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      Locally aggressive monostotic fibrous dysplasia of the cervical spine mimicking malignancy: a case report and literature review


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          We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process.

          Owing to the vertebral instability, a surgical procedure combining C0–C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5–6 laxity below the intervertebral fixation.

          This case combines three radiological findings, which are unusual in fibrous dysplasia: monostotic presentation involving the spine, some aggressive radiographic features, and a pathological fracture.

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          Identification of a mutation in the gene encoding the alpha subunit of the stimulatory G protein of adenylyl cyclase in McCune-Albright syndrome.

          McCune-Albright syndrome (MAS) is characterized by polyostotic fibrous dysplasia, café-au-lait lesions, and a variety of endocrine disorders, including precocious puberty, hyperthyroidism, hypercortisolism, growth hormone excess, and hyperprolactinemia. The diverse metabolic abnormalities seen in MAS share the involvement of cells that respond to extracellular signals through activation of the hormone-sensitive adenylyl cyclase system (EC Mutations that lead to constitutive activation of Gs alpha, the guanine nucleotide-binding regulatory protein that stimulates adenylyl cyclase activity, have been identified in a subset of human growth hormone-secreting pituitary tumors and human thyroid tumors. We report here the identification of a mutation in the gene encoding Gs alpha in a patient with MAS. Denaturing gradient gel electrophoresis was used to analyze amplified DNA fragments including exon 8 or exon 9 of the Gs alpha gene. In one subject with MAS a G-to-A transition was found in exon 8 of one of the two alleles encoding Gs alpha. This single-base substitution results in the replacement of arginine by histidine at position 201 of the mature Gs alpha protein. Semiquantitative analysis of amplified DNA indicated that the mutant allele was less prevalent than the wild-type allele in peripheral leukocytes and was present in very low levels in skin. These findings support the previous contention that the segmental distribution and variable expression of the cutaneous, skeletal, and endocrine manifestations of MAS reflect an underlying somatic mosaicism. Further, these results suggest that the molecular basis of MAS is a postzygotic mutation in Gs alpha that causes constitutive activation of adenylyl cyclase.
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              The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study.


                Author and article information

                SICOT J
                SICOT J
                EDP Sciences
                27 September 2019
                : 5
                : ( publisher-idID: sicotj/2019/01 )
                : 34
                [1 ] Department of Radiology, Hôpital Lariboisière, Assistance Publique des Hôpitaux de Paris 2 rue Ambroise Paré 75010 Paris France
                [2 ] Department of Pathology, Hôpital Lariboisière, Assistance Publique des Hôpitaux de Paris 2 rue Ambroise Paré 75010 Paris France
                [3 ] Department of Pathology, Hôpital Cochin, Assistance Publique des Hôpitaux de Paris 27 rue du Faubourg Saint-Jacques 75014 Paris France
                [4 ] Department of Neurosurgery, Fondation Ophtalmologique de Rothschild 29 rue Manin 75019 Paris France
                [5 ] Department of Rheumatology, Hôpital La Croix Saint-Simon 125 rue d’Avron 75020 Paris France
                Author notes
                [* ]Corresponding author: audrey.milon.am@ 123456gmail.com
                Author information
                sicotj190060 10.1051/sicotj/2019024
                © The Authors, published by EDP Sciences, 2019

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                : 04 July 2019
                : 07 July 2019
                Page count
                Figures: 3, Tables: 1, Equations: 0, References: 38, Pages: 5
                Review Article

                monostotic fibrous dysplasia,cervical spine,pathological fracture,aggressive


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