6,785 results match your criteria Fibrous Dysplasia


Adamantinoma of Pelvis: A Rare Tumor at an Uncommon Site, with Diagnostic Implications.

Indian J Orthop 2021 May 18;55(Suppl 1):261-266. Epub 2020 Jul 18.

Department of Surgical Pathology, Rajiv Gandhi Cancer Institute, Rohini, Delhi India.

Adamantinoma is a low-grade malignant bone tumor with metastatic potential in the range of 15-20%, commonly affecting mid-diaphyseal tibial region and jaw. Numerous cases of adamantinoma affecting the appendicular skeleton have been reported but only three in the pelvis till date. We present the case of a 24-year-old male, who was initially reported as fibrous dysplasia for a lesion in pelvis. Read More

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An Atypical Bilateral Presentation of Fibrous Dysplasia (FD) in the Mandible: Clinical, imaging and therapeutic characterization.

Int J Surg Case Rep 2021 May 29;84:106049. Epub 2021 May 29.

Department of Oral and Maxillofacial Surgery, School of Dentistry, Central University of Ecuador, Quito, Ecuador.

Introduction And Importance: Fibrous Dysplasia (FD) is a benign fibro-osseous lesion, characterized by replacement with fibrous connective tissue instead of normal bone. The best treatment option for the condition has not yet been established, although several therapeutic approaches have been reported. The present case report describes the clinical, imaging and therapeutic aspects of an atypical bilateral presentation of FD in the mandible. Read More

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The importance of casting an eye beyond the dental arch: A case report.

J Orthod 2021 Jun 8:14653125211021871. Epub 2021 Jun 8.

Department of Paediatric Dentistry, Glasgow Dental Hospital, Glasgow, UK.

Study models are frequently used in orthodontics and are a key part of both initial assessment and treatment planning. They are an important part of comprehensive orthodontic records; furthermore, study models are an essential diagnostic aid that can be studied in detail without the patient being present. We present a case of a 14-year-old boy with an abnormal palatal swelling detected by his specialist orthodontist after review of study models taken at the initial appointment. Read More

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Exome sequencing reveals variants in known and novel candidate genes for severe sperm motility disorders.

Hum Reprod 2021 Jun 5. Epub 2021 Jun 5.

Biosciences Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK.

Study Question: What are the causative genetic variants in patients with male infertility due to severe sperm motility disorders?

Summary Answer: We identified high confidence disease-causing variants in multiple genes previously associated with severe sperm motility disorders in 10 out of 21 patients (48%) and variants in novel candidate genes in seven additional patients (33%).

What Is Known Already: Severe sperm motility disorders are a form of male infertility characterised by immotile sperm often in combination with a spectrum of structural abnormalities of the sperm flagellum that do not affect viability. Currently, depending on the clinical sub-categorisation, up to 50% of causality in patients with severe sperm motility disorders can be explained by pathogenic variants in at least 22 genes. Read More

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Safety of therapy with and withdrawal from denosumab in fibrous dysplasia and McCune-Albright syndrome: an observational study.

J Bone Miner Res 2021 Jun 2. Epub 2021 Jun 2.

Department of Internal Medicine, Division of Endocrinology, Center for Bone Quality, Leiden University Medical Center, Leiden, The Netherlands.

Denosumab (Dmab) treatment can benefit patients with fibrous dysplasia/McCune-Albright syndrome (FD/MAS) by suppressing the receptor activator of nuclear factor κB ligand (RANKL)-mediated increased bone resorption. However, limited data of two pediatric cases indicate that a rebound phenomenon may occur after withdrawal. Therefore we studied the safety of Dmab discontinuation in FD/MAS. Read More

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Imaging of Fibrous Dysplasia Protuberans, an Extremely Rare Exophytic Variant of Fibrous Dysplasia.

Diagnostics (Basel) 2021 May 31;11(6). Epub 2021 May 31.

Department of Radiology, Nuclear Medicine Division, Naval Medical Center San Diego, 34800 Bob Wilson Drive, San Diego, CA 92134, USA.

This paper details the case report of a 26-year-old man who presented with a growing right-sided skull mass evaluated with ultrasound, non-contrast CT, contrast-enhanced MRI and Tc-MDP whole body bone scan with SPECT/CT. These studies suggested a broad differential diagnosis favoring benign osseous lesions. Given a more recent increase in the rate of growth, headache and large size, the lesion was excised via craniotomy followed by cranioplasty. Read More

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Strategies for managing the destruction of calcar femorale.

