Osseous Dysplasia with Gross Jaw Expansion: A Review of 18 Lesions

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Abstract

<p class="first" id="d6430501e118"> Fourteen cases with 18 grossly expansive lesions diagnosed over a period of 15 years as either “familial gigantiform cementoma” or “osseous dysplasia with jaw expansion” in an African population sample were reviewed. Eight lesions occurred in the anterior mandible, the maxilla was affected by four, three patients presented with more than one lesion and the most common associated pathologies were tooth displacement, conventional non expanding florid osseous dysplasia and simple bone cyst. No history of similar lesions in relatives of the diseased were recorded. The radiolucent fibrous component contained globular bone deposits and cellular osteoid with trabecular differentiation which matured into radiodense mineralized masses. Resorption of the cellular bone created cavities which are proposed to represent the early stage of simple bone cyst formation. It is recommended that “expansive osseous dysplasia” replace the out-dated term “familial gigantiform cementoma”. The differential diagnoses of expansive osseous dysplasias are discussed. </p>

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NF-κB signaling and bone resorption.

Salma Abu El-Nazar (2013)

The transcription factor NF-κB is a family of proteins involved in signaling pathways essential for normal cellular functions and development. Deletion of various components of this pathway resulted with abnormal skeletal development. Research in the last decade has established that NF-κB signaling mediates RANK ligand-induced osteoclastogenesis. Consistently, it was shown that inhibition of NF-κB was an effective approach to inhibit osteoclast formation and bone resorptive activity. Identification of the molecular machinery underlying NF-κB activation permitted osteoclast-specific deletion of the major components of this pathway. As a result, it was clear that deletion of members of the proximal IKK kinase complex and the distal NF-κB subunits and downstream regulators affected skeletal development. These studies provided several targets of therapeutic intervention in osteolytic diseases. NF-κB activity has been also described as the centerpiece of inflammatory responses and is considered a potent mediator of inflammatory osteolysis. Indeed, inflammatory insults exacerbate physiologic RANKL-induced NF-κB signals leading to exaggerated responses and to inflammatory osteolysis. These superimposed NF-κB activities appear to underlie several bone pathologies. This review will describe the individual roles of NF-κB molecules in bone resorption and inflammatory osteolysis.

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MDM2 and CDK4 immunohistochemistry is a valuable tool in the differential diagnosis of low-grade osteosarcomas and other primary fibro-osseous lesions of the bone.

Frederique Larousserie, Anne Gomez-Brouchet, Matthieu Bui Nguyen Binh … (2011)

Low-grade osteosarcoma is a rare malignancy that may be subdivided into two main subgroups on the basis of location in relation to the bone cortex, that is, parosteal osteosarcoma and low-grade central osteosarcoma. Their histological appearance is quite similar and characterized by spindle cell stroma with low-to-moderate cellularity and well-differentiated anastomosing bone trabeculae. Low-grade osteosarcomas have a simple genetic profile with supernumerary ring chromosomes comprising amplification of chromosome 12q13-15, including the cyclin-dependent kinase 4 (CDK4) and murine double-minute type 2 (MDM2) gene region. Low-grade osteosarcoma can be confused with fibrous and fibro-osseous lesions such as fibromatosis and fibrous dysplasia on radiological and histological findings. We investigated MDM2-CDK4 immunohistochemical expression in a series of 72 low-grade osteosarcomas and 107 fibrous or fibro-osseous lesions of the bone or paraosseous soft tissue. The MDM2-CDK4 amplification status of low-grade osteosarcoma was also evaluated by comparative genomic hybridization array in 18 cases, and the MDM2 amplification status was evaluated by fluorescence in situ hybridization or quantitative real-time polymerase chain reaction in 31 cases of benign fibrous and fibro-osseous lesions. MDM2-CDK4 immunostaining and MDM2 amplification by fluorescence in situ hybridization or quantitative real-time polymerase chain reaction were investigated in a control group of 23 cases of primary high-grade bone sarcoma, including 20 conventional high-grade osteosarcomas, two pleomorphic spindle cell sarcomas/malignant fibrous histiocytomas and one leiomyosarcoma. The results showed that MDM2 and/or CDK4 immunoreactivity was present in 89% of low-grade osteosarcoma specimens. All benign fibrous and fibro-osseous lesions and the tumors of the control group were negative for MDM2 and CDK4. These results were consistent with the MDM2 and CDK4 amplification results. In conclusion, immunohistochemical expression of MDM2 and CDK4 is specific and provides sensitive markers for the diagnosis of low-grade osteosarcomas, helping to differentiate them from benign fibrous and fibro-osseous lesions, particularly in cases with atypical radio-clinical presentation and/or limited biopsy samples.

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Benign fibro-osseous lesions: a review of current concepts.

Randal Fowler, John R. Brannon (2001)

The benign fibro-osseous lesions (BFOL) represent a clinically diverse group of disorders of bone that share similar histopathologic features. As a group, they are relatively common in the craniofacial complex, especially the jaws. Although the general concept of BFOL is relatively well known, specific diagnostic interpretation of individual cases is often challenging. New concepts and controversies have arisen over the past 10 to 15 years regarding classification and diagnostic criteria. However, among the new theories and contentions, there is now essential agreement that the osseous dysplasias represent a single disease process, while the so-called "juvenile active ossifying fibroma" and other "aggressive," "active," "psammomatoid" ossifying/cementifying fibromas remain controversial. This review is presented to update the surgical pathologist on the various entities comprising the spectrum of BFOL and to examine the criteria for their diagnosis.