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Fibrous dysplasia

Yu Isobe, Katsu Takahashi, Honoka Kiso, Kazumasa Nakao, Masayuki Ikeno, Noriaki Koyama, Manabu Sugai, Akira Shimizu, Hironori Haga, Kazuhisa Bessho
OBJECTIVE: Fibrous dysplasia (FD) is a benign bone disease characterized by fibro-osseous lesions. FD is caused by somatic mutations in the gene, guanine nucleotide-binding protein, alpha stimulating activity polypeptide 1 (GNAS), which encodes the G protein subunit, Gsα. FD manifests early in life, but the growth of lesions usually ceases in adulthood. FD lesions often exhibit somatic mutation mosaicism. In this study, the relationship between lesion growth and mutation prevalence within a lesion was investigated...
May 30, 2018: Archives of Oral Biology
Safoura Seifi, Ramin Foroghi, Arezo Rayyani, Marjan Kiani, Mohamad Reza Mohammadi
Ossifying fibroma is a benign osteogenic mesenchymal tumor that is classified in the fibro-osseous lesions. Fibro-osseous lesions contain minerals, blood vessels, and giant cells that have the same radiographic and pathologic features but have different clinical behaviors, and were replaced with the normal bone. These lesions include fibrous dysplasia, cemento-osseous dysplasia, and ossifying fibroma. Juvenile ossifying fibroma is a type of uncommon and has invasive behavior, high incidence and occurs in young men, especially in maxilla, and is different from the type of adult in age, location, and clinical behavior and microscopic view...
June 2018: Indian Journal of Surgical Oncology
Adeena Khan, Tariq Mahmud Mirza, Atiq Ur Rehman Slehria
Fibrous dysplasia is a slow growing fibro-osseous tumor, which is infrequent in craniofacial location and very rarely found in paranasal sinuses. Its occurrence is usually in children and young adolescents. The complications common in this location are related to the sinus ostia obstruction, like sinusitis or mucocele formation. Aneurysmal bone cyst formation has also been reported. We present a case of frontoethmoid fibrous dysplasia which extends to the nasal cavity and extraaxial frontal region. The peculiarity about the case is its association with the intradiploic CSF cyst formation, which to best of our knowledge has never been reported before...
June 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Sonia Mahajan, Reema Goel, Christopher Riedl, Gary A Ulaner
Mazabraud's, a syndrome consisting of fibrous dysplasia and soft tissue myxomas, is a rare disorder with less than 100 cases reported in the literature. Appearance of lesions in Mazabraud's syndrome on FDG PET/CT scan in an oncological scenario can be misinterpreted as malignancy. We present the case of a 69-year-old woman, diagnosed with metastatic colon carcinoma and suspected osseous as well as soft tissue metastases, who was later found to have concomitant Mazabraud's syndrome. This case describes the diagnostic dilemma associated with this unusual entity and importance of differentiating benign and malignant etiologies on FDG PET/CT scan...
May 31, 2018: Clinical Nuclear Medicine
Markus Martini, Anne Klausing, Nils Heim, Hans-Peter Fischer, Alexandra Sommer, Rudolf Hermann Reich
Fibrous dysplasia is a benign bone disease, presenting as monostotic or polyostotic lesions, or as part of a syndrome (McCune-Albright/Mazabraud). Its clinical course shows a variegated picture and the progression of its growth is unpredictable. In the workup of 39 fibrous dysplasia cases in the cranio-facial area, four cases presented fast growth tendencies, of which two patients with McCune-Albright syndrome showed malignant-like rapid growth. This local aggressive form is extremely rare, and the concept of this issue has not been clearly defined...
May 12, 2018: Journal of Cranio-maxillo-facial Surgery
Camila de Nazaré Alves de Oliveira Kato, Laiz Fernandes Mendes Nunes, Loliza Luiz Figueiredo Houri Chalub, Adriana Etges, Tarcília Aparecida Silva, Ricardo Alves Mesquita
PURPOSE: The aims of this study were to describe the clinical and radiologic features of 383 fibro-osseous lesions (FOLs) from an oral pathology reference service in Brazil and to compare the findings with previous studies. The hypothesis of the study was that the most common type of FOL would differ from other investigations. MATERIALS AND METHODS: We conducted a descriptive and retrospective study with review of the records of the clinical and biopsy services (1990 to 2015)...
