2,912 results match your criteria: "Fibrous Dysplasia Imaging"

Craniofacial fibrous dysplasia: A review of current literature.

Bone

December 2024

Department of Otorhinolaryngology - Head and Neck Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, POBox 263, FI-00029 HUS Helsinki, Finland. Electronic address:

Article Synopsis
  • *The systematic literature review from 2010-2023 revealed that management strategies for CFD must be personalized, with asymptomatic patients monitored and those with symptoms possibly needing medical or surgical intervention.
  • *The review emphasizes the importance of a multidisciplinary approach to CFD treatment, as well as the need for ongoing monitoring due to potential complications and the risk of malignant transformation.
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Dysregulated FGF23 production is a demonstrated cause of hypophosphatemia and osteomalacia. Diseases associated with these conditions include phosphaturic mesenchymal tumor (PMT) causing tumor induced osteomalacia, various forms of rickets, and fibrous dysplasia (FD). Coexistence of 2 conditions that can increase FGF23 concentrations is rare.

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This study aimed to assess the diagnostic value of the airway post-processing technique of multi-slice spiral CT(MSCT) in Accessory cardiac bronchus(ACB)and to improve the understanding of this disease. The original MSCT axial images and various post-processing reconstructed images of 9 ACB cases were retrospectively analyzed.Airway post-processing techniques, including multi-planar reformation(MPR), minimum intensity projection (MinIP), volume rendering technique(VRT), CT virtual endoscopy(CTVE) and tissue transition projection (TTP), were employed.

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Introduction And Importance: Mazabraud's syndrome is a rare condition, describing the presence of fibrous bone dysplasia and intramuscular myxomas, with an incidence of 1:1,000,000. The aim of this article is to provide a review of the clinical presentation of Mazabraud's syndrome, including indications for surgical treatment, and follow-up strategies.

Case Presentation: A 46-year-old woman presented with a 3-month history of a painless mass in the right gluteal region, she referred a rapid increase in the mass's volume in the 3 weeks prior to consultation.

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Article Synopsis
  • Fibrous dysplasia (FD) is a nonheritable genetic condition that causes abnormal bone growth, leading to benign lesions that can sometimes become malignant, as exemplified in a case of a 61-year-old man with painful craniofacial FD.
  • The patient presented with a rapidly growing mass in the skull, which MRI revealed as an extensive dysplastic mass causing significant pressure on the brain; he underwent surgery to remove the mass.
  • Post-surgery pathology confirmed it as a high-grade undifferentiated pleomorphic sarcoma, leading to further treatment with chemotherapy, though the effectiveness of such treatment remains debated.
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Key Clinical Message: Cemento-osseous dysplasia (COD) belongs to a group of fibrous osseous disorders that can masquerade as periapical inflammatory conditions in the jawbones. We present a rare case of COD occurring in a patient who also had periapical periodontitis concurrently. When faced with a patient exhibiting no symptoms, diagnosis may be challenging.

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Fibrous dysplasia (FD) is a rare skeletal disorder characterized by the replacement of normal bone with fibrous connective tissue, leading to abnormal bone formation. This case report details the successful treatment of a 61-year-old woman with FD at the craniovertebral junction (CVJ). The patient, who had a history of intracranial meningioma and had already been diagnosed with FD, experienced worsening gait disturbance and muscle weakness following a fall.

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[Imaging manifestations and misdiagnosis of liposclerosing myxofibroma fumor].

Zhongguo Gu Shang

October 2024

Department of Radiology, Huzhou Central Hospital, Huzhou 313000, Zhejiang, China.

Objective: To focus on the imaging features of liposclerosing myxofibrous tumor (LSMFT) and diagnostic challenges to enhance clinical recognition and differential diagnosis accuracy.

Methods: Retrospective analysis was conducted on the imaging and pathological data from 21 cases diagnosed with LSMFT between January 2014 and November 2022, including 14 males and 7 females (aged from 21 to 73 years; the course of disease ranged from 4 to 48 months). Patient demographics, clinical presentations, and imaging modalities including X-ray, CT, and MRI were reviewed.

