6,946 results match your criteria Fibrous Dysplasia

A Multimodal Approach to Huge Fibrous Dysplasia With Ocular Symptoms Using a Navigation System and Endoscope.

J Craniofac Surg 2022 May 12;33(3):e342-e345. Epub 2021 Oct 12.

Department of Plastic and Reconstructive Surgery, Seoul National University Bundang Hospital, Seongnam.

Background: Fibrous dysplasia (FD) is a rare sporadic benign disease, which involves from single to several bones with unilateral distribution. Recently, image-based surgical navigation systems have played a significant role in surgical process on neurological and orthopedic operations. However, because an intraoral approach can visualize the field for maxillary surgery, there are few cases using endoscopes for excision of FD. Read More

View Article and Full-Text PDF

Bony Cyst in a Ground Glass Matrix: A Rare Case Report of Craniofacial Fibrous Dysplasia with Secondary Aneurysmal Bone Cyst.

Indian J Radiol Imaging 2022 Mar 1;32(1):145-148. Epub 2022 May 1.

Department of Radiodiagnosis and Imaging, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Fibrous dysplasia has various ways of presentation including syndromic associations and secondary changes within the lesion. We present a case of a 21-year-old female with craniofacial fibrous dysplasia, presenting with proptosis and intermittent blurring of vision due to focal fibrous dysplasia involving the frontal bone with secondary aneurysmal bone formation that was provisionally diagnosed on imaging and confirmed on histopathology. This case demonstrates the typical imaging findings of fibrous dysplasia with seldom encountered secondary aneurysmal bone cyst formation and also discusses about the pathology and management of the craniofacial fibrous dysplasia. Read More

View Article and Full-Text PDF

Mandibular osteoma in a Haflinger mare: clinical and pathological features.

J Equine Vet Sci 2022 Jun 15:104048. Epub 2022 Jun 15.

D.V.M., M.Sc., University of Teramo, Faculty of Veterinary Medicine, Loc. Piano d'Accio, 64100, Teramo, Italy.

A 11-year-old, Haflinger breed mare developed a slowly growing mass, protruding on the ventral aspect of the mandible. On palpation, the lesion was pedunculated, very firm, cold and not painful. Radiographic examinations showed a radiopaque, multi-lobular, "mushroom-like" lesion, which arose from the mandibular cortex without involving the bone marrow cavity. Read More

View Article and Full-Text PDF

Skeletal Disease Acquisition in Fibrous Dysplasia: Natural History and Indicators of Lesion Progression in Children.

J Bone Miner Res 2022 Jun 13. Epub 2022 Jun 13.

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

Fibrous dysplasia (FD) is a rare mosaic disorder resulting in fractures, pain, and disability. Bone lesions appear during childhood and expand during skeletal growth. The rate at which FD lesions progress and the biochemical determinants of FD lesion formation have not been established, making it difficult to investigate and implement preventative therapies. Read More

View Article and Full-Text PDF

Risk of developing spontaneous MRONJ in fibrous dysplasia patients treated with bisphosphonates: a systematic review of the literature.

Quintessence Int 2022 Jun;53(7):616-623

Objective: The objective of this systematic review was to evaluate the risks of medication-related osteonecrosis of the jaw (MRONJ) in fibrous dysplasia (FD) and McCune-Albright syndrome (MAS) patients treated with bisphosphonates.

Method And Materials: A systematic review of the literature was performed by searching PubMed and Embase databases using MeSH terms (fibrous dysplasia of bone, "fibrous dysplasia, polyostotic," osteonecrosis, jaw, therapeutics, diphosphonates, denosumab, teriparatide, estrogens, hormones, raloxifene hydrochloride, calcitonin, cathepsin K) and non-MeSH terms (antiresorptive therapy, antiresorptives, bisphosphonate, estrogen therapy, hormone therapy, bazedoxifene, cathepsin K inhibitor). Articles were limited to human studies, in English language, in which patients were on antiresorptives for at least 1 year. Read More

View Article and Full-Text PDF

GNAS mutation analysis assists in differentiating chronic diffuse sclerosing osteomyelitis from fibrous dysplasia in the jaw.

Mod Pathol 2022 Jun 7. Epub 2022 Jun 7.

National Engineering Laboratory for Digital and Material Technology of Stomatology, Peking University School and Hospital of Stomatology, Beijing, PR China.

