• Imaging Science in Dentistry
• /
• v.52 no.4
• /
• pp.421-427
• /
• 2022

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by a clinical triad of polyostotic fibrous dysplasia (FD), skin pigmentation, and hyperfunctioning endocrinopathies. A 42-year-old man visited our medical hospital for the treatment of intermittent headaches and was diagnosed with MAS with acromegaly. This patient showed various clinical features of MAS, including pituitary adenoma, polyostotic FD, and hypogonadotropic hypogonadism. The FD lesions showed characteristic radiographic features, such as widespread, sclerotic bony lesions in the cranial bones, mixed radiolucent-radiopaque multilocular lesions in the mandible, and radiolucent lesions in the axial and appendicular skeleton. Over the years, the patient had been hospitalized multiple times due to accidental bony fractures associated with the fragile bony state of FD. This report presents a retrospective description of a case of MAS, with a review of the relevant literature.

• Journal of Korean Dental Science
• /
• v.16 no.2
• /
• pp.211-217
• /
• 2023

Fibrous dysplasia (FD) accompanying osteomyelitis (OM) has been reported to result in recurrent, refractory pain and swelling. Although radical resection of the lesions has been suggested, effective surgical treatment strategies have not yet been established due to the limited number of studies on this pathological condition. In this report, we present the conservative surgical management of FD accompanying OM in two patients who exhibited recurrent signs and symptoms. The present report suggests that OM occurring in patients with FD can be successfully managed with conservative surgical treatment and following removal of the odontogenic origin although bone defect in which inflammatory fibro-osseous lesions is removed through decompression surgery may exhibit regeneration of dysplastic bone. In addition, for the prevention of OM in patients with FD, careful clinical examination and thorough management of dental-related pathologies are necessary with regular follow-up examinations to screen for the possibility of malignant changes.

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

• The Journal of the Korean dental association
• /
• v.38 no.6 s.373
• /
• pp.498-509
• /
• 2000

• Archives of Plastic Surgery
• /
• v.34 no.3
• /
• pp.388-391
• /
• 2007

Purpose: Malignant fibrous histiocytoma (MFH) is mainly a soft tissue sarcoma containing fibroblast-like cells and histiocytic cells. MFH in bone accounts for 5% of all malignant bone tumors. MFH of the maxilla is extremely rare and difficult to diagnose due to its scarcity. Treatment mainstay is a complete surgical excision. Radiation therapy is also available when surgery alone is incomplete. Prognosis is not clear but can be devastating. Authors report one case of MFH developed in the maxilla. Methods: A 24-year-old man firstly diagnosed as fibrous dysplasia based on CT findings. Considering facial contour, partial excision was done. But pathology report confirmed malignant fibrous histiocytoma and secondary wide excision was done including zygoma and grossly all affected area. After surgery, radiation therapy was continued. Results: There are no evidence of tumor recurrence after clinical and radiological treatment. Conclusion: MFH of maxilla is very rare and this can leads to misdiagnosis in many clinicians. Surgeon should differentiate this disease from fibrous dysplasia and pathology and MRI are accurate methods for diagnosis of MFH.

• Archives of Craniofacial Surgery
• /
• v.11 no.2
• /
• pp.91-94
• /
• 2010

Purpose: For the best possible aesthetic reconstruction after craniofacial bone tumor resection, pasteurization has been adopted to devitalize neoplastic cells while maintaining osteoinductive properties and mechanical strength. This case report aims to demonstrate a long-term follow-up result of a monostotic fibrous dysplasia in frontal bone which was reconstructed by pasteurized tumoral autograft in situ. Methods: A 14-year-old girl presented with a hard, nontender, slowly growing mass of 6-year duration on her left supraorbital area. CT showed $5{\times}4{\times}3cm$ sized well defined bony mass confined to frontal bone with heterogeneous density. Tumor was excised completely through bicoronal approach and reimplanted to its original site after pasteurization at $60^{\circ}C$ for 30 minutes. The pathologic examination confirmed fibrous dysplasia. Results: She revisited our clinic 5 years later after suffering some assault on her face. On CT examination, pasteurized tumoral autograft was incorporated to host bone except the fractured upper orbital rim without any evidence of recurrence. She has been satisfied with the result. Conclusion: Pasteurization offers a simple, reliable, cosmetic, economic, and durable reconstruction method for craniofacial skeletal tumor. It has advantages of both biologic incorporation ability and mechanical strength without risk of recurrence. So, it should be considered as one of the primary options in benign as well as resectable malignant tumors of craniofacial skeleton.