BMC Musculoskelet Disord 2021 May 19;22(1):460. Epub 2021 May 19.

Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, 610072, Sichuan Province, China.

Background: The calcar femorale was identified long ago. However, our current understanding of the calcar is insufficient, and its related concepts are sometimes confused. The calcar femoral is an important anatomical structure of the proximal femur, and its function can be overlooked. Read More

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Monostotic Fibrous Dysplasia of the Mandible in a 9-Year-Old Male Patient Treated with a Conservative Surgical Treatment: A Case Report and 15-Year Follow-Up.

Case Rep Dent 2021 3;2021:9963478. Epub 2021 May 3.

Department of Oral Medicine and Maxillofacial Radiology, Faculty of Dental Medicine, Lebanese University, Lebanon.

Fibrous dysplasia is a developmental disorder of the bone that originates from a genetic defect disturbing the osteogenesis leading to the replacement of normal bone with the excess proliferation of fibrous tissue. It can be associated with hyperpigmentation of the skin and endocrine disorders. Fibrous dysplasia can manifest in a monostotic form affecting one bone or in a polyostotic form involving several bones. Read More

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Biopsied non-dental plaque-induced gingival diseases in a Chinese population: a single-institute retrospective study.

BMC Oral Health 2021 05 17;21(1):265. Epub 2021 May 17.

Department of Oral Pathology, National Clinical Research Center for Oral Diseases, Peking University School and Hospital of Stomatology, Beijing, China.

Background: While inflammatory diseases such as gingivitis and periodontitis induced by dental plaque biofilms constitute the majority of gingival lesions, gingiva can also be affected by a variety of diseases with aetiologies different from bacterial biofilms. The aim of this study was to retrospectively analyze the frequency and distribution of non-dental plaque-induced gingival diseases (NDPIGDs) in the Chinese population in a single institute.

Methods: A total of 6859 samples of biopsied gingival diseases during the period 2000-2019 were obtained from the Department of Pathology, Peking University Hospital of Stomatology. Read More

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Burosumab treatment for fibrous dysplasia.

Bone 2021 May 11;150:116004. Epub 2021 May 11.

Metabolic Bone Disorders Unit, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, USA. Electronic address:

Background: Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) is a rare mosaic disorder of Gα activation. Fibroblast Growth Factor 23 (FGF23)-mediated hypophosphatemia is a feature of FD/MAS that has been associated with poor skeletal outcomes. Standard therapy includes oral phosphorus and vitamin D analogs; however, treatment is limited by potential adverse renal and gastrointestinal effects. Read More

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Augmented reality navigation method for recontouring surgery of craniofacial fibrous dysplasia.

Sci Rep 2021 May 11;11(1):10043. Epub 2021 May 11.

Department of Oral and Craniomaxillofacial Surgery, Shanghai 9Th People's Hospital, Shanghai Jiaotong University College of Medicine, Shanghai, China.

The objective of this study is to introduce the application of augmented reality (AR) navigation system developed by the authors in recontouring surgery of craniofacial fibrous dysplasia. Five consecutive patients with craniofacial fibrous dysplasia were enrolled. Through three-dimensional (3D) simulation, a virtual plan was designed to reconstruct the normal anatomical contour of the deformed region. Read More

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A Minimally Invasive Novel Technique Using a New Device to Treat Proximal Femoral Cystic Lesions.

J Orthop Case Rep 2020 May-Jun;10(3):23-26

Department of Orthopaedic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Introduction: Bone grafts are widely performed to fill bone cavities and accelerate bone ingrowth. Advances in minimally invasive surgical techniques to treat bone tumors provide the impetus for minimally invasive bone graft procedures. A special funnel with an inner plunger has been manufactured specifically for the delivery of bone graft substitutes. Read More

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DNA methylation-based profiling of bone and soft tissue tumours: a validation study of the 'DKFZ Sarcoma Classifier'.

J Pathol Clin Res 2021 Jul 5;7(4):350-360. Epub 2021 May 5.

Research Department of Pathology, University College London, UCL Cancer Institute, London, UK.