Chronic diffuse sclerosing osteomyelitis of the mandible (DSOM) and fibrous dysplasia (FD) are distinct lesions with overlapping clinicopathological features that complicate their diagnosis. This study aimed to evaluate the efficacy of GNAS mutation analysis in differentiating between these two conditions. DNA samples from patients with DSOM (n = 35) and FD (n = 29) were collected to analyze the presence of GNAS mutations in exons 8 and 9, the two previously reported hotspot regions, using polymerase chain reaction and direct sequencing. Read More

View Article and Full-Text PDF

Intravenous Bisphosphonate Therapy As a Rescue Analgesic in Refractory Fibrous Dysplasia.

Pain Med 2022 Jun 2. Epub 2022 Jun 2.

Assistant Professor of Anesthesiology, Mayo Clinic Hospital, Rochester, MN.

View Article and Full-Text PDF

Case Report of a Hemangioblastoma With Large Blood Vessels and Rare Vascular Anomalies: Is It Fibromuscular Dysplasia or Arteriovenous Malformation Association?

Cureus 2022 Apr 27;14(4):e24527. Epub 2022 Apr 27.

Neuropathology, Instituto Nacional de Neurología y Neurocirugia, Mexico City, MEX.

Hemangioblastoma is considered a benign neoplasm characterized by abnormal vasculature and stromal cells; several pathophysiological mechanisms have been proposed, such as genetic predisposition, hormonal factors, and arterial wall ischemia. Fibromuscular dysplasia is characterized by hyperplasia or thinning of the smooth muscle, elastic fibre destruction, fibrous tissue proliferation, and arterial wall disorganization. We present a cerebellar hemangioblastoma case not associated with Von Hippel Lindau syndrome. Read More

View Article and Full-Text PDF

Fibrous Dysplasia With Aneurysmal Bone Cyst Presenting as Sinonasal Mass.

Cureus 2022 Apr 25;14(4):e24485. Epub 2022 Apr 25.

Department of Radiodiagnosis, All India Institute of Medical Sciences, Rishikesh, Rishikesh, IND.

Craniofacial involvement in fibrous dysplasia is a rare occurrence and complications of it are even rarer. Involvement of the sinonasal region can pose a challenge in both diagnosis and management. We hereby present a case of craniofacial fibrous dysplasia complicated with secondary aneurysmal bone cyst formation in a 15-year-old male patient. Read More

View Article and Full-Text PDF

GRM1 Immunohistochemistry Distinguishes Chondromyxoid Fibroma From its Histologic Mimics.

Am J Surg Pathol 2022 Jun 1. Epub 2022 Jun 1.

Department of Pathology, Stanford University School of Medicine, Stanford, CA.

Chondromyxoid fibroma (CMF) is a rare benign bone neoplasm that manifests histologically as a lobular proliferation of stellate to spindle-shaped cells in a myxoid background, exhibiting morphologic overlap with other cartilaginous and myxoid tumors of bone. CMF is characterized by recurrent genetic rearrangements that place the glutamate receptor gene GRM1 under the regulatory control of a constitutively active promoter, leading to increased gene expression. Here, we explore the diagnostic utility of GRM1 immunohistochemistry as a surrogate marker for GRM1 rearrangement using a commercially available monoclonal antibody in a study of 230 tumors, including 30 CMF cases represented by 35 specimens. Read More

View Article and Full-Text PDF

Distal Femoral Replacement as a Salvage Treatment After Desmoplastic Fibroma Resection.

Arthroplast Today 2022 Aug 23;16:39-45. Epub 2022 May 23.

Department of Orthopaedic Surgery, University of Puerto Rico, Medical Sciences Campus, San Juan, Puerto Rico.

Desmoplastic fibroma is an extremely rare primary bone tumor that can mimic the presentation of other bone lesions. We describe the case of a middle-aged male with a mass on the left distal femur initially diagnosed as fibrous dysplasia that underwent a wide margin excision followed by a distal femoral replacement to restore anatomy and functionality. Histologic examination of the complete surgical specimen was consistent with a desmoplastic fibroma. Read More

View Article and Full-Text PDF

Presumed congenital nictitating membrane dysplasia in a Japanese white rabbit.

Vet Med Sci 2022 May 19. Epub 2022 May 19.

Central Research Laboratories, Senju Pharmaceutical Co., Ltd., Kobe, Hyogo, Japan.

Objective: The present report characterises a spontaneous nictitating membrane abnormality in a Japanese white rabbit.

Animal Studied: The animal was a male Japanese white rabbit (Oryctolagus cuniculus, Kbs:JW, 10 weeks old at the time of purchase) that had not received any treatment. A morphological abnormality of the nictitating membrane in the animal's right eye was detected. Read More

View Article and Full-Text PDF

Oral Complications in Cancer Patients-Medication-Related Osteonecrosis of the Jaw (MRONJ).

