Cases reported "Fibroma, Ossifying"

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1/13. Aggressive cemento-ossifying fibroma of the jaws.

    The cemento-ossifying fibroma is probably the most frequent fibro-osseous lesion seen by oral pathologists. Occasionally, reports of lesions behaving in an aggressive fashion appear in the literature. At the present time, the relationship if these "aggressive" lesions to the "usual" fibromas is unclear. Two cases of "aggressive" cemento-ossifying fibroma are reported. Certainly, clinical, radiological, and histological characteristics of aggressive and usual fibromas do coincide. There are, however, diverging features warranting separation of different forms. These mild differences may be detected during preoperative assessment of the lesion, allowing a diagnosis of aggressive form to be made.
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2/13. Developing cementoblastoma: case report and update of differential diagnosis.

    The benign cementoblastoma is a rare odontogenic tumor that tends to occur in the jaws of young people. It is most commonly characterized as a circumscribed radiopaque mass attached to the tooth roots and rimmed by a thin radiolucent zone. Early lesions, however, are radiolucent and can be confused with periapical conditions resulting from nonvital teeth. The tumor is seldom recognized until it produces pain or expansion of the jaw. If the diagnosis is established early, the tooth can be saved by endodontic treatment followed by apical root resection and surgical enucleation of the tumor. This case report documents the origin, development, and unlimited growth potential of a cementoblastoma. Radiographs taken at yearly intervals reveal an initial widening of the periodontal ligament space that grew into a 3-cm mixed radiolucent and radiopaque tumor over a 4-year period. The differential diagnosis and some criteria to help distinguish the cementoblastoma from similar-appearing lesions are reviewed.
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3/13. Gnathodiaphyseal dysplasia: a syndrome of fibro-osseous lesions of jawbones, bone fragility, and long bone bowing.

    We report an unusual generalized skeletal syndrome characterized by fibro-osseous lesions of the jawbones with a prominent psammomatoid body component, bone fragility, and bowing/sclerosis of tubular bones. The case fits with the emerging profile of a distinct syndrome with similarities to previously reported cases, some with an autosomal dominant inheritance and others sporadic. We suggest that the syndrome be named gnathodiaphyseal dysplasia. The patient had been diagnosed previously with polyostotic fibrous dysplasia (PFD) elsewhere, but further clinical evaluation, histopathological study, and mutation analysis excluded this diagnosis. In addition to providing a novel observation of an as yet poorly characterized syndrome, the case illustrates the need for stringent diagnostic criteria for FD. The jaw lesions showed fibro-osseous features with the histopathological characteristics of cemento-ossifying fibroma, psammomatoid variant. This case emphasizes that the boundaries between genuine GNAS1 mutation-positive FD and other fibro-osseous lesions occurring in the jawbones should be kept sharply defined, contrary to a prevailing tendency in the literature. A detailed pathological study revealed previously unreported features of cemento-ossifying fibroma, including the participation of myofibroblasts and the occurrence of psammomatoid bodies and aberrant mineralization, within the walls of blood vessels. transplantation of stromal cells grown from the lesion into immunocompromised mice resulted in a close mimicry of the native lesion, including the sporadic formation of psammomatoid bodies, suggesting an intrinsic abnormality of bone-forming cells.
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4/13. Unusual jaw lesions in the paediatric and adolescent patient: a management challenge.

    While major maxillofacial pathology in the young patient is relatively uncommon, non-malignant conditions may pose a significant treatment dilemma due to their aggressive or unpredictable behaviour. Several such diseases managed by the Oral and Maxillofacial Surgery Unit at the Royal Children's Hospital of Melbourne have been selected for review. Illustrative case reports to highlight the principles of management are presented.
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5/13. Familial ossifying fibromas: report of two cases.

    Ossifying fibroma is a benign fibro-osseous lesion of the jaw containing varying amounts of calcified deposits such as bone, cementum or both. This type of lesion is referred to as dysplastic or neoplastic in nature. In 2000, a 52-year-old male patient was referred to our clinic complaining of a giant swelling in the mandibular premolar-molar region. A histopathological diagnosis of ossifying fibroma was made. Three months later, his daughter was admitted with a swelling on her mandible. Following biopsy, this patient was also diagnosed as having ossifying fibroma. The present report describes these two cases of familial and multiple ossifying fibromas.
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6/13. Ossifying fibroma of the upper jaw: report of a case and review of the literature.

