94. Fibrous Dysplasia

  1. Clifford J. Rosen MD
  1. Michael T. Collins,
  2. Mara Riminucci and
  3. Paolo Bianco

Published Online: 19 JUL 2013

DOI: 10.1002/9781118453926.ch94

Primer on the Metabolic Bone Diseases and Disorders of Mineral Metabolism, Eighth Edition

Primer on the Metabolic Bone Diseases and Disorders of Mineral Metabolism, Eighth Edition

How to Cite

Collins, M. T., Riminucci, M. and Bianco, P. (2013) Fibrous Dysplasia, in Primer on the Metabolic Bone Diseases and Disorders of Mineral Metabolism, Eighth Edition (ed C. J. Rosen), John Wiley & Sons, Inc., Ames, USA. doi: 10.1002/9781118453926.ch94

Publication History

  1. Published Online: 19 JUL 2013

ISBN Information

Print ISBN: 9781118453889

Online ISBN: 9781118453926

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Keywords:

  • bone scintigraphy;
  • fibrous dysplasia of bone (FD);
  • osteogenic sarcoma

Summary

This chapter discusses the etiology and pathogenesis, clinical features, management and treatment of fibrous dysplasia of bone (FD). FD is an uncommon skeletal disorder with a broad spectrum of clinical presentation. The disease may involve one bone (monostotic FD), multiple bones (polyostotic FD), or the entire skeleton (panostotic FD). Osteogenic sarcoma is the most common type of bone tumor that may complicate FD. The clinical course is usually aggressive, and surgery is the primary treatment; chemotherapeutic regimens do not appear to improve prognosis significantly. Diagnosis of FD must be established based on expert assessment of clinical, radiographic, and histopathological features. Markers of bone turnover are usually elevated. The extent of the skeletal disease is best determined with total body bone scintigraphy, which can be used to assess the skeletal disease burden and predict functional outcome.