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Fibrous dysplasia

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https://www.readbyqxmd.com/read/28211081/h3f3a-mutation-in-giant-cell-tumour-of-the-bone-is-detected-by-immunohistochemistry-using-a-monoclonal-antibody-against-the-g34w-mutated-site-of-the-histone-h3-3-variant
#1
Julian Lüke, Alexandra von Baer, Jordan Schreiber, Christoph Lübbehüsen, Thomas Breining, Kevin Mellert, Ralf Marienfeld, Markus Schultheiss, Peter Möller, Thomas Fe Barth
AIMS: Giant cell tumour of bone (GCTB) is a neoplasm predominantly of long bones characterised by the H3F3A mutation G34W. Conventional diagnostic is challenged by the tumour's giant cell-rich morphology, which overlaps with other giant cell containing lesions of the bone. Recently, a monoclonal antibody specific for the H3F3A mutation has been generated. Our aim was to test this antibody on a cohort of giant cell containing lesions. METHODS AND RESULTS: We used the antibody for analysis of 22 H3F3A-mutated GCTB, including two patients with recurrences; for comparison we analysed a cohort of 36 H3F3A-wild-type giant cell-rich lesions of the bone and soft tissue, containing one brown tumour, six aneurysmal bone cysts, six chondroblastomas, five non-ossifying-fibromas, two fibrous dysplasias, nine tenosynovial giant cell tumours, one giant cell-rich sarcoma and six osteosarcomas...
February 17, 2017: Histopathology
https://www.readbyqxmd.com/read/28208959/secondary-aneurysmal-bone-cyst-of-the-scapula-treated-by-ct-guided-percutaneous-polidocanol-injection-a-case-report
#2
Rahul Mohan, Gopakumar Thanuvan Sreekumaran
Aneurysmal Bone Cyst (ABC) is a rare benign tumour, usually affecting early age group and at the metaphysis of long bones. Scapular ABC's are even more rare, especially successfully treated cases. Standard treatment methods like curettage have high recurrence rates hence, adjuvant therapy may be required to avoid recurrence. Polidocanol sclerotherapy is becoming popular because of its safety and efficacy and is being tried successfully for both active as well as aggressive primary ABC of size 3-5cm. We used the same principle in a large secondary ABC of the scapula, which to the best of our knowledge has not been yet reported...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28207472/intradiploic-epithelial-inclusion-cyst-of-the-cranium-mimicking-fibrous-dysplasia
#3
Max Mandelbaum, Jerry W Chao, Gary F Rogers, Cheng-Ying Ho, Suresh N Magge
Intradiploic inclusion cysts are exceedingly rare in the pediatric population. The authors present a 16-year-old male patient who presented with a large growing calvarial mass with a preoperative diagnosis of fibrous dysplasia based on radiologic imaging. Craniectomy followed by autogenous reconstruction was performed. Histopathological examination revealed a relatively small inclusion cyst of the intradiploic space, surrounded by reactive bone. This patient demonstrates a highly unusual presentation of a rare entity, and the authors discuss the diagnosis and management of intradiploic inclusion cysts...
February 15, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28194495/florid-cemento-osseous-dysplasia-review-of-an-uncommon-fibro-osseous-lesion-of-the-jaw-with-important-clinical-implications
#4
REVIEW
Sarah Fenerty, Wei Shaw, Rahul Verma, Ali B Syed, Riya Kuklani, Jie Yang, Sayed Ali
Florid cemento-osseous dysplasia (FCOD) is a rare, benign, multifocal fibro-osseous dysplastic process affecting tooth-bearing areas of the jaw, characterized by replacement of normal trabecular bone with osseous tissue and dense acellular cementum in a fibrous stroma. It is one clinicopathologic variant in a spectrum of related non-neoplastic fibro-osseous lesions known as cemento-osseous dysplasias (CODs), thought to arise from elements of the periodontal ligament. Diagnosis primarily relies upon radiographic and clinical findings; unnecessary biopsy should be avoided, as inoculation with oral pathogens may precipitate chronic infection in these hypovascular lesions...