Diagnosing bone and soft tissue neoplasms remains challenging because of the large number of subtypes, many of which lack diagnostic biomarkers. DNA methylation profiles have proven to be a reliable basis for the classification of brain tumours and, following this success, a DNA methylation-based sarcoma classification tool from the Deutsches Krebsforschungszentrum (DKFZ) in Heidelberg has been developed. In this study, we assessed the performance of their classifier on DNA methylation profiles of an independent data set of 986 bone and soft tissue tumours and controls. Read More

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Squamous Cell Carcinoma of the Lung in McCune-Albright Syndrome.

Cureus 2021 Mar 28;13(3):e14159. Epub 2021 Mar 28.

Internal Medicine, Detroit Medical Center (DMC) Harper Hospital, Detroit, USA.

McCune Albright Syndrome (MAS) is caused by a mutation in the GNAS gene that results in multiple endocrinopathies such as Cushing syndrome, acromegaly, hyperthyroidism, and precocious puberty. Despite the presence of pleiotropy coupled with a GNAS gene mutation, malignancy is a rare occurrence in MAS. There is minimal literature showcasing squamous cell carcinoma (SCC) of the lung in patients with MAS. Read More

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Jaffe Lichtenstein Type of Polyostotic Skeletal Fibrous Dysplasia With the Involvement of Cranial Bones.

J Craniofac Surg 2021 Apr 29. Epub 2021 Apr 29.

Department of Maxillofacial Radiology, Faculty of Dentistry, Yeni Yuzyil University, Istanbul Department of Orthopedics and Traumatology, Van Training and Research Hospital, Van Department of Orthopedics and Traumatology, Tepecik Training and Research Hospital Department of Maxillofacial Radiology, Faculty of Dentistry, Ege University, Izmir, Turkey.

Abstract: Fibrous dysplasia (FD), a benign fibro-osseous bone tumor commonly involving the craniofacial region, presents a wide spectrum of radiographic patterns. When FD lesions are suspected, various imaging modalities and histopathologic analyses may be required to reach a firm diagnosis. Here, the authors report the case of a 10-year-old male patient presenting with polyostotic type of FD with follow-up data for the next 9 years. Read More

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Cemento-Ossifying Fibroma in Maxillofacial Region: A Series of 16 Cases.

J Maxillofac Oral Surg 2021 Jun 8;20(2):240-245. Epub 2019 Nov 8.

Department of Oral and Maxillofacial Surgery, Sri Guru Ram Das Institute of Dental Sciences and Research, Mall Mandi, GT Road, Amritsar, 143001 India.

Introduction: Cemento-ossifying fibroma is considered as a benign osseous tumour, closely related to other lesions such as fibrous dysplasia and cementifying periapical dysplasia. These lesions occur in the second to the fourth decade of life. It is a bony tumour of maxilla and mandible of possibly odontogenic origin with aggressive behaviour and high tendency for recurrence. Read More

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Locking compression plate fixation of femoral intertrochanteric fractures in patients with preexisting proximal femoral deformity: a retrospective study.

J Orthop Surg Res 2021 Apr 29;16(1):285. Epub 2021 Apr 29.

Department of Orthopedics Trauma, Xi'an Honghui Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an, 710054, P.R. China.

Background: To investigate the clinical efficacy of locking compression plate fixation for the treatment of femoral intertrochanteric fractures in patients with preexisting proximal femoral deformity.

Methods: A retrospective analysis was conducted on 37 patients with femoral intertrochanteric fractures combined with preexisting proximal femoral deformity between January 2013 and July 2019. The patients included 24 males and 13 females aged from 23 to 69 years old, with an average age of 47. Read More

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Regression of fibrous dysplasia in response to denosumab therapy: A report of two cases.

Bone Rep 2021 Jun 9;14:101058. Epub 2021 Apr 9.

Department of Internal Medicine, Division of Endocrinology, Center for Bone Quality, LUMC, Albinusdreef 2, 2333ZA Leiden, the Netherlands.

We present two patients with fibrous dysplasia who showed a decrease in lesional size and activity after denosumab therapy. Both patients also experienced a reduction in pain and bone turnover markers, which had not been accomplished during previous bisphosphonate therapy. These cases highlight the potential of denosumab to decrease lesional size in fibrous dysplasia. Read More

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[The role of piezosurgery in the treatment of a rare case of superinfected cemento-osseous dysplasia caused by actinomyces: a case report].

Pan Afr Med J 2021 2;38:106. Epub 2021 Feb 2.

