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fibro osseous dysplasia

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...
September 9, 2016: Journal of Craniofacial Surgery
Sai Kiran Chennoju, Ramaswamy Pachigolla, Vanya Mahitha Govada, Satish Alapati, Smitha Balla
Bone dysplasias comprise of a condition where the normal bone is replaced with fibrous tissue. Periapical Cemento-Osseous Dysplasia (PCOD) is a benign fibro-osseous condition where bone tissue is supplanted with fibrous tissue and cementum-like material. This condition affects mostly mandibular anterior region and rarely occurs in the maxilla. PCOD is seen above 30 years of age and has slight female predilection. Generally the teeth related to such lesions appear to be vital and are usually asymptomatic. These lesions are mostly seen during routine radiographic examination whose presentation may vary from complete radiolucency to dense radiopacity...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
Paolo Cariati, Fernando Monsalve Iglesias, José Fernández Solís, Alfredo Valencia Laseca, Ildefonso Martinez Lara
Cherubism is a rare disorder with autosomal dominant inheritance. It is classified as a benign fibro-osseous lesions and may involve either facial bone. Its typical dentofacial deformities are caused by mutations in the SH3BP2 gene. The protein encoded by SH3BP2 had a significant role in the regulation of osteoblasts and osteoclasts. Accordingly with the radiological findings, differential diagnoses includes fibrous dysplasia, giant cell granuloma, osteosarcoma, juvenile ossifying fibroma, fibrous osteoma, odontogenic cyst and hyperparathyroidism...
July 11, 2016: Reumatología Clinica
Sean E Pack, Ahmad A Al Share, Faisal A Quereshy, Dale A Baur
Fibrous dysplasia (FD) is a benign fibro-osseous lesion that typically behaves as a painless, slowly expanding tumor. On rare occasion, FD will undergo malignant transformation. When sarcomatous change occurs, osteosarcoma is the typical variant, followed by chondrosarcoma and fibrosarcoma. The incidence of malignant change varies from 1 to 4% depending on whether the disease is mono-ostotic or polyostotic and syndromic (McCune-Albright or Jaffe-Lichtenstein syndrome). Despite the low incidence of malignant change, the potential lethality of this disease behooves treating surgeons to be keenly aware of the signs and symptoms indicative of malignancy...
June 21, 2016: Journal of Oral and Maxillofacial Surgery
Nirmal Raj Gopinathan, Mahesh Prakash, Balaji Saibaba, Ashim Das
Osteofibrous dysplasia or ossifying fibroma is an uncommon benign fibro-osseous lesion of childhood, commonly described in the maxilla and the mandible. Among long bones, it usually presents in the tibia as a painless swelling or anterior bowing. Ossifying fibroma of clavicle has never been reported in English literature, to the best of our knowledge. Here, we would like to present an unusual case of osteofibrous dysplasia of clavicle clinically mimicking chronic osteomyelitis.
April 2016: Indian Journal of Radiology & Imaging
G N Mainville, D P Turgeon, A Kauzman
Benign fibro-osseous lesions of the maxillofacial skeleton constitute a heterogeneous group of disorders that includes developmental, reactive (dysplastic) and neoplastic lesions. Although their classification has been reviewed multiple times in the past, the most common benign fibro-osseous lesions are fibrous dysplasia, osseous dysplasia and ossifying fibroma. For the dental clinician, the challenges involve diagnosis and treatment (or lack thereof). A careful correlation of all clinical, radiologic and microscopic features is essential to establish a proper diagnosis and a clear treatment plan...
July 7, 2016: Oral Diseases
Yu-Hsi Liu, Kuo-Ping Chang
Fibrous dysplasia is a slowly progressive benign fibro-osseous disease, rarely occurring in temporal bones. In these cases, most bony lesions developed from the bony part of the external auditory canals, causing otalgia, hearing impairment, otorrhea, and ear hygiene blockade and probably leading to secondary cholesteatoma. We presented the medical history of a 24-year-old woman with temporal monostotic fibrous dysplasia with secondary cholesteatoma. The initial presentation was unilateral conductive hearing loss...
April 2016: Journal of International Advanced Otology
Kandhari Vk, Bava Ss, Desai Mm, Wade Rn
INTRODUCTION: Fibrous dysplasia is a rare benign disorder of the skeletal system characterized by fibro osseous proliferation with intervening areas of normal or immature bone in the intramedullary region. It can either be a monostotic (involves one bone) or a polyostotic (involves more than one bone) presentation and usually occurs equally in males and females. Deformities like scoliosis and shepherd's crook deformity are frequently encountered in the polyostotic form. We report a rare managed case of bilateral non-union of the pathological fracture of femur neck with shepherd's crook deformity of the proximal femur in a case of polyostotic fibrous dysplasia...