Front Oral Health 2022 26;3:866871. Epub 2022 Apr 26.

Department of Oral and Maxillofacial Diagnostic Sciences, Division of Oral Medicine, University of Florida College of Dentistry, Gainesville, FL, United States.

Medication-Related Osteonecrosis of the Jaw (MRONJ) was first reported in 2003. Despite the progress in the understanding of this oral complication in cancer patients for the past 18 years, there is still discussion about the best way to define MRONJ, prevent the complication, how to diagnose, and the options of treatment available. The initial reports associated MRONJ to bisphosphonates and denosumab, medications that work as bone-modifying agents. Read More

View Article and Full-Text PDF

Polyostotic Fibrous Dysplasia Complicated by Pathological Fracture of Right Femoral Shaft with Nonunion: A Case Report.

Front Surg 2022 12;9:879550. Epub 2022 Apr 12.

Department of Orthopedics, The First Hospital of Jilin University, Changchun, China.

Introduction: Fibrous dysplasia is a benign fibrous bone tumor that accounts for 5% to 10% of benign bone tumors. It can manifest as simple fibrous dysplasia (70%-80%), polyostotic fibrous dysplasia (20%-30%), with approximately the same incidence in men and women. We report a patient with a rare case of multiple fibrous dysplasia combined with proximal femoral shepherd deformity with pathological fracture of the femoral shaft complicated by nonunion. Read More

View Article and Full-Text PDF

[Early manifestation and progressive multicomponent current of McCune-Albright-Braitsev syndrome in a girl 9 years old: a clinical case and literature review].

Probl Endokrinol (Mosk) 2021 12 12;68(2):72-89. Epub 2021 Dec 12.

Surgut State University.

McCune-Albright-Braitsev Syndrome (MAB syndrome) is a very rare multisystem disease manifested by fibrous bone dysplasia, coffee-and-milk colored spots, hyperfunction of various endocrine glands and a number of pathologies of other body systems. We present a description of a clinical case of a severe progressive course of MAB Syndrome in a nine-year-old girl. With this diagnosis, the girl is observation of the girl began when she was 2. Read More

View Article and Full-Text PDF
December 2021

Uremic Leontiasis Ossea in a Pediatric Patient.

Cureus 2022 Mar 23;14(3):e23428. Epub 2022 Mar 23.

Radiology, Buraidah Central Hospital., Buraidah, SAU.

Uremic leontiasis ossea (ULO) is a rare disease characterized by extensive thickening of the cranium, resulting in a characteristic, lion-like facial appearance. It is considered the most severe osseous complication of renal dystrophy. Although rare, ULO can occur even in young patients, which can be catastrophic, as it can not only lead to life-threatening conditions but also multiple complications that cause severe determent to the quality of life. Read More

View Article and Full-Text PDF

Expression of Beta-Catenin, Cadherins and P-Runx2 in Fibro-Osseous Lesions of the Jaw: Tissue Microarray Study.

Biomolecules 2022 04 16;12(4). Epub 2022 Apr 16.

Department of Clinical and Experimental Medicine, University of Foggia, Viale Pinto 1, 71122 Foggia, Italy.

Fibrous dysplasia (FD) and hyperparathyroidism-jaw tumor syndrome (HPT-JT) are well-characterized benign bone fibro-osseous lesions. The intracellular mechanism leading to excessive deposition of fibrous tissue and alteration of differentiation processes leading to osteomalacia have not yet been fully clarified. Tissue Microarray (TMA)-based immunohistochemical expression of β-catenin, CK-AE1/AE3, Ki-67, cadherins and P-Runx2 were analyzed in archival samples from nine patients affected by FD and HPT-JT and in seven controls, with the aim of elucidating the contribution of these molecules (β-catenin, cadherins and P-Runx2) in the osteoblast differentiation pathway. Read More

View Article and Full-Text PDF

Benign mesenchymal odontogenic tumors of the maxillofacial bones: A report of 3 cases with 3 years follow-up.

J Oral Maxillofac Pathol 2022 Feb 28;26(Suppl 1):S46-S50. Epub 2022 Feb 28.

Department of Oral Surgery, Beirut Arab University, Beirut, Lebanon.

Benign mesenchymal odontogenic tumors are lesions derived from the mesenchymal components of the tooth-forming apparatus and are consequently found within the jawbone. Benign fibro-osseous tumors are part of this category of lesions in which normal bone is substituted, initially by fibrous tissue and within time become infiltrated by osteoid and cementoid elements. They are asymptomatic, slow-growing lesions and remain undiagnosed until swelling of the face becomes prominent and they share similar radiological characteristics. Read More

View Article and Full-Text PDF
February 2022

Reduction of giant parietooccipital fibrous dysplasia using dynamic mirror image guidance: a case report and review of the literature.