    A number of processes generically referred to as benign fibroosseous lesions comprise different disorders such as fibrous dysplasia, sclerotizing osteomyelitis and ossifying or cementing fibroma. These processes are all characterized by the existence of a vascular fibroblastic stroma, with the production of a calcified matrix ranging from bone to cementum. Ossifying fibroma involves slow-evolving growth with deforming swelling generally arising in the mandible, with possible early dental displacement. From the radiological perspective the disorder generally manifests as a well defined and delimited, unilocular radiotransparency, as a radiotransparent image with central opacifications, or as multilocular transparencies. The lesions exceptionally can be radiopaque. We present the case of a 22-year-old male presenting for evaluation of a three-month, asymptomatic tumor mass in the anterior sector of the upper jaw. Radiologically, the lesion appeared as a radiotransparent zone surrounded by a poorly delimited sclerotic halo. The definitive diagnosis following surgical resection of the lesion was ossifying fibroma. The case is discussed, and a review is provided of the literature on the subject.
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7/13. Clinico-pathological conference 2002.

    INTRODUCTION: Six cases are reported, each presented at the 11th Biennial Congress of the International association of Oral Pathologists as an instructive case for differential diagnosis on the basis of clinical, imaging or histological features. CLINICAL PICTURE: Case diagnoses included a large, possibly intraosseous, myofibroma presenting with an oral mass; Langerhans cell histiocytosis with facial skin lesions; an intraosseous vascular hamartoma of the maxilla with worrying radiological features; an unusual mixed radiolucency of the jaw caused by cemento-ossifying fibroma; an osteosarcoma of the posterior mandible causing a well-defined radiolucency and an intraoral squamous cell carcinoma in a child.
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8/13. "Atypical" fibro-osseous lesions: diagnostic challenges and treatment concepts.

    The term fibro-osseous lesion is a generic designation of a group of jaw disorders (ranging from inflammatory to neoplastic) that microscopically exhibit, a connective-tissue matrix and islands/trabeculae of bone. Although the histologic appearance, and frequently the clinical and radiographic features, may be similar for many of these lesions, they demonstrate a wide range of biologic behavior. Treatment therefore is varied, and there are few well-established guidelines to help the surgeon. Five fibro-osseous lesions with atypical features are presented to demonstrate the diagnostic difficulties associated with these lesions and to show the need for accurate classification in order to help direct treatment and predict outcome. Final diagnoses for "atypical" fibro-osseous lesions included: multiple ossifying fibromas, unclassifiable fibro-osseous lesion, osteosarcoma with concomitant multiple ossifying fibromas, juvenile or psammomatoid ossifying fibroma, and central low-grade osteosarcoma. "Fibro-osseous lesion" should be viewed as a working diagnosis, and a definitive diagnosis should be actively sought; the addition of "atypical" to this designation suggests the likelihood of an aggressive lesion. Treatment of lesions having atypical and nonclassical microscopic features must be based on the biologic behavior of the tumor.
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9/13. Psammomatoid ossifying fibroma of the paranasal sinuses. An extragnathic variant of cemento-ossifying fibroma. Report of three cases.

    Psammomatoid ossifying fibroma (POF) is a diagnostic designation for a sinonasal or orbital fibroosseous lesion characterized by numerous small round mineralized spherules lying in a fibroblastic stroma. We report 3 cases. One occurred in the frontal sinus of an 18-years-old male; 1 in the frontal sinus of a 20-year-old male and 1 in the maxillary sinus of a 36-year-old male. Rather limited excision resulted in a disease-free follow-up time from 2 to 6 years. The histomorphology of POF was compared with other fibro-osseous lesions of the craniofacial skeleton including the jaws. It appeared that POF is similar to cemento-ossifying fibroma occurring in the jaws and the necessity for different names for lesions only differing in their site of occurrence is considered to be at least debatable.
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10/13. Ophthalmologic abnormalities in encephalocraniocutaneous lipomatosis.

    Encephalocraniocutaneous lipomatosis (ECCL) is a sporadically occurring disorder that belongs to the group of neurocutaneous syndromes. Important characteristics of the case we present are: intracranial lipomas, a skull hamartoma, bilateral lipodermoids and jaw tumors (ossifying fibromas and compound odontomas). We propose four minimal criteria for the diagnosis of ECCL and review the ocular abnormalities reported to date.
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