• 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 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.

• Journal of the Korean Orthopaedic Association
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• v.54 no.6
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• pp.519-527
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• 2019

Purpose: To evaluate the treatment result in polyostotic fibrous dysplasia classified according to the involvement of the femoral head. Materials and Methods: Twenty-three patients from March 1987 to March 2014 were reviewed retrospectively. Patients with no involvement of the physeal scar in the femoral head were classified as Type I, and those with involvement of the physeal scar were classified as Type II. A plain radiograph was used to measure the femoral neck shaft angle, articulo-trochanteric distance (ATD), and anterior bowing through the lateral view. A teleoroentgenogram of the lower limb was used to measure the leg length discrepancy and lower extremity mechanical axis. The pre- and postoperative femoral neck-shaft angle and ATD were compared to assess the degree of correction of the deformity. Results: Among a total of 46 cases (23 patients), 28 cases (23 patients) had lesions in the proximal femur. Type I were 16/28 cases (15/23 patients) and Type II were 12/28 cases (9/23 patients). The preoperative proximal femoral neck-shaft angle was 116.8° in Type I and 95.3° in Type II. The ATD was 12.08 mm in Type I and -5.54 mm in Type II. The deformity correction showed significant improvement immediately after surgery, the deformity correction was lost in Type II (neck shaft angle Type I: 133.8°-130.8°, Type II: 128.6°-116.9°, and ATD Type I: 17.66-15.72 mm, Type II: 7.44-4.16 mm). The extent of anterior bowing was 12.74° in Type I and 20.19° in Type II. The mean differences of 12 mm between the 9 patients who showed a leg length discrepancy and the lower extremity mechanical axis showed 4 cases of lateral deviation and 7 cases of medial deviation. Conclusion: In polyostotic fibrous dysplasia, when the femur head is involved, the femur neck shaft angle, ATD, and anterior bowing of the femur had more deformity, and the postoperative correction of deformity was lost, suggesting that the involvement of the femoral head was an important factor in the prognosis of the disease.

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

• Imaging Science in Dentistry
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• v.30 no.2
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• pp.117-122
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• 2000

Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs and extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most cranial bones, facial bones. The right mandibular lesion showed very expansile lesion with mottled appearance. Bone scans showed mutifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic varus deformity of right femur (shepherd's crook defomity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.