Service de Médecine et Chirurgie Buccales, Clinique Universitaire de Médecine et Chirurgie Dentaire, Monastir, Tunisie.

Cemento-osseous dysplasia is a benign fibro-osseous lesion affecting the alveolar bone. It is classified into three forms: periapical, focal or florid dysplasia. It is often asymptomatic and fortuitously discovered during a routine radiological examination. Read More

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Fibrous Dysplasia of the Spine-A Case Involving the Polyostotic Form Isolated to the Thoracolumbar Spine.

Int J Spine Surg 2021 Feb 29;14(s4):S46-S51. Epub 2020 Dec 29.

Department of Orthopaedics and Traumatology, Faculty of Medicine, Padjadjaran University, Bandung, Indonesia.

Fibrous dysplasia of the spine in a polyostotic form is very rare, with fewer than 36 cases discussed in the literature and there is no such case in Indonesia that has been reported. The aim of this report is to present a case from Indonesia of polyostotic fibrous dysplasia isolated in the spine. We report a case of a 38-year-old Sundanese man with a 1-year history of progressive back pain and weakness of both lower extremities. Read More

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February 2021

Fibrous Dysplasia Mimicking Skeletal Metastasis on 68Ga-FAPI PET Imaging.

Clin Nucl Med 2021 Apr 20. Epub 2021 Apr 20.

From the Department of Nuclear Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology; and Hubei Province Key Laboratory of Molecular Imaging, Wuhan, China.

Abstract: 68Ga-labeled fibroblast activation protein inhibitor (68Ga-FAPI) PET imaging has been introduced for detecting many primary and metastatic tumors. However, false-positive uptakes have been reported in some benign lesions. Here, we presented a 68Ga-FAPI-avid lesion in the left temporal bone in a 41-year-old man with a history of signet ring cell gastric adenocarcinoma. Read More

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The importance of clinical and radiological findings for the definitive histopathologic diagnosis of benign fibro-osseous lesions of the jaws: Study of 276 cases.

J Stomatol Oral Maxillofac Surg 2021 Apr 18. Epub 2021 Apr 18.

Istanbul University, Institute of Oncology, Department of Tumour Pathology, Istanbul, Turkey.

Purpose: Benign fibro-osseous lesions (BFOLs) are heterogeneous intraosseous disease processes sharing overlapping histopathologic features and demonstrate a wide range of biological behavior. The aim of this study is to highlight the importance of clinical and radiological findings for the definitive histopathologic diagnosis of benign fibro-osseous lesions of the jaws.

Materials And Methods: This is a cross-sectional retrospective study evaluating the three main groups of BFOLs: Cemento-osseous dysplasia, ossifying fibromas, and fibrous dysplasia. Read More

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Immediate allograft reconstruction of the infraorbital nerve following resection of polyostotic fibrous dysplasia lesion.

Case Reports Plast Surg Hand Surg 2021 Feb 5;8(1):12-17. Epub 2021 Feb 5.

Department of Oral Maxillofacial Surgery and Pathology, School of Dentistry - University of Mississippi Medical Center, Jackson, MS, USA.

Processed nerve allografts (PNA) have increasingly been used as alternative to autogenous nerve grafts to repair nerve injuries in oral-maxillofacial surgeries. This case report describes an immediate PNA reconstruction of infraorbital nerve injury sustained during the ablation of a large expansile polyostotic fibrous dysplasia centered in the left maxilla. Read More

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February 2021

Stigma and psychological distress among pediatric participants in the FD/MAS Alliance Patient Registry.

Authors:
Amanda Konradi

BMC Pediatr 2021 04 14;21(1):173. Epub 2021 Apr 14.

Department of Sociology, Loyola University Maryland, 4501 North Charles St., Baltimore, MD, 20210, USA.

Background: Stigma, both enacted and internalized, is part of the illness experience of many chronic conditions / diseases and has been found to increase psychological distress, lower self-esteem, and impact social engagement lowering quality of life (QOL). Stigma among pediatric patients is of particular concern due to its potential impact on identity formation. Using patient data from the online FD/MAS Alliance Patient Registry (FDMASAPR), this study seeks to 1) determine levels of enacted and self-stigma in a pediatric population of fibrous dysplasia (FD) / McCune Albright syndrome (MAS) patients and 2) to explore the relationship between stigma and anxiety and depression. Read More

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Malignant transformation of monostotic fibrous dysplasia into angiosarcoma in the proximal tibia.

