eMedicine Specialties > Orthopedic Surgery > Neoplasms

Fibrous Dysplasia: Multimedia

Author: Bernardo Vargas, MD, Consulting Staff, Department of Orthopedic Surgery, Hôpital Universitaire de Geneva, Switzerland
Coauthor(s): Mark Clayer, MD, MBBS, FRACS, FAOrthA, Head of Musculoskeletal Tumor Service, Department of Orthopaedics and Trauma, Queen Elizabeth Hospital; Senior Visiting Medical Specialist, Royal Adelaide Hospital and Women's and Children's Hospital, Australia
Contributor Information and Disclosures

Updated: Aug 13, 2008

Multimedia

Intermediate-power view of typical histology of f...Media file 1: Intermediate-power view of typical histology of fibrous dysplasia. Note the bland fibrous stromal tissue with islands of disorganized, immature osteoid. A key feature is the absence of rimming osteoblasts around the osteoid. While not present in this slide, foci of cartilage also may occasionally be present.
Intermediate-power view of typical histology of f...

Intermediate-power view of typical histology of fibrous dysplasia. Note the bland fibrous stromal tissue with islands of disorganized, immature osteoid. A key feature is the absence of rimming osteoblasts around the osteoid. While not present in this slide, foci of cartilage also may occasionally be present.

Plain radiograph of a tibia in a patient who is s...Media file 2: Plain radiograph of a tibia in a patient who is skeletally mature, demonstrating expansion of the metaphysis and diaphysis, endosteal scalloping, and a ground-glass appearance of the matrix.
Plain radiograph of a tibia in a patient who is s...

Plain radiograph of a tibia in a patient who is skeletally mature, demonstrating expansion of the metaphysis and diaphysis, endosteal scalloping, and a ground-glass appearance of the matrix.

Technetium-99m methylene diphosphonate (MDP) bone...Media file 3: Technetium-99m methylene diphosphonate (MDP) bone scan demonstrating increased uptake in the tibia corresponding to the radiographic margins.
Technetium-99m methylene diphosphonate (MDP) bone...

Technetium-99m methylene diphosphonate (MDP) bone scan demonstrating increased uptake in the tibia corresponding to the radiographic margins.

CT scan of the tibia demonstrating expansion of t...Media file 4: CT scan of the tibia demonstrating expansion of the tibia due to an expanding intramedullary lesion.
CT scan of the tibia demonstrating expansion of t...

CT scan of the tibia demonstrating expansion of the tibia due to an expanding intramedullary lesion.

A T1-weighted MRI image demonstrating intermediat...Media file 5: A T1-weighted MRI image demonstrating intermediate signal intensity and no soft tissue component.
A T1-weighted MRI image demonstrating intermediat...

A T1-weighted MRI image demonstrating intermediate signal intensity and no soft tissue component.

A T2-weighted MRI image demonstrating increased s...Media file 6: A T2-weighted MRI image demonstrating increased signal intensity of the matrix of the lesion.
A T2-weighted MRI image demonstrating increased s...

A T2-weighted MRI image demonstrating increased signal intensity of the matrix of the lesion.

The metaplastic bone formed by fibrous dysplasia ...Media file 7: The metaplastic bone formed by fibrous dysplasia has the appearance of Chinese letters.
The metaplastic bone formed by fibrous dysplasia ...

The metaplastic bone formed by fibrous dysplasia has the appearance of Chinese letters.

More on Fibrous Dysplasia

Overview: Fibrous Dysplasia
Workup: Fibrous Dysplasia
Treatment: Fibrous Dysplasia
Follow-up: Fibrous Dysplasia
Multimedia: Fibrous Dysplasia
References

References

  1. Alman BA, Greel DA, Wolfe HJ. Activating mutations of Gs protein in monostotic fibrous lesions of bone. J Orthop Res. Mar 1996;14(2):311-5. [Medline].

  2. DiCaprio M. R., Enneking W. F. Fibrous Dysplasia. Pathophysiology, Evaluation, and Treatment. J Bone Joint Surg Am. 2005;87:1848-1864. [Medline].

  3. Marie P. Dysplasie fibreuse : aspects tissulaires, cellulaires et moléculaires. Revue du rhumatisme. 2003;7:681–686.

  4. Albright F, Butler AM, Hampton AO. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females. New Engl J Med. 1937;216:727-46.

  5. Fraser WD, Walsh CA, Birch MA. Parathyroid hormone-related protein in the aetiology of fibrous dysplasia of bone in the McCune Albright syndrome. Clin Endocrinol. 2000;53(5):621-8. [Medline].

  6. Parekh S, Donthineni-Rao R, Ricchetti E. Fibrous dysplasia. J Am Acad Orthop Surg. 2004;12:305-313. [Medline].

  7. Santos CT, Choo CT, Loh AH. Orbital fibrous dysplasia with soft tissue hamartoma--a variant of Mazabraud's syndrome. Orbit. 2008;27(3):207-9. [Medline].