May 10, 2018: Journal of Oral and Maxillofacial Surgery
Leilei Xu, Zheng Tian, Akbar Yunus, Xiaoshuai Wang, Jiangtao Chen, Chong Wang, Xinghua Song
Objective: To discuss the effect of three-dimensional (3D) printing individualized model and guide plate in bone tumor surgery. Methods: Between October 2015 and December 2016, 3D printing individualized model and guide plate for making preoperative surgical planning and intraoperative treatment were used in 5 patients of bone tumor. All the patients were male, with a median age of 32 years (range, 9-58 years). There were 1 case of cystic echinococcosis at left pelvis and pathological fracture of the proximal femur; 1 case of left iliac bone osteoblastoma associated with aneurysmal bone cyst; 1 case of fibrous dysplasia of the left femur (sheep horn deformity) with pathological fracture; 1 case of metastatic carcinoma of right calcaneus (tumor staging was T 2 N 0 M 0 ); and 1 case of Ewing sarcoma of left femur (tumor staging was T 2 N 0 M 0 )...
September 1, 2017: Chinese Journal of Reparative and Reconstructive Surgery
Jiasheng Lin, Rong Chen, Wei Yan, Yiyuan Zhang, Dongdong Chen
Objective: To explore the effectiveness of dynamic hip screw (DHS) and intralesional curettage via Watson-Jones approach in treatment of benign bone lesions of the proximal femur. Methods : Between March 2012 and December 2016, 20 patients (21 lesions) with benign bone tumors or tumor like conditions of proximal femurs were treated with DHS and intralesional curettage via Watson-Jones approach. Their average age was 27.8 years (range, 11-51 years), including 13 males and 7 females...
January 1, 2018: Chinese Journal of Reparative and Reconstructive Surgery
Michele Calleja, Fernanda Amary, Paul O'Donnell
Mazabraud's syndrome is a rare disorder characterised by the association of fibrous dysplasia with intramuscular myxomas. We present a 36-year-old woman with right anterior knee pain and a buttock mass. Imaging showed aggressive bone destruction within an area of fibrous dysplasia in the right femur and a mass with myxoid signal characteristics in the right adductor region. Biopsy of the femur revealed both fibrous dysplasia and a high-grade spindle cell sarcoma. Biopsy of the adductor mass confirmed a soft-tissue myxoma...
May 27, 2018: Skeletal Radiology
Kristen S Pan, Edmond J FitzGibbon, Janice S Lee, Michael T Collins, Alison M Boyce
No abstract text is available yet for this article.
June 2018: World Neurosurgery
Carmine Zoccali, Dario Attala, Barbara Rossi, Giovanni Zoccali, Virginia Ferraresi
Fibrous dysplasia (FD) is a benign fibro-osseous disease of the bone that may be solitary or multicentric. It is important to distinguish this type of lesion from low-grade osteosarcomas (LGOS) and from secondary sarcomas, because malignant transformation has rarely been reported. It is classically described as having a ground-glass appearance, endosteal scalloping, and thinning of the cortex. Cortical disruption is considered evidence of malignancy, but it can also be present in benign FD with aggressive behavior...
May 23, 2018: Skeletal Radiology
Christian Calvo-Henríquez, Gabriel Martínez-Capoccioni, Aldo Rosario-Ortiz
No abstract text is available yet for this article.
May 19, 2018: Acta Otorrinolaringológica Española
Salvatore Stefanelli, Pravin Mundada, Anne-Laure Rougemont, Vincent Lenoir, Paolo Scolozzi, Laura Merlini, Minerva Becker
Although rare, masses and mass-like lesions of developmental and genetic origin may affect the paediatric craniofacial skeleton. They represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation and disfigurement. The most common lesions include fibrous dysplasia, dermoid cysts, vascular malformations and plexiform neurofibromas. Less common lesions include torus mandibularis and torus palatinus, cherubism, nevoid basal cell carcinoma syndrome, meningoencephalocele and nasal sinus tract...
May 15, 2018: Insights Into Imaging
Mine Özşen, Ülviye Yalçinkaya, Muhammed Sadık Bilgen, Zeynep Yazici
OBJECTIVE: Fibrous dysplasia is a slowly progressing bone lesion resulting from displacement of the normal medullary bone with abnormal fibroosseous tissue. The aim of this study was to assess the similarities and differences of our cases in relation to published reports. MATERIAL AND METHOD: In this study, the archives of the Uludag University Medical Faculty Department of Pathology were screened for fibrous dysplasia cases between 2004 and 2016. RESULTS: Within the mentioned period, there were 36 cases diagnosed as fibrous dysplasia...