Indian J Pathol Microbiol 2021 Apr-Jun;64(2):394-397

Department of Radiology, Faculty of Medicine, Uludag University, Bursa, Turkey.

The majority of bone angiosarcomas are primary tumors while secondary angiosarcomas arise after radiation therapy or bone infarctus. This article presents a case of malignant transformation of monostotic fibrous dysplasia into angiosarcoma. An 80-year-old female presented with pain on right cruris. Read More

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Excision of fibrous band and application of tension band plating in focal fibrocartilaginous dysplasia: A case report and literature review.

Acta Orthop Traumatol Turc 2021 Mar;55(2):184-188

Department of Orthopaedics and Traumatology, Hacettepe University, School of Medicine, Ankara, Turkey.

Focal fibrocartilaginous dysplasia (FFCD) is a rare disease that can cause angular deformities of long bones. The common pathologic finding is a thick fibrotic band extending from epiphysis to metaphysis on one side of the bone. The tethering effect of the fibrotic band around the growth plate is thought to be the main etiology for the development and progression of the deformity. Read More

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Assessing quality of life in pediatric fibrous dysplasia and McCune Albright syndrome: PEDS-QL and HADS data from the Fibrous Dysplasia Foundation Patient Registry.

Authors:
Amanda Konradi

J Patient Rep Outcomes 2021 Apr 12;5(1):34. Epub 2021 Apr 12.

Department of Sociology, Loyola University Maryland, 4501 Charles St., Baltimore, MD, 21210, USA.

Purpose: The International FD/MAS Consortium recently encouraged using the Pediatric Quality of Life Inventory (PEDS-QL) and the Hospital Anxiety and Depression scales (HADS) in clinical care. This study examines scores on these measures among pediatric fibrous dysplasia and McCune Albright (FD/MAS) patients to initiate consideration of their use in clinical treatment.

Methods: This is a retrospective analysis of pediatric data from 39 minors, ages 2-17, entered in the Fibrous Dysplasia Foundation Patient Registry from July 2016 to December 2018. Read More

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Benign Osseous Tumors and Tumor-Like Conditions.

Semin Ultrasound CT MR 2021 Apr 29;42(2):150-163. Epub 2020 Aug 29.

Department of Musculoskeletal Imaging, The University of Texas MD Anderson Cancer Center, Houston, TX.

We will provide an imaging-focused discussion of 3 benign bone tumors that do not fit in the categories of cartilaginous tumors or osteoid tumors. We have chosen giant cell tumor of bone, unicameral bone cyst, and fibrous dysplasia. All 3 of these entities are common enough that one does not have to be a musculoskeletal radiologist in a cancer hospital to encounter them occasionally, but none of them should be seen frequently. Read More

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Fibrous Dysplasia at Unusual Anatomic Sites: A Series of 86 Cases With Emphasis on Histologic Patterns.

Int J Surg Pathol 2021 Apr 1:1066896921997141. Epub 2021 Apr 1.

195112Mayo Clinic, Rochester, MN, USA.

. Fibrous dysplasia (FD) is a benign fibro-osseous neoplasm that most commonly arises in the ribs, femur, and craniofacial bones. We analyzed features of FD arising in the spine/short tubular/small bones of the hands/feet (STSBHF), specifically assessing for pattern of bone formation (conventional, complex/anastomosing, psammomatoid/cementum like), myxoid change, and presence of osteoclast-type giant cells. Read More

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Denosumab reduces lesional Fluoride skeletal burden on Na[18F]F PET-CT in patients with Fibrous Dysplasia/McCune-Albright syndrome.

J Clin Endocrinol Metab 2021 Mar 31. Epub 2021 Mar 31.

Center for Bone Quality, dept. of Internal Medicine, division of Endocrinology, Leiden University Medical Center (LUMC), Leiden, the Netherlands.

Context: The correlation between Fibrous Dysplasia/McCune-Albright syndrome (FD/MAS) skeletal disease burden on Na[18F]F-PET-CT and serum bone turnover markers (BTMs) was recently described. The effect of treatment on lesional fluoride burden in FD/MAS is unknown.

Objective: To investigate treatment response measurements in FD/MAS patients who underwent Na[18F]F-PET-CT and treatment with antiresorptives. Read More

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