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Article Synopsis
  • This study examines the link between fibrous dysplasia (FD), a rare bone disorder, and the presence of cystic changes in vestibular schwannomas (VSs), specifically focusing on how FD may influence the development of cystic VSs.* -
  • Out of 1,255 patients studied, 11.3% had cystic VSs, with findings indicating that female patients, those with concurrent FD, and larger VS volumes showed higher percentages of cystic formation.* -
  • The results suggest that concurrent FD and larger VS volumes are significant risk factors for cystic VSs, highlighting the need for further research into the mechanisms behind this relationship.*
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Article Synopsis
  • Fibrous dysplasia of the bone is a condition linked to a GNAS mutation that can affect craniofacial bones and impact nearby neurovascular structures; this case presents the first known instance of this condition affecting the jugular foramen, causing blockage of critical brain sinuses.
  • A 33-year-old man experiencing dizziness and occasional swallowing difficulties underwent imaging that revealed a mass in the left jugular foramen, leading to a successful surgical removal of the tumor while preserving his lower cranial nerves.
  • This case underscores the need for awareness of fibrous dysplasia in jugular fossa assessments and emphasizes the importance of surgical expertise in ensuring patient outcomes and preserving nerve function.
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Article Synopsis
  • Osteofibrous dysplasia (OFD) is a rare, benign bone lesion primarily found in the tibia, characterized by the growth of fibrous tissue and variable bone involvement, making diagnosis challenging.
  • A case study highlights a young patient with OFD-related pain and introduces a new vacuum-assisted bone harvester technique for more effective biopsy and curettage.
  • This innovative method improves the ability for surgeons to obtain tissue samples and perform necessary procedures in cases of OFD.
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Erratum to: McCune-Albright syndrome with acromegaly: A case report with characteristic radiographic features of fibrous dysplasia.

Imaging Sci Dent

September 2024

Department of Oral and Maxillofacial Radiology and Wonkwang Dental Research Institute, College of Dentistry, Wonkwang University, Iksan, Korea.

[This corrects the article on p. 421 in vol. 52, PMID: 36605861.

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Development and validation of a robotic system for milling individualized jawbone cavities in oral and maxillofacial surgery.

J Dent

November 2024

State Key Laboratory of Oral & Maxillofacial Reconstruction and Regeneration, National Clinical Research Center for Oral Diseases, Shaanxi Key Laboratory of Stomatology, Digital Center, School of Stomatology, The Fourth Military Medical University, No.145 Changle West Road, Xincheng District, Xi'an, Shaanxi 710032, China. Electronic address:

Article Synopsis
  • The study focused on creating and testing a robotic system designed for precise and minimally invasive jawbone milling, aimed at improving oral and maxillofacial surgery outcomes.
  • The robotic system includes a UR5E arm and advanced software that generates tailored cutting paths based on 3D models and surgical needs.
  • Results showed that the robotic approach outperformed static guides in terms of accuracy, with better overall precision in milling surgeries, indicating its potential for clinical use.
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Article Synopsis
  • Fibrous dysplasia is a rare condition where normal bone is replaced by fibrous tissue, leading to skeletal lesions, as observed in an 18-year-old male with lesions in multiple skull bones.
  • The patient underwent surgery involving the removal of the tumor, followed by a complex skull base reconstruction using various grafts and a custom 3D-printed model to replicate the skull structure.
  • The case highlights the effectiveness of personalized reconstruction techniques using 3D printing and affordable materials in treating skull base defects after fibrous dysplasia surgery.
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Objectives: The objective of this study was to identify treatment indications for symptomatic and asymptomatic florid cemento-osseous dysplasia (FCOD) in adult patients and explore relationships between clinical variables and radiographic findings (PROSPERO # CRD42023411228).

Study Design: A systematic review was conducted by independent investigators using databases: PubMed, ProQuest, Embase, Web of Science, Dentistry and Oral Sciences Database (DOSS), and TRIP to identify studies on FCOD treatment options in adults. Inclusion criteria for this systematic review included: originally in English; open-access; published between 2001 and 2021.