July 2015: Journal of Orthopaedic Case Reports
Chelsey Deel, Lewis Hassell
Liposclerosing myxofibrous tumor is a benign fibro-osseous lesion, with distinct radiographic and clinical features and diverse histologic patterns. This lesion occurs in the fourth decade of life, with equal male and female incidence. Most lesions are discovered incidentally, but patients can present with bone pain or fracture. Liposclerosing myxofibrous tumor exhibits a very strong predilection for the proximal femur with characteristic radiographic findings, often providing an initial clue to diagnosis. Microscopically, this tumor is characterized by a variety of patterns, including myxofibrous tissue, fibrous dysplasia-like features, and ischemic ossification...
May 2016: Archives of Pathology & Laboratory Medicine
Telmo Augusto Barba Belsuzarri, João Flavio Mattos Araujo, Carlos Alberto Morassi Melro, Maick Willen Fernandes Neves, Juliano Nery Navarro, Leandro Gomes Brito, Luis Otavio Carneiro Pontelli, Luis Gustavo de Abreu Mattos, Tiago Fernandes Gonçales, Wolnei Marques Zeviani
BACKGROUND: Fibrous dysplasia (FD) is a benign fibro-osseous lesion related to an abnormal bone development and replacement by fibrous tissue. FD has three clinical patterns namely monostotic, polyostotic, and the McCune-Albright syndrome (MAS). MAS is a rare genetic disorder (about 3% of all FD's) that comprises a triad of polyostotic FD, café-au-lait skin macules, and precocious puberty. MAS can involve the orbit region and cause stenosis in the optic canal, leading the patient to a progressive visual loss...
2016: Surgical Neurology International
Zdenek Rehak, Beatrix Bencsikova, Iva Zambo, Tomas Kazda
Fibrous dysplasia (FD) is a benign bone lesion in which normal bone marrow is replaced by fibro-osseous tissue. The usual high fluorodeoxyglucose (F-FDG) uptake in FD may lead to the misdiagnosis of bone malignancy. Herein, we describe the case of a 42-year old man with histologically verified FD of the pubic bone, which has been subsequently examined during follow-up for rectal cancer, using both F-FDG and fluorothymidine (F-FLT) PET/CT imaging. The FD lesion was characterized by a high uptake of F-FDG (hot spot) but very low uptake of F-FLT (cold spot) as compared with the contralateral unaffected pubic bone...
June 2016: Clinical Nuclear Medicine
Maxime Guérin, Juliette Thariat, Mounia Ouali, Corinne Bouvier, Anne-Valérie Decouvelaere, Elisabeth Cassagnau, Sébastien Aubert, Sébastien Lepreux, Jean-Michel Coindre, Séverine Valmary-Degano, Frédérique Larousserie, Julie Meilleroux, Fabrice Projetti, Nathalie Stock, Christine Galant, Béatrice Marie, Isabelle Peyrottes, Gonzague de Pinieux, Anne Gomez-Brouchet
In contrast to long bone osteosarcoma, mandibular osteosarcoma is highly heterogeneous and morphologically overlaps with benign tumors, obscuring diagnosis and treatment selection. Molecular characterization is difficult due to the paucity of available specimens of this rare disease. We aimed to characterize the spectrum of mandibular osteosarcoma using immunohistochemistry and molecular techniques (quantitative polymerase chain reaction and sequencing) and compare them with benign fibro-osseous lesions. Forty-nine paraffin-embedded mandible osteosarcoma tissue samples were collected retrospectively and compared with 10 fibrous dysplasia and 15 ossifying fibroma cases...
April 2016: Human Pathology
Manveen Kaur Jawanda, Ravi Narula, Madhu Shankari, Shruti Gupta
Hybrid lesions are the lesions consisting of association of features from different pathologies. We present a rare case of hybrid lesion with features of central giant cell granuloma (CGCG) and fibrous dysplasia (FD) involving mandible in a 33-year-old male. Hybrid lesions consisting of features of both benign fibro-osseous lesions and CGCG are very rare. Thus, the purpose of this paper is not only to present a rare case of hybrid lesion with features of CGCG and FD but also to emphasize on the need of careful clinical, radiological and histopathological examination of each and every tissue...
