eMedicine Specialties > Radiology > Musculoskeletal

Fibrous Dysplasia: Follow-up

Author: Mahesh Kumar Neelala Anand, MBBS, DNB, FRCR, Clinical Director, Consultant Radiologist, Department of Radiology, Pennine Acute Hospitals NHS Trust, Manchester, UK
Contributor Information and Disclosures

Updated: Jul 29, 2009

Intervention

Special Concerns

  • Malignant degeneration
    • The estimated frequency is 0.4-1% in fewer than 50 reported cases.
    • The interval from the diagnosis of fibrous dysplasia to the development of malignancy varies and is usually years or decades.
    • Most often, skull and facial bones undergo malignant change in monostotic disease, whereas femoral and facial bones undergo malignant change in polyostotic disease.
    • Osteosarcoma and fibrosarcoma are the most common tumors. Chondrosarcomas occur less frequently.
    • Radiographic features suggestive of malignant degeneration include a rapid increase in the size of the lesion and a change from a previously mineralized bony lesion to a lytic lesion. Clinical findings of increasing pain and an enlarging soft-tissue mass suggest malignant change.
  • Metabolic changes
    • Hypophosphatemic rickets and osteomalacia have been noted in patients with fibrous dysplasia.
    • One hypothesis to explain the associated metabolic disorder suggests that lesions such as fibrous dysplasia synthesize phosphaturic hormone.
 


More on Fibrous Dysplasia

Overview: Fibrous Dysplasia
Imaging: Fibrous Dysplasia
Follow-up: Fibrous Dysplasia
Multimedia: Fibrous Dysplasia
References
Further Reading
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References

  1. Macdonald-Jankowski D. Fibrous dysplasia: a systematic review. Dentomaxillofac Radiol. May 2009;38(4):196-215. [Medline].

  2. Harris WH, Dudley HR, Barry RJ. The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study. J Bone Joint Surg Am. Mar 1962;44-A:207-33.

  3. Leet AI, Magur E, Lee JS, et al. Fibrous dysplasia in the spine: prevalence of lesions and association with scoliosis. J Bone Joint Surg Am. Mar 2004;86-A(3):531-7.

  4. Lichenstein L, Jaffe HL. Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Pathol. 1942;33:777.

  5. National Institutes of Health. Osteoporosis and Related Bone Disorders-National Resource Center Web site. Fast Facts on Fibrous Dysplasia page. Available at: http://www.osteo.org/default.asp. Washington, DC: National Institutes of Health;2001. [Full Text].

  6. Resnick D, Niwayama G. Diagnosis of Bone and Joint Disorders. 2nd ed. Philadelphia, Pa: WB Saunders;. 1988: 4057-70.

  7. Kruse A, Pieles U, Riener MO, Zunker Ch, Bredell MG, Grätz KW. Craniomaxillofacial fibrous dysplasia: a 10-year database 1996-2006. Br J Oral Maxillofac Surg. Jun 2009;47(4):302-5. [Medline].

  8. Mancini F, Corsi A, De Maio F, Riminucci M, Ippolito E. Scoliosis and spine involvement in fibrous dysplasia of bone. Eur Spine J. Feb 2009;18(2):196-202. [Medline].

  9. Ziadi S, Trimeche M, Mokni M, Sriha B, Khochtali H, Korbi S. [Eighteen cases of craniofacial fibrous dysplasia.]. Rev Stomatol Chir Maxillofac. Jul 15 2009;[Medline].

  10. Rahman AM, Madge SN, Billing K, Anderson PJ, Leibovitch I, Selva D, et al. Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes. Eye. Jan 30 2009;[Medline].

  11. Valentini V, Cassoni A, Marianetti TM, Terenzi V, Fadda MT, Iannetti G. Craniomaxillofacial fibrous dysplasia: conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg. Feb 2009;123(2):653-60. [Medline].

  12. Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci. Jan 1964;247:1-20. [Medline].

  13. Lädermann A, Stern R, Ceroni D, De Coulon G, Taylor S, Kaelin A. Unusual radiologic presentation of monostotic fibrous dysplasia. Orthopedics. Mar 2008;31(3):282. [Medline].

  14. Bulakbasi N, Bozlar U, Karademir I, Kocaoglu M, Somuncu I. CT and MRI in the evaluation of craniospinal involvement with polyostotic fibrous dysplasia in McCune-Albright syndrome. Diagn Interv Radiol. Dec 2008;14(4):177-81. [Medline].

  15. Sood A, Raman R, Jhobta A, Dhiman DS, Seam RK. Normal technetium-99m-MDP uptake in fibrous dysplasia of the hip. Hell J Nucl Med. Jan-Apr 2009;12(1):72-3. [Medline].

  16. Bonekamp D, Jacene H, Bartelt D, Aygun N. Conversion of FDG PET activity of fibrous dysplasia of the skull late in life mimicking metastatic disease. Clin Nucl Med. Dec 2008;33(12):909-11. [Medline].

  17. Daffner RH, Kirks DR, Gehweiler JA Jr, Heaston DK. Computed tomography of fibrous dysplasia. AJR Am J Roentgenol. Nov 1982;139(5):943-8. [Medline].

  18. De Smet A, Travers H, Neff JR. Chondrosarcoma occurring in a patient with polyostotic fibrous dysplasia. Skeletal Radiol. 1981;7:197.

  19. King RM, Payne WS, Olafsson S, Unni KK. Surgical palliation of respiratory insufficiency secondary to massive exuberant polyostotic fibrous dysplasia of the ribs. Ann Thorac Surg. Feb 1985;39(2):185-7.

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Keywords

fibrous dysplasia, Lichtenstein-Jaffe's disease, Lichtenstein-Jaffe disease, McCune-Albright's disease, McCune-Albright disease, fibrous osteodystrophy, osteodystrophia fibrosa, osteitis fibrosa disseminata, monostotic form, polyostotic form, craniofacial form, cherubism

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Contributor Information and Disclosures

Author

Mahesh Kumar Neelala Anand, MBBS, DNB, FRCR, Clinical Director, Consultant Radiologist, Department of Radiology, Pennine Acute Hospitals NHS Trust, Manchester, UK
Mahesh Kumar Neelala Anand, MBBS, DNB, FRCR is a member of the following medical societies: British Medical Association, British Society of Interventional Radiology, Radiological Society of North America, and Royal College of Radiologists
Disclosure: Nothing to disclose.

Medical Editor

Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Michael A Bruno, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Society of Nuclear Medicine, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Murali Sundaram, MBBS, FRCR, FACR, Consulting Staff, Department of Diagnostic Radiology, The Cleveland Clinic Foundation
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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