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Article Synopsis
  • Burosumab is a new medication designed for treating disorders related to hereditary fibroblast growth factor 23 (FGF23), specifically hypophosphatemia.
  • An 11-year-old girl with McCune-Albright syndrome suffered from low phosphate levels and multiple bone lesions due to excess FGF23, but her condition improved significantly with burosumab treatment.
  • This case marks the first instance of burosumab improving bone lesions in a patient with McCune-Albright syndrome, highlighting its potential therapeutic benefits.
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Monostotic fibrous dysplasia of jaw bones: a case series.

BMC Oral Health

September 2024

Department of Oral & Maxillofacial Pathology and Microbiology, Sharad Pawar Dental College & Hospital, Datta Meghe Institute of Higher Education and Research, Sawangi (Meghe), Wardha, 442004, Maharashtra, India.

Article Synopsis
  • Fibrous dysplasia (FD) is a benign condition affecting bone development and can complicate the diagnosis of jaw lesions due to the need for various clinical assessments.
  • A study analyzed five cases of monostotic FD in maxillofacial bones over a decade, finding equal gender representation and primarily involving the maxilla.
  • The condition can cause facial disfigurement, making early detection important, and genetic analysis may enhance understanding of its occurrence in specific populations.
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Fibroblast Activation Protein Is Expressed by Altered Osteoprogenitors and Associated to Disease Burden in Fibrous Dysplasia.

Cells

August 2024

Skeletal Disorders and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA.

Fibrous dysplasia (FD) is a mosaic skeletal disorder involving the development of benign, expansile fibro-osseous lesions during childhood that cause deformity, fractures, pain, and disability. There are no well-established treatments for FD. Fibroblast activation protein (FAPα) is a serine protease expressed in pathological fibrotic tissues that has promising clinical applications as a biomarker and local pro-drug activator in several pathological conditions.

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Beware of the Iceberg Phenomenon: A Case Report of Chest Wall Fibrous Dysplasia.

Diagnostics (Basel)

August 2024

Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands.

Article Synopsis
  • Thoracic fibrous dysplasia (FD) is a benign tumor in the chest wall that originates from bone marrow, representing 30-50% of benign bone neoplasms in this area.
  • A case is presented where a massive intrathoracic polyostotic FD from the rib was diagnosed after it caused significant symptoms, despite its extrathoracic portion remaining stable for decades.
  • The tumor was surgically removed using a hemi-clamshell approach, and the importance of regular follow-up imaging post-diagnosis is emphasized to ensure timely intervention and prevent complications.
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Undifferentiated sarcoma arising from fibrous dysplasia in a young adult.

Orthopadie (Heidelb)

September 2024

Department of Oncology, Santa Fe Fundación Santa Fe de Bogotá, Bogotá, Colombia, Calle 119 No. 7-03.

Fibrous dysplasia (FD) is a skeletal disorder characterized by the replacement of normal bone by fibrous tissue. Malignant transformation of FD is extremely rare and has been reported in both monostotic and polyostotic forms of FD. The most frequently reported malignant transformation is osteosarcoma.

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PLCG2 variants in cherubism.

J Allergy Clin Immunol

December 2024

Department of Pediatrics, Division of Allergy, Immunology, and Rheumatology, Columbia University, New York, NY. Electronic address:

Background: Cherubism is most commonly caused by rare heterozygous gain-of-function (GOF) missense variants in SH3BP2, which appear to signal through phospholipase C gamma 2 (PLCG2) to cause excessive osteoclast activity leading to expansile lesions in facial bones in childhood. GOF variants in PLCG2 lead to autoinflammatory PLCG2-associated antibody deficiency and immune dysregulation (autoinflammatory PLAID, or PLAID-GOF), characterized by variably penetrant autoinflammatory, autoimmune, infectious, and atopic manifestations. Cherubism has not been reported in PLAID to date.

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Background: Most primary bone tumors are often found in the bone around the knee joint. However, the detection of primary bone tumors on radiographs can be challenging for the inexperienced or junior radiologist. This study aimed to develop a deep learning (DL) model for the detection of primary bone tumors around the knee joint on radiographs.

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