Chondrosarcoma arising in monostotic fibrous dysplasia is very rare.Wide surgical margin provides good local control, thus become the mainstay treatment of chondrosarcoma.Limb salvage surgery in chondrosarcoma provides good functional outcome.Fibrous dysplasia (FD) is a formative tumor-like disease that is characterized as an excessive expansion of fibrous connective tissue intermingled with irregular hard trabeculae by replacing ordinary bone. This disease has a lot of clinical symptoms variants due to varying degrees of mosaicism. It can be asymptomatic to severely disabling symptoms. It may occur in alone, or as a syndrome with extra skeletal disease manifestations. Alteration of fibrous dysplasia to malignancy is rare with incidence in <1% of fibrous dysplasia. The most prevalence type of malignancy developed from fibrous dysplasia is osteosarcoma (70% of cases), followed by fibrosarcoma , and chondrosarcoma (10% of cases), with malignant fibrous histiocytoma (4% of cases). We reported a 54-years-old male whom initially diagnosed with monostotic fibrous dysplasia in femoral diaphysis and had transformed into chondrosarcoma after 3 years. This case report has been reported in line with the SCARE Criteria.Fibrous dysplasia is a rare and potentially debilitating bone disease.Animal models of fibrous dysplasia are key for further research and drug discovery.A systematic review (PRISMA format) was conducted to compare animal models.Seven unique animal models of the disease have been developed with varying validity.We used the NanoString technology to analyze Formalin Fixed Decalcified Paraffin Embedded (FFDPE) Fibrous Dysplasia samples.

We show that Fibrous Dysplasia lesions may have different molecular profiles consistent with its histological heterogeneity.NanoString technology is a valuable tool for molecular studies on rare bone diseases by using FFDPE archival material.Fibrous dysplasia (FD) is a rare skeletal disorder caused by a postzygotic mutation in the GNAS gene. FD can present as a solitary lesion in monostotic FD or as polyostotic disease with lesions in multiple bones. McCune Albright syndrome (MAS) is established when FD coincides with endocrinopathies or with other extra skeletal features such as GNAS-positive tumors or café au lait patches, while FD with intramuscular myxomas is identified as Mazabraud syndrome. In FD/MAS (OMIM#174800) fibro-osseous skeletal lesions are formed, inducing a variable, potentially severe clinical presentation that can include pain, fractures and deformities. The GNAS mutation disrupts the maturation of mutated osteoprogenitor cells into osteoblasts and thus bone formation. Bone remodeling is further disturbed by the increased production of cytokines including IL-6 and RANKL, stimulating bone resorption .

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Mishita
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Journal of Bone Research and Reports