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A unique case of multiple non-ossifying fibromas with polyostotic monomelic distribution and aggressive clinical course

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Abstract

Multiple non-ossifying fibromas (MNOFs) occur either isolated or in association with other anomalies, are usually localized in the long bones of the lower limbs, may be radiographically confused with other skeletal lesions, and tend to heal spontaneously with the completion of the skeletal growth. Segmental distribution, either monomelic or polymelic and ipsilateral, is rare and commonly observed in the context of developmental diseases known as “RASopathies”, which are caused by mutations in genes that encode components or regulators within the Ras/mitogen-activated protein kinase signaling pathway. We describe here the radiographic and pathologic features of an 18-year-old Caucasian boy, whose clinical history started at the age of 3 when the diagnosis of aneurysmal bone cyst was made on a lytic lesion of his left clavicle. Over the following 2 years, the patient developed polyostotic and monomelic lesions within the left humerus, radius, and ulna. No other skeletal and extra-skeletal anomalies were clinically detected. The lesions were interpreted as consistent with polyostotic fibrous dysplasia and MNOFs and showed an unusually aggressive clinical course with progressive increase in size and coalescence. The definitive diagnosis of MNOFs was made after the exclusion of fibrous dysplasia by molecular analysis. The polyostotic and monomelic distribution of the lesions and the unusually aggressive clinical course contribute to make this case of MNOFs unique.

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Authors and Affiliations

  1. Department of Molecular Medicine, Sapienza University, Rome, Italy

    Alessandro Corsi, Cristina Remoli & Mara Riminucci

  2. Department of Orthopaedic Surgery, University of Rome Tor Vergata, c/o PTV, Viale Oxford 81, 00133, Rome, Italy

    Ernesto Ippolito

  3. Department of Orthopaedic Surgery, Aghia Sofia Children’s Hospital, Athens, Greece

    John Dimitriou

Authors
  1. Alessandro Corsi
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  2. Cristina Remoli
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  3. Mara Riminucci
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  4. Ernesto Ippolito
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  5. John Dimitriou
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Corresponding author

Correspondence to Ernesto Ippolito.

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All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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The authors declare that they have no conflicts of interest.

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Corsi, A., Remoli, C., Riminucci, M. et al. A unique case of multiple non-ossifying fibromas with polyostotic monomelic distribution and aggressive clinical course. Skeletal Radiol 46, 233–236 (2017). https://doi.org/10.1007/s00256-016-2523-3

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  • DOI: https://doi.org/10.1007/s00256-016-2523-3

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