Br J Neurosurg 2022 Apr 21:1-7. Epub 2022 Apr 21.

Department of Neurological Surgery, University of Pittsburgh, Pittsburgh, PA, USA.

Background: Craniofacial fibrous dysplasia (CFD) typically occurs in the facial bones and anterior cranial vault and can produce both disfigurement and functional limitations for patients disfigurement. Treatment consists of reducing the abnormal bone. Bone contouring can become challenging when the exposure does not extend to the corresponding normal contralateral structures for comparison or when normal landmarks are not available, which may compromise the overall aesthetic outcome. Read More

View Article and Full-Text PDF

Effects of zoledronic acid therapy in fibrous dysplasia of bone: a single-center experience.

Arch Endocrinol Metab 2022 Apr 11;66(2):247-255. Epub 2022 Apr 11.

Ambulatório de Osteometabolismo, Rede SARAH de Hospitais de Reabilitação, Brasília, DF, Brasil.

Objective: Fibrous dysplasia (FD) is a rare bone disorder that can involve any part of the skeleton, leading to bone pain, deformities, and fractures. Treatment with intravenous bisphosphonates has been used with variable results. Therefore, we aimed to evaluate the effects of zoledronic acid (ZA) therapy in patients with monostotic or polyostotic FD. Read More

View Article and Full-Text PDF

Management Strategies of Fibrous Dysplasia Involving the Paranasal Sinus and the Adjacent Skull Base.

Ear Nose Throat J 2022 Apr 14:1455613221088728. Epub 2022 Apr 14.

Department of Otolaryngology-Head and Neck Surgery, 66375Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, PR China.

Objective: Current management of fibrous dysplasia (FD) involving the paranasal sinuses and the adjacent skull base is currently controversial. This study aims to present our experience in the management strategy of FD that involves the paranasal sinuses and the adjacent skull base.

Methods: Twenty three patients from 2006 to 2019 with monostotic fibrous dysplasia (MFD), polyostotic fibrous dysplasia (PFD), or McCune-Albright syndrome (MAS) involving the paranasal sinuses and the adjacent skull base were retrospectively reviewed. Read More

View Article and Full-Text PDF

Determinants of Quality of Life in Adult Patients with Chronic Non-Bacterial Osteomyelitis (CNO) of the Sternocostoclavicular Region (SCCH): A Dutch Single Center Study.

J Clin Med 2022 Mar 27;11(7). Epub 2022 Mar 27.

Division of Endocrinology, Department of Internal Medicine, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.

Sternocostoclavicular hyperostosis (SCCH), the main clinical manifestation of chronic non-bacterial osteomyelitis (CNO) in adults, is associated with various degrees of chronic pain and restricted shoulder girdle function. We evaluated the impact of CNO/SCCH on quality of life (QoL) and its determinants in 136 adult patients with this rare auto-inflammatory bone disorder using the Short Form 36, Brief Pain Inventory, Brief Illness Perception, Utrecht Coping List, and Shoulder Rating questionnaires. Data were compared with those of the general Dutch population, patients with chronic pain, fibrous dysplasia, or osteoarthritis. Read More

View Article and Full-Text PDF

Monostotic Fibrous Dysplasia in the Femur Strongly Expressing RANKL With Concomitant Osteoporotic Vertebral Compression Fracture: A Case Report.

Cancer Diagn Progn 2022 Jan-Feb;2(1):93-100. Epub 2022 Jan 3.

Department of Orthopaedic Surgery, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan.

Background/aim: This study aimed to present a rare case of fibrous dysplasia (FD) in a healthy young adult man with a concomitant osteoporotic vertebral compression fracture. FD is a benign lesion of the bone characterized by replacement of the medullary component with fibro-osseous tissue that contains abnormally arranged trabeculae of immature woven bone. Recently it has been reported that several bone tumors including FD express the receptor activator of nuclear factor-kappa B (RANK) and its ligand (RANKL). Read More

View Article and Full-Text PDF
January 2022

Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome.

Front Med (Lausanne) 2022 15;9:857079. Epub 2022 Mar 15.

Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States.

Patients diagnosed with McCune-Albright Syndrome (MAS) frequently manifest craniofacial fibrous dysplasia (FD). Craniofacial FD can impinge nerve fibers causing visual loss as well as craniofacial pain. Surgical decompression of affected nerves is performed, with variable efficacy, in an attempt to restore function or alleviate symptoms. Read More

View Article and Full-Text PDF

Case Report: The Imperfect Association Between Craniofacial Lesion Burden and Pain in Fibrous Dysplasia.