February 13, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28186860/traumatic-rib-injury-patterns-imaging-pitfalls-complications-and-treatment
#5
Brett S Talbot, Christopher P Gange, Apeksha Chaturvedi, Nina Klionsky, Susan K Hobbs, Abhishek Chaturvedi
The ribs are frequently affected by blunt or penetrating injury to the thorax. In the emergency department setting, it is vital for the interpreting radiologist to not only identify the presence of rib injuries but also alert the clinician about organ-specific injury, specific traumatic patterns, and acute rib trauma complications that require emergent attention. Rib injuries can be separated into specific morphologic fracture patterns that include stress, buckle, nondisplaced, displaced, segmental, and pathologic fractures...
February 10, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28180039/endoscopic-endonasal-optic-nerve-decompression-for-fibrous-dysplasia
#6
Timothy R DeKlotz, S Tonya Stefko, Juan C Fernandez-Miranda, Paul A Gardner, Carl H Snyderman, Eric W Wang
Objective To evaluate visual outcomes and potential complications for optic nerve decompression using an endoscopic endonasal approach (EEA) for fibrous dysplasia. Design Retrospective chart review of patients with fibrous dysplasia causing extrinsic compression of the canalicular segment of the optic nerve that underwent an endoscopic endonasal optic nerve decompression at the University of Pittsburgh Medical Center from 2010 to 2013. Main Outcome Measures The primary outcome measure assessed was best-corrected visual acuity (BCVA) with secondary outcomes, including visual field testing, color vision, and complications associated with the intervention...
February 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28178102/giant-hemifacial-fibrous-dysplasia-functional-treatment-and-place-of-pamidronate
#7
Jean-Thomas Bachelet, Leonor Costa Mendes, Clarence Delafond, Thomas Barba, Arnaud Gleizal, Christian Paulus
The authors report the case of a 9-year-old child with severe form of hemifacial fibrous dysplasia. The authors review the pathology of this treatment modality through the case description and detail the place of pamidronate in the treatment of fibrous dysplasia.
February 7, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28118187/might-trauma-be-a-triggering-factor-for-craniofacial-fibrous-dysplasia
#8
Mehmet Onur Yüksel, Mehmet Sabri Gürbüz, Hilmi Onder Okay, Mehmet Esref Kabalar
Fibrous dysplasia (FD) is a rare, benign disease of unclear etiology where normal bone is replaced with abnormal fibrous and weak osseous tissue. Any bone of the skeleton might be involved but skull is one of the most commonly affected sites. Fibrous dysplasia is known to be caused by a genetic mutation leading to inappropriate proliferation and differentiation of osteoblastic cells. However; it is not known whether any triggering factor exists which might contribute to this genetic mutation. The authors postulated that trauma might be a triggering factor for this disease...
January 23, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28072620/fibrous-dysplasia-mimicking-malignancy-on-68ga-dotatate-pet-ct
#9
Georgios Z Papadakis, Corina Millo, Samira M Sadowski, Apostolos H Karantanas, Ulas Bagci, Nicholas J Patronas
Fibrous dysplasia of the bone is a developmental benign skeletal disorder characterized by replacement of normal bone and normal bone marrow with abnormal fibro-osseous tissue. We report on a case of a biopsy-proven fibrous dysplasia lesion in the left temporal bone, with intensely increased activity (SUVmax, 56.7) on Ga-DOTATATE PET/CT. The presented data indicate cell surface overexpression of somatostatin receptors by fibrous dysplastic cells and highlight the need of cautious management of Ga-DOTATATE-avid bone lesions, which could mimic malignancy especially in patients with history of neuroendocrine tumors...
March 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28057011/functional-and-radiological-outcomes-of-a-minimally-invasive-surgical-approach-to-monostotic-fibrous-dysplasia
#10
Mamer S Rosario, Katsuhiro Hayashi, Norio Yamamoto, Akihiko Takeuchi, Shinji Miwa, Yuta Taniguchi, Hiroyuki Tsuchiya
BACKGROUND: Reports showing high recurrence rates for intralesional curettage and bone grafting have made the current treatment principle for fibrous dysplasia controversial. This study aimed to report the postoperative clinical outcomes from three minimally invasive surgical strategies we use for monostotic fibrous dysplasia (MFD). PATIENTS AND METHODS: Twelve patients with MFD presenting with no pathologic fracture or deformity and treated with one of three surgical strategies-plain open biopsy, plain alpha-tricalcium phosphate (ATP) reconstruction, and prophylactic bridge plating-were included...