September 2015: Journal of Oral and Maxillofacial Pathology: JOMFP
Surajit Bhattacharya, R K Mishra
Fibrous dysplasia (FD) is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect in FD is post-natal mutation of GNAS1 gene, which leads to the proliferation and activation of undifferentiated mesenchymal cells arresting the bone development in woven phase and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation...
September 2015: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
Arabella I Leet, Alison M Boyce, Khalda A Ibrahim, Shlomo Wientroub, Harvey Kushner, Michael T Collins
BACKGROUND: Polyostotic fibrous dysplasia is a skeletal disease that results from somatic activating mutations in the gene GNAS in skeletal stem cells, leading to proliferation of immature osteogenic cells with replacement of normal marrow and bone with fibro-osseous tissue. Lesions may cause bone deformity or fracture. In the surgical care of polyostotic fibrous dysplasia, the role of grafting and the optimal grafting material are not clear. The purpose of this study was to evaluate the long-term survival of bone-grafting procedures in subjects with polyostotic fibrous dysplasia over time...
February 3, 2016: Journal of Bone and Joint Surgery. American Volume
M Ciniglio Appiani, B Verillaud, D Bresson, E Sauvaget, J-P Blancal, J-P Guichard, J-P Saint Maurice, M Wassef, A Karligkiotis, R Kania, P Herman
Fibro-osseous benign lesions rarely affect the sinonasal tract and are divided into 3 different entities, namely osteoma, fibrous dysplasia and ossifying fibroma. They share several clinical, radiological and histological similarities, but have different behaviours. Ossifying fibroma, and in particular the "juvenile" histological subtype, may have a locally aggressive evolution and a high risk for recurrence if removal is incomplete. The purpose of the present study is to compare the clinical behaviour of ossifying fibroma with the other benign fibro-osseous lesions; highlight different behaviour between the histological subtypes; compare the advantages, limitations and outcomes of an endoscopic endonasal approach with reports in the literature...
October 2015: Acta Otorhinolaryngologica Italica
Sook-Bin Woo
Currently, central cemento-ossifying fibroma is classified by the World Health Organization as a primary bone-forming tumor of the jaws. However, histopathologically, it is often indistinguishable from cemento-osseous dysplasias in that it forms osteoid and cementicles (cementum droplets) in varying proportions. It is believed that pluripotent cells within the periodontal membrane can be stimulated to produce either osteoid or woven bone and cementicles when stimulated. If this is true, cemento-ossifying fibroma would be better classified as a primary odontogenic neoplasm arising from the periodontal ligament...
December 2015: Journal of Oral and Maxillofacial Surgery
Atul Ratra, Adam Wooldridge, George Brindley
Osteofibrous dysplasia and adamantinoma are rare lesions of primary benign and malignant bone tumors with an incidence of less than 1%. These lesions arise primarily in long bones with a predilection for the tibia and fibula. Osteofibrous dysplasia is a benign fibro-osseous lesion typically found in children younger than 10 years. Adamantinomas, however, are highly malignant and invasive tumors found predominantly in adult men, with an average age of diagnosis between 20 and 50 years. Debate continues on whether osteofibrous dysplasia and adamantinoma occupy the same disease spectrum...
October 2015: American Journal of Orthopedics
Débora Delai, Anarela Bernardi, Gabriela Santos Felippe, Cleonice da Silveira Teixeira, Wilson Tadeu Felippe, Mara Cristina Santos Felippe
Florid cemento-osseous dysplasia (FCOD) belongs to the group of fibro-osseous lesions in which normal bone is replaced by fibrous connective tissue and calcified cementum tissue of the avascular type. Among the various types of fibro-osseous lesions, FCOD is one of the most commonly encountered diseases in clinical practice and may involve 3 or 4 of the quadrants. FCOD is located in the periapical regions of teeth, and the lesions are predominantly radiolucent (osteolytic phase), become mixed over time (cementoblast phase), and ultimately become radiopaque (osteogenic phase) with a thin radiolucent peripheral halo...
November 2015: Journal of Endodontics
Mirna S Cordeiro, André R Backes, Antônio F Durighetto Júnior, Elmar H G Gonçalves, Jefferson X de Oliveira
Fibrous dysplasia (FD) is a developmental anomaly in which the normal medullary space of the affected bone is replaced by fibro-osseous tissue. This condition is typically encountered in adolescents and young adults. It affects the maxillofacial region and it can often cause severe deformity and asymmetry. Therefore, accurate diagnosis is critical to determine the appropriate treatment of each case. In this sense, computed tomography (CT) is a relevant resource among the imaging techniques for correct diagnosis of this condition...
February 2016: Journal of Digital Imaging: the Official Journal of the Society for Computer Applications in Radiology
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