• The Journal of the Korean bone and joint tumor society
• /
• v.8 no.1
• /
• pp.27-31
• /
• 2002

Clinical symptoms of fibrous dysplasia in proximal femur include pain, limping, and leglength discrepancy. Occasionally varus deformity, which may range from mild coxa vara to a marked shepherd's crook deformity was developed. Surgical intervention generally is considered advisable in the presence of persistent pain unresponsive to conservative treatment or significant or progressive deformity. Depending on the lesion size, lesion site, and deformity, several treatment methods have been used. This is a report on one case of bilateral shepherd's crook deformity in fibrous dysplasia, which was treated with corrective osteotomy by Huckstep nail.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.38 no.1
• /
• pp.29-37
• /
• 2012

Introduction: Fibrous-osseous lesions of the jaws are difficult to diagnose precisely until excised biopsy results are found, so they might be confused with malignant lesions. This clinical study focused on the diagnostic aids of lesions that demonstrate different clinical, radiologic, and histological findings. Materials and Methods: A total of 16 patients with benign fibrous-osseous lesions on the jaws (6 fibrous dysplasias, 6 ossifying fibromas, 3 cemental dysplasias, and one osteoblastoma) were reviewed. Nine patients with malignant fibrous-osseous lesions (8 osteosarcomas and one Ewing's sarcoma) were also retrospectively reviewed. Results: Osteosarcoma patients complained of facial swelling and tooth mobility. The radiographic findings showed the irregular resorption of cortical bone and periosteal reactions. Histological features included cellular pleomorphism and atypical mitosis. An Ewing's sarcoma patient complained of tooth mobility and facial swelling. Onion-skin appearance and irregular expansile marginal bony radiolucency were seen in the radiography. Fibrous dysplasia patients complained of facial swelling and asymmetry. The radiographic features were mostly ground-glass radiopacity. Histological findings showed a bony trabeculae pattern surrounded by fibrous ground substances. Ossifying fibroma patients complained of buccal swelling and jaw pains, showing expanded cortical radiolucent lesions with a radiopaque margin. Histological findings were revealed as cellular fibrous stroma with immature woven bones. In cemental dysplasia, most of their lesions were found in a routine dental exam. Well-circumscribed radiopaque lesions were observed in the radiography, and cementum-like ossicles with fibrous stroma were seen in the microscopy. An osteoblastoma patient complained of jaw pain and facial swelling. Radiographic findings were mottled, dense radiopacity with osteolytic margin. Trabeculae of the osteoid with a vascular network and numerous osteoblasts with woven bone were predominantly found in the microscopy. Conclusion: Our study showed similar results as other studies. We suggest the clinical parameters of diagnosis and treatment for malignant and benign fibrous-osseous lesions of the jaws.

• Imaging Science in Dentistry
• /
• v.40 no.1
• /
• pp.53-58
• /
• 2010

Common radiographic appearances of ossifying fibroma (OF) are well demarcated margin, radiolucent or mixed lesion. Lesions for the radiographic differential diagnosis with OF include fibrous dysplasia, focal cemento-osseous dysplasia. Other confusing lesions might be the mixed lesions such as calcifying odontogenic cyst, adenomatoid odontogenic tumor, calcifying epithelial odontogenic tumor, and benign cementoblastoma. We reported three cases of OF in posterior mandible. These cases showed a little distinguished radiographic features of OF and diagnosed from a combination of clinical, radiographic, and histopathologic information. We need to further refine radiographic and histopathological features of OF and other confusing lesions with literatures review because some cases of these lesions are not easily differentiated radiographically and histopathologically.

• Imaging Science in Dentistry
• /
• v.46 no.4
• /
• pp.259-265
• /
• 2016

Fibrous dysplasia (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Mainly, FD is found in children, and by adulthood it usually becomes quiescent. Our case showed FD of more than 14-year duration in the left maxilla. Our evaluation was that growth ceased in adulthood and had achieved the static stage. Because FD cases in elderly patients are rarely reported, we hereby present a monostotic FD case in a 65-year-old female. We presented sequential radiographic images and scintigraphic images of this case, and combined them with a literature review that emphasized the progression of the disease.