January 5, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28054178/hemosuccus-pancreaticus-caused-by-a-mucinous-cystic-neoplasm-of-the-pancreas
#11
Yuri Matsumoto, Hiroshi Miyamoto, Akira Fukuya, Fumika Nakamura, Takahiro Goji, Shinji Kitamura, Tetsuo Kimura, Koichi Okamoto, Masahiro Sogabe, Naoki Muguruma, Mitsuo Shimada, Yoshimi Bando, Tetsuji Takayama
Hemosuccus pancreaticus is a gastrointestinal hemorrhage through the main pancreatic duct. Here, we report a rare case of hemosuccus pancreaticus due to a mucinous cystic neoplasm of the pancreas. A 62-year-old woman who had been followed for a branch duct intraductal papillary mucinous neoplasm visited our emergency room due to severe abdominal pain and bloody discharge. Computed tomography revealed that the pancreatic cyst increased the tension of the wall and a high-density area indicative of bleeding into the cyst was observed...
January 4, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/28050548/monostotic-fibrous-dysplasia-of-the-metacarpal-a-case-report
#12
Kátia Tôrres Batista, Hugo José de Araújo, Ulises Prieto Y Schwartzman
Fibrous dysplasia is a bone disease characterized by abnormal differentiation of fibrous tissue in the bones; it is often asymptomatic. It may affect one bone (monostotic) or several bones (polyostotic). The monostotic form primarily affects the ribs, but hardly ever affects the hand. It is important to make the differential diagnosis with malignant bone tumors. This article describes the treatment and outcome of a rare case of a patient admitted with a history of tumor growth in the right hand, diagnosed as fibrous dysplasia of the right second metacarpal...
November 2016: Revista Brasileira de Ortopedia
https://www.readbyqxmd.com/read/28050513/an-atypical-presentation-of-multiple-central-osteomas-mimicking-craniofacial-fibrous-dysplasia-a-pictorial-essay
#13
Rashmi Kewal Agarwal, Amit A Mhapuskar, Manjula Hebbale, Meenal Tepan, Ayushee
Osteoma is benign neoplasm with slow growth characterized by deposition of compact lamellar cortical or cancellous bone creating a tumour mass. It is still unclear whether osteomas are benign neoplasms or hamartomas. They have typical clinical presentations and are easily diagnosed with the help of radiographs. We present a rare case of non-syndromic multiple osteomas in the craniofacial region which are typically restricted to the midline and presents radiographically as craniofacial fibrous dysplasia causing a diagnostic dilemma...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28035304/fibrous-dysplasia-of-the-maxilla-in-an-elderly-female-case-report-on-a-14-year-quiescent-phase
#14
Byung-Do Lee, Wan Lee, Yong-Chan Park, Myoung-Hyoun Kim, Moon-Ki Choi, Jung-Hoon Yoon
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...
December 2016: Imaging Science in Dentistry
https://www.readbyqxmd.com/read/28031510/use-of-a-magnetic-bone-nail-for-lengthening-of-the-femur-and-tibia
#15
Ozgur Karakoyun, Sami Sokucu, Mehmet Fatih Erol, Metin Kucukkaya, Yavuz Selim Kabukçuoğlu
PURPOSE: To report our experience with the PRECICE nail for limb lengthening in 23 patients. METHODS: Records of 15 female and 8 male patients aged 14 to 38 (mean, 23.6) years who underwent lengthening of the tibia (n=6) or femur (n=21) using the PRECICE nail were reviewed. The reasons for lengthening included trauma (n=7), hemihypertrophy (n=2), focal femoral deficiency (n=2), Ellis-van Creveld syndrome (n=1), hip septic arthritis sequelae (n=1), hereditary multiple exostosis (n=1), club foot sequela (n=1), congenital tibial pseudoarthrosis (n=1), fibrous dysplasia (n=1), idiopathic limb length discrepancy (n=7), and cosmetic (n=1)...
