disease, so the disorder require careful inspection for accurate diagnosis. We describe a case that was misdiagnosed properly and treated inappropriately and the natural history of the disease in adult.

• The Korean Journal of Nuclear Medicine
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• v.37 no.2
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• pp.128-134
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• 2003

섬유성 골이형성증은 비정상적인 섬유성 골조직으로 대체되며 느리게 진행하는 골병변이다. 섬유성 골이형성증과 부갑상선기능항진증은 흔하게 관찰되는 질환이나 McCune-Albright 증후군 없이 일차성 부갑상선기능항진증에 동반된 섬유성 골 이형성증은 거의 보고된바가 없다. Tc-99m MDP 골스캔이 섬유성 골이형성증의 진단에 유용하다고 알려져 있으나, Tc-99m MIBI 영상은 아직 보고된 바가 없다. 저자들은 McCune-Albright 증후군이 없이 부갑상선기능항진증에 동반된 섬유성 골 이형성증의 Tc-99m MIBI 스캔과 Tc-99m MDP 골스캔의 영상을 비교하였다. Tc-99m MDP 골스캔상 병변 부위에서 증가된 섭취 소견을 보였으며, Tc-99m MIBI 조기영상에서도 동일한 위치에서 섭취증가가 관찰되었다. 2시간 지연 Tc-99m MIBI 영상에서는 Tc-99m MIBI의 섭취가 배출되는 소견을 보였다. 섬유성 골이형성증 병변에서 Tc-99m MIBI 스캔이 유용할 것이라고 생각하며, 섬유성 골이형성증 병변에서 Tc-99m MIBI의 섭취를 증가시키는 인자에 대한 더 많은 연구가 필요할 것으로 생각한다.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.2
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• pp.74-79
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• 2014

Purpose: Fibrous dysplasia is related to the mutation of gene encoding the alpha-subunit of a signal-transducing G-protein and has variable clinical course. Operation can be performed to prevent functional disorder or structural deformity. After curettage, autologous bone graft were used to fill the defects after curettage. The aim of this study is to compare the result of autogenous cancellous bone grafting and allogenic bone grafting for fibrous dysplasia. Materials and Methods: Among the patients who visit our hospital during the period of April, 1997 to October, 2013, we selected 34 patients who diagnosed fibrous dysplasia and visited our clinic over 1 year. There were 13 males and 21 females. Average age was 26.4 (range 2 to 57) years old. Autogenous bone graft (group I) in 5 cases, Non-autogenous bone graft (group II) in 30 cases. Iliac bone is used in all cases of autogenous bone graft. There were no significant difference in age, follow-up period, preoperational laboratory finding between two groups. Radiographic image was done to evaluate the recurrence of fibrous dysplasia or secondary degeneration. Results: There were four cases in recurrence (group I: 1 case, group II: 3 cases, p=0.554). In all recurrent cases, reoperations were done using curettage and autogenous iliac bone graft. There was no re-recurrence after reoperation. One case of secondary aneurysmal bone cyst was confirmed (group II) and 1 cases of pathologic fractures had developed (group I: 0 case, group II: 1 cases, p=0.559). No malignant change occurred. Conclusion: There were no significant difference between autogenous bone graft group and non-autogenous bone graft group. Our result suggested that autogenous bone graft seems to be good method to treat fibrous dysplasia, in the case of small volume of tumor lesion or non-weight bearing portion.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.2
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• pp.106-111
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• 2000

Focal fibrocartilaginous dysplasia(FFCD) is an uncommon, benign condition associated with unilateral tibia vara among young children. FFCD has a typical plain radiographic finding, which has a concave radiolucent defect in the metadiaphyseal junction of medial aspect of the proximal tibia. The varus deformity occurs at the site of the lesion. Spontaneous remodeling and resolution of bony defect may be expected, but the corrective osteotomy may also be needed in some cases. The authors described a case of unilateral tibia vara caused by FFCD, diagnosed by excisional biopsy and treated with dome-shaped proximal tibial osteotomy and bone graft.