  8. Chapurlat RD, Orcel P. Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol. Mar 2008;22(1):55-69. [Medline].

  9. Lichtenstein 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;33:777-816.

  10. Ippolito E. Bray E. W., Corsic A. et als. Natural history and treatment of fibrous dysplasia of bone:a multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society. J of Ped Orthop Part B. 2003,;12:155–177. [Medline].

  11. Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer. 1994;73(5):1411-24. [Medline].

  12. Yabut SM Jr, Kenan S, Sissons HA, Lewis MM. Malignant transformation of fibrous dysplasia. A case report and review of the literature. Clin Orthop. 1988;228:281-9. [Medline].

  13. Marie PJ, de Pollak C, Chanson P, Lomri A. Increased Proliferation of Osteoblastic CellsExpressing the Activating Gsa Mutation in Monostotic and Polyostotic Fibrous Dysplasia. Am J Pathol. 1997;150:1059-1069. [Medline].

  14. Guille JT, Kumar SJ, MacEwen GD. Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment. J Bone Joint Surg Am. May 1998;80(5):648-58. [Medline].

  15. Stanton RP, Diamond L. Surgical management of fibrous dysplasia in McCune-Albright syndrome. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:446-52. [Medline].

  16. Kumta SM, Leung PC, Griffith JF, et al. Vascularised bone grafting for fibrous dysplasia of the upper limb. J Bone Joint Surg Br. Apr 2000;82(3):409-12. [Medline].

  17. Chapurlat RD, Hugueny P, Delmas PD, Meunier PJ. Treatment of fibrous dysplasia of bone with intravenous pamidronate: long-term effectiveness and evaluation of predictors of response to treatment. Bone. 2004;Volume 35, Issue 1:235-242. [Medline].

  18. Liens D, Delmas PD, Meunier PJ:. Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet. 1994;343:953-954. [Medline].

  19. Zacharin M, O'Sullivan M. I. Intravenous pamidronate treatment of polyostotic fibrous dysplasia associated with the McCune Albright syndrome. J Pediatr. 2000;137(3):403-9. [Medline].

  20. DiMeglio LA. Bisphosphonate therapy for fibrous dysplasia. Pediatr Endocrinol Rev. Aug 2007;4 Suppl 4:440-5. [Medline].

  21. Lietman SA, Ding C, Levine MA. A. A highly sensitive polymerase chain reaction method detects activating mutations of the GNAS gene in peripheral blood cells in McCune-Albright syndrome or isolated fibrous dysplasia. J Bone Joint Surg Am. 2005;87:2489-2494. [Medline].

  22. Murray DJ, Edwards G, Mainprize JG, Antonyshyn O. Advanced technology in the management of fibrous dysplasia. J Plast Reconstr Aesthet Surg. Aug 2008;61(8):906-16. [Medline].

  23. Bridge JA, Rosenthal H, Sanger W. Desmoplastic fibroma arising in fibrous dysplasia. Clin Orthop. 1998;247:272-8. [Medline].

  24. McCune DJ:. Osteitis fibrosa cystica: the case of a nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism. Am J Dis Child. 1936;52:743-747.

Further Reading

Keywords

fibrous dysplasia, bone dysplasia, osteochondrodysplasia, Albright disease, bone disease, dysplasia, fibrous dysplasia of bone, fibrous dysplasia bone, cherubism, dysplastic disorder, connective tissue, fibroosseous tissue, lamellar bone, monostotic fibrous dysplasia, polyostotic fibrous dysplasia, precocious puberty, skin pigmentation, McCune-Albright syndrome, Mazabraud syndrome, McCune-Albright's syndrome, Mazabraud's syndrome, Albright's disease, Albright syndrome, Albright's syndrome

Contributor Information and Disclosures

Author

Bernardo Vargas, MD, Consulting Staff, Department of Orthopedic Surgery, Hôpital Universitaire de Geneva, Switzerland
Disclosure: Nothing to disclose.

Coauthor(s)

Mark Clayer, MD, MBBS, FRACS, FAOrthA, Head of Musculoskeletal Tumor Service, Department of Orthopaedics and Trauma, Queen Elizabeth Hospital; Senior Visiting Medical Specialist, Royal Adelaide Hospital and Women's and Children's Hospital, Australia
Mark Clayer, MD, MBBS, FRACS, FAOrthA is a member of the following medical societies: Australian Medical Association and Australian Orthopaedic Association
Disclosure: Orthopedics hyperguide Honoraria Independent contractor; Stryker Grant/research funds Employment

Medical Editor

Howard A Chansky, MD, Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center
Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Sean P Scully, MD, PhD, Professor, Department of Orthopedics, University of Miami
Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

 
 
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