December 2016: Journal of Orthopaedic Surgery
https://www.readbyqxmd.com/read/28007843/clinical-characteristics-and-management-of-growth-hormone-excess-in-patients-with-mccune-albright-syndrome
#16
Yong Yao, Yang Liu, Linjie Wang, Kan Deng, Hongbo Yang, Lin Lu, Feng Feng, Bing Xing, Hui You, Zimeng Jin, Renzhi Wang, Hui Pan, Shi Chen, Huijuan Zhu
OBJECTIVE: McCune-Albright syndrome (MAS) is a sporadic, postzygotic disease presenting with fibrous dysplasia, cafe-au-lait spots and multiple endocrinopathies. Growth hormone (GH) excess is an uncommon but potentially severe complication of MAS. This study aims to describe the clinical manifestations of GH excess in the context of MAS and analyze the responses of these patients to treatments. DESIGN: Retrospective clinical study. METHODS: Clinical data from 52 MAS patients were analyzed...
March 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28005795/orbitocranial-fibrous-dysplasia-outcome-of-radical-resection-and-immediate-reconstruction-with-titanium-mesh-and-pericranial-flap
#17
Khalid Nasser Fadle, Ahmed Gaber Hassanein, Abdin K Kasim
INTRODUCTION: Fibrous dysplasia (FD) is a non-neoplastic developmental fibro-osseous disease. It represents 2.5% of all bone tumors and 5% to 7% of the benign bone tumors. Orbitocranial region is involved in about 20% of the patients. The main presentations are craniofacial deformity and headache. Loss of vision is the most devastating result of this disease. There is no medical treatment to cure or prevent FD. Radiation therapy is contraindicated. Surgery for the orbitocranial FD is often challenging because of the proximity of neurovascular and ocular structures...
November 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27998739/can-differences-in-vascularity-serve-as-a-diagnostic-aid-in-fibro-osseous-lesions-of-the-jaws
#18
Tom Shmuly, Dror M Allon, Marilena Vered, Gavriel Chaushu, Benjamin Shlomi, Ilana Kaplan
PURPOSE: Different lesions in the fibro-osseous group share microscopic features; thus, establishing a definitive diagnosis based on microscopic features alone can be a challenge. There is a need for additional microscopic tools to aid in differentiating these lesions. This study compared parameters related to vascularity among 3 lesions in the fibro-osseous group: fibrous dysplasia (FD), central ossifying fibroma (COF), and cemento-osseous dysplasia (COD). MATERIALS AND METHODS: This study was a cross-sectional analysis of biopsied lesions retrieved from 3 medical centers over a 14-year period...
November 24, 2016: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/27975011/-well-digging-subcraniotomy-strategy-with-navigation-for-optic-nerve-decompression-in-frontoorbital-fibrous-dysplasia-preliminary-experience
#19
Yunhe Lu, Junyi Yang, Yingzhi Wu, Sida Pan, Jiawen Lu, Xiongzheng Mu
BACKGROUND: During the past decades, surgical intervention has been the primary treatment modality for frontoorbital fibrous dysplasia involving optic nerve. However, controversy has surrounded the role of optic nerve decompression in a number of ways. Herein, we describe 3 patients with frontoorbital fibrous dysplasia involving optic nerve, who underwent a "well digging" subcraniotomy strategy with navigation for intraorbital unit optic nerve decompression. METHODS: From 2013 to 2015, 3 patients with frontoorbital fibrous dysplasia were investigated in a retrospective manner...
November 2016: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/27922965/prevalence-of-different-forms-and-involved-bones-of-craniofacial-fibrous-dysplasia
#20
Liya Yang, Huanhuan Wu, Jianjian Lu, Li Teng
BACKGROUND: The purposes of this study were to analyze the prevalence of various craniofacial bones involved in patients with craniofacial fibrous dysplasia (CFD) and to demonstrate the most common form and bone involvement in patients with CFD for surgeons. METHODS: To address the research purpose, the authors designed and performed a systematic review with meta-analysis. A comprehensive electronic search without date was performed in August 2013. Data extracted from the previously published literature were analyzed with STATA 11...
January 2017: Journal of Craniofacial Surgery
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