May 10, 2018: Türk Patoloji Dergisi
Eduardo Augusto Rosa, Aura Maria Hurtado-Puerto, Denise Pinheiro Falcão, Aline Patricia Brietzke, Luiz Eduardo De Almeida Prado Franceschi, Florlorêncio Figueiredo Cavalcanti Neto, Valdenize Tiziane, Fabiana Pirani Carneiro, Evelyn Mikaela Kogawa, Heitor Moreno, Rivadávio Fernandes Batista Amorim
The association of oral lichen planus (OLP) lesions with malignant transformation risk has remained a controversial topic and is of clinical importance. Therefore, the present study evaluated the expression levels of p16, Ki-67, budding uninhibited by benzimidazoles 3 (Bub-3) and sex-determining region Y-related high mobility group box 4 (SOX4), and their roles as precancerous biomarkers in OLP. A retrospective study was performed, in which tissue blocks of OLP, oral dysplasia (OD), cutaneous lichen planus (CLP) and oral fibrous hyperplasia (OFH) were used (n=120)...
May 2018: Experimental and Therapeutic Medicine
Bas C J Majoor, Andreas Leithner, Michiel A J van de Sande, Natasha M Appelman-Dijkstra, Neveen A T Hamdy, P D Sander Dijkstra
BACKGROUND: Fibrous dysplasia of the proximal femur presents with heterogeneous clinical manifestations dictating different surgical approaches. However, to date there are no clear recommendations to guide the choice of surgical approach and no general guidelines for the optimal orthopedic management of these lesions. The objective of this study was to evaluate treatment outcomes of angled blade plates and intramedullary nails, using as outcome indicators revision-free survival, pain, function and femoral neck-shaft-angle...
May 2, 2018: Orphanet Journal of Rare Diseases
Ali Fuat Cicek, Melih Kilinc, Mukerrem Safali, Omer Gunhan
BACKGROUND: Fibrous dysplasia (FD) is a maturation defect characterized by immature woven bones and stroma. However, especially in craniofacial bones, lamellation can be seen and this is associated with the maturation. AIM: To show maturation in FD and discuss the factors that may affect the maturation. MATERIALS AND METHODS: Ninety-five FD cases were divided into three subgroups according to the lamellation percentage as Groups 1, 2 and 3 (low, moderate and high lamellation, respectively)...
April 20, 2018: Histology and Histopathology
Daniele Tessaris, Alison M Boyce, Margaret Zacharin, Patrizia Matarazzo, Roberto Lala, Luisa De Sanctis, Michael T Collins
CONTEXT: In fibrous dysplasia (BFD), normal bone and bone marrow are replaced by fibro-osseous tissue, leading to fracture, deformity and pain. BFD may be isolated, or in association with cutaneous hyperpigmentation and/or hyperfunctioning endocrinopathies, termed McCune-Albright syndrome (MAS). GH hypersecretion has been described in 10%-20% of MAS-BFD patients. Aim of the study was to determine the impact of GH-insulin like growth factor 1 (IGF1) axis hyperactivity on MAS-BFD morbidities and the efficacy of GH excess therapy...
April 19, 2018: Clinical Endocrinology
Jason A Berglund, Sri Harsha Tella, Kaitlyn F Tuthill, Lauren Kim, Lori C Guthrie, Scott M Paul, Robert Stanton, Michael T Collins, Alison M Boyce
Scoliosis is a complication of fibrous dysplasia/McCune-Albright syndrome (FD/MAS); however, risk factors and long-term outcomes are unknown. Bisphosphonates are commonly used; however, it is unknown whether their use decrease the risk of progressive scoliosis. Clinical data from the National Institutes of Health (NIH) cohort study was reviewed. Cobb angles were measured, and variables associated with scoliosis progression were identified. Of 138 subjects with available radiographs, 84 (61%) had scoliosis, including 55 (65%) classified as mild (Cobb angle >10 to ≤30 degrees), 11 (13%) as moderate (>30 to ≤45 degrees), and 18 (22%) as severe (>45 degrees)...
April 18, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
Soyoun Um, Hui Young Kim, Byoung-Moo Seo
OBJECTIVES: The purpose of this study was to evaluate the effects of BMP-2 on bone-grafting procedures for treatment of fibrous dysplasia based on in vitro and in vivo experiments. SUBJECTS AND METHODS: Proliferation of stem cells was determined by colony-forming assay, CCK-8 assay, and BrdU staining. OCT4 and NANOG expression was analyzed by flow cytometry and immunocytochemistry. Osteogenic differentiation was assessed by measuring ALP activity, alizarin red S staining, and in vivo transplantation...
April 18, 2018: Oral Diseases
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