Journal article
Fibrous dysplasia for radiologists: beyond ground glass bone matrix
Insights into imaging, v 9(6), pp 1035-1056
01 Dec 2018
PMID: 30484079
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Fibrous dysplasia (FD) is a congenital disorder arising from sporadic mutation of the α-subunit of the Gs stimulatory protein. Osseous changes are characterised by the replacement and distortion of normal bone with poorly organised, structurally unsound, fibrous tissue. The disease process may be localised to a single or multiple bones. In McCune-Albright syndrome (MAS), fibrous dysplasia is associated with hyperfunction of endocrine organs and overproduction of melanin in the skin, while Mazabraud syndrome FD is associated with intramuscular myxomas. In radiology, FD is very often automatically associated with the term “ground glass matrix”. However, FD is a complex disease, and knowledge of its unique pathogenesis and course are crucial to understanding imaging findings and potential complications. This article aims to not only summarise the spectrum of radiological findings of osseous and extra-osseous abnormalities associated with FD but also to highlight the pathological base of the disease evolution, corresponding imaging changes and complications based on the disease distribution. We also have provided current recommendations for clinical management and follow-up of patients with FD.
Teaching Points
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FD is often a part of complex disease, involving not only bone but also multiple other organs.
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FD lesions are characterised by age-related histological, radiographical and clinical transformations.
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Radiologists play a crucial role in the identification of osseous complications associated with FD.
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The craniofacial form of the disease is the most common type of FD and the most difficult form to manage.
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Patients with McCune-Albright syndrome may have different extra-skeletal abnormalities, which often require follow-up.
Metrics
Details
- Title
- Fibrous dysplasia for radiologists: beyond ground glass bone matrix
- Creators
- Yevgeniya S. Kushchayeva - National Institute of Diabetes and Digestive and Kidney DiseasesSergiy V. Kushchayev - Johns Hopkins HospitalTetiana Y. Glushko - Catholic Medical CenterSri Harsha Tella - University of South CarolinaOleg M. Teytelboym - Catholic Medical CenterMichael T. Collins - National Institute of Dental and Craniofacial ResearchAlison M. Boyce - National Institutes of Health
- Publication Details
- Insights into imaging, v 9(6), pp 1035-1056
- Publisher
- Springer Berlin Heidelberg
- Grant note
- This research was supported by the Intramural Research Program of the National Institute of Dental and Craniofacial Research
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Radiology (Radiologic Sciences); Medicine (Graduate)
- Web of Science ID
- WOS:000451775100013
- Scopus ID
- 2-s2.0-85057805476
- Other Identifier
- 991021897585604721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Radiology, Nuclear Medicine & Medical Imaging