Front Neurol 2022 16;13:855157. Epub 2022 Mar 16.

Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital and Harvard Medical School, Boston, MA, United States.

Patients with fibrous dysplasia (FD) often present with craniofacial lesions that affect the trigeminal nerve system. Debilitating pain, headache, and migraine are frequently experienced by FD patients with poor prognosis, while some individuals with similar bone lesions are asymptomatic. The clinical and biological factors that contribute to the etiopathogenesis of pain in craniofacial FD are largely unknown. Read More

View Article and Full-Text PDF

Fibrous dysplasia in maxilla in a patient on hemodialysis with severe secondary hyperparathyroidism.

Korean J Intern Med 2022 05 29;37(3):695-696. Epub 2022 Mar 29.

Faculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.

View Article and Full-Text PDF

Surgical treatment of femoral deformities in polyostotic fibrous dysplasia and McCune-Albright syndrome: A literature review.

World J Orthop 2022 Mar 18;13(3):329-338. Epub 2022 Mar 18.

Department of Clinical Science and Translational Medicine, University of Rome Tor Vergata, Section of Orthopaedics and Traumatology, Tor Vergata Hospital, Rome 00133, Italy.

Background: Surgical correction of femoral deformities in polyostotic fibrous dysplasia (PFD) or McCune-Albright syndrome (MAS), such as coxa vara or shepherd's crook deformity, is a challenge.

Aim: To evaluate the treatment of patients with femoral deformities caused by PDF or MAS treated by osteotomies and stabilized with different methods, by analyzing the most relevant studies on the topic.

Methods: A literature search was performed in Medline database (PubMed). Read More

View Article and Full-Text PDF

Coexistence of craniopharyngioma and cranial fibrous dysplasia: a case series of clinicopathological study.

Yang-Hua Fan Zhi Li

Orphanet J Rare Dis 2022 03 18;17(1):126. Epub 2022 Mar 18.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No.119, the South Fourth Ring West Road, Fengtai District, Beijing, 100070, China.

Background: Craniopharyngioma (CP) and cranial fibrous dysplasia (CFD) are rare embryonic benign cranial diseases that most commonly present during childhood or adolescence. The coexistence of CP and CFD is extremely rare and has not yet been reported.

Methods: We retrospectively reviewed the data of five patients with concomitant CP and CFD treated at Beijing Tiantan Hospital from January 2003 to January 2021 and summarized their clinicopathological features, treatment modalities, and outcomes. Read More

View Article and Full-Text PDF

Craniomaxillofacial morphology in a murine model of ephrinB1 conditional deletion in osteoprogenitor cells.

Arch Oral Biol 2022 May 4;137:105389. Epub 2022 Mar 4.

Adelaide Dental School, The University of Adelaide, Adelaide, SA, Australia; South Australian Health and Medical Research Institute, Adelaide, SA, Australia; Mesenchymal Stem Cell Laboratory, School of Biomedicine, The University of Adelaide, Adelaide, SA, Australia; Cleft and Craniofacial SA, Women's and Children's Hospital, Adelaide, SA, Australia.

Objective: EFNB1 mutation causes craniofrontonasal dysplasia (CFND), a congenital syndrome associated with craniomaxillofacial anomalies characterised by coronal craniosynostosis, orbital hypertelorism, and midface dysplasia. The aim of this murine study was to investigate the effect of the EfnB1 conditional gene deletion in osteoprogenitor cells on the craniomaxillofacial skeletal morphology.

Design: The skulls of male and female mice, in which EfnB1 was deleted by Cre (a site-specific DNA recombinase) under the control of the Osterix (Osx) promoter (EfnB1), were compared to those without EfnB1 deletion (Osx:Cre control) at two ages (4 and 8 weeks; n = 6 per group). Read More

View Article and Full-Text PDF

Clinical and imaging features of 112 patients with irregular and flat bone osteosarcoma.

Quant Imaging Med Surg 2022 Mar;12(3):1988-2001

Department of Radiology, The University of Hong Kong-Shenzhen Hospital, Shenzhen, China.

Background: This study evaluated the clinical characteristics and imaging findings of 112 patients with irregular and flat bone osteosarcoma (IFBO).

Methods: The age, gender, location, tumor size, density and signal intensity, osteoid matrix, periosteal reaction, and histological subtypes were analyzed for 112 patients with IFBO.

Results: A total of 112 patients with IFBO, including 64 males and 48 females, with a mean age of 34. Read More

View Article and Full-Text PDF