• 제목/요약/키워드: Fibrous Dysplasia

검색결과 125건 처리시간 0.026초

McCune-Albright syndrome with acromegaly: A case report with characteristic radiographic features of fibrous dysplasia

  • Han-Gyeol, Yeom;Byung-Do, Lee
    • Imaging Science in Dentistry
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    • 제52권4호
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    • pp.421-427
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    • 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.

Conservative Surgical Management of Fibrous Dysplasia with Osteomyelitis

  • Sung Bin Youn;Jeong Joon Han;Hoon Myoung
    • Journal of Korean Dental Science
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    • 제16권2호
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    • pp.211-217
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    • 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.

광범위하게 진행된 다발성 섬유성이형성증 (A Case of Extensive Polyostotic Fibrous Dysplasia)

  • 이병도;황의환;이상래
    • Imaging Science in Dentistry
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    • 제30권2호
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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.

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상악골에 발생한 악성 섬유성 조직구종 1례 (Malignant Fibrous Histiocytoma of the Maxilla: A Case Report)

  • 송승용;김용욱;박병윤;탁관철
    • Archives of Plastic Surgery
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    • 제34권3호
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    • pp.388-391
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    • 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.

저온 열처리 자가 종양골이식을 이용한 이마뼈의 단골성 섬유성이형성증의 재건 (Pasteurized Tumoral Autograft for the Reconstruction of Monostotic Fibrous Dysplasia in Frontal Bone)

  • 이의태
    • 대한두개안면성형외과학회지
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    • 제11권2호
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    • pp.91-94
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    • 2010
  • 저온 열처리법은 두개안면골 종양의 절제 후 재건에 있어 간단하고, 믿을만하며, 모양이 우수하고, 경제적이면서 또한 오랫동안 유지가 가능한 방법이다. 이 방법은 종양 재발의 걱정없이 생물학적으로 융합이 가능함과 동시에 기계적인 강도가 높은 장점을 가지고 있다. 따라서 두개안면골의 양성종양뿐만 아니라 절제가 가능한 악성종양의 경우에도 재건 시 우선적으로 고려해볼 만한 방법으로 사료된다.

섬유성 골이형성증에서 Huckstep 정을 이용한 Shepherd's Crook 변형의 치료 - 증례 보고 - (Treatment of Shepherd's Crook Deformity with Huckstep's nail in Fibrous Dysplasia - A Case Report -)

  • 황건성;김태승;김병훈;이종민
    • 대한골관절종양학회지
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    • 제8권1호
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    • pp.27-31
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    • 2002
  • 대퇴 근위부에 발생한 섬유성 골이형성증의 임상 증상으로는 동통, 파행, 하지 부등등이 유발되며 때로는 shepherd's crook 변형과 같은 심한 변형을 초래한다. 수술적 치료는 보존적 치료에 반응하지 않는 지속적인 동통이 있거나, 변형이 진행하는 경우에 시행하며 병변의 크기나 침범 부위, 변형 정도에 따라 다양한 치료가 시행되고 있다. 이에 저자들은 섬유성 골이 형성증으로 중증의 양측성 shepherd's crook 변형을 일으킨 환자 1례에 대해 전자부 교정 절골술과 Huckstep정을 이용하여 치료한 결과를 보고하고자 한다.

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Clinical study of benign and malignant fibrous-osseous lesions of the jaws

  • Lee, Ju-Min;Song, Won-Wook;Lee, Jae-Yeoul;Hwang, Dae-Seok;Kim, Yong-Deok;Shin, Sang-Hun;Chung, In-Kyo;Kim, Uk-Kyu
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • 제38권1호
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    • pp.29-37
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    • 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.

하악골 후방부위에서 다양한 방사선학적 소견을 보이는 골화성섬유종의 증례보고 (Cases report of ossifying fibroma showing various radiographic appearances in posterior mandible)

  • 이병도;오승환;손현진
    • Imaging Science in Dentistry
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    • 제40권1호
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    • pp.53-58
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    • 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.

Fibrous dysplasia of the maxilla in an elderly female: Case report on a 14-year quiescent phase

  • Lee, Byung-Do;Lee, Wan;Park, Yong-Chan;Kim, Myoung-Hyoun;Choi, Moon-Ki;Yoon, Jung-Hoon
    • Imaging Science in Dentistry
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    • 제46권4호
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    • pp.259-265
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    • 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.