Paget's disease of the bone

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Paget's disease of the bone is an uncommon benign disorder of bone with distinctive radiographic findings.

Paget's disease of the bone
Diagnosis in short

Paget's disease of the bone. H&E stain.

LM bone matrix has jigsaw-puzzle like pattern (jigsaw-puzzle pieces each ~ 100-500 micrometres in size in largest dimension), increased osteoclast activity
Site bone

Associated Dx fracture of bone, secondary osteosarcoma
Signs +/-deafness
Symptoms +/-bone pain
Blood work elevated ALP
Radiology bony deformity, "sunburst" skull
Prognosis benign

It is unrelated to the Paget's disease of the breast and extramammary Paget's disease.

General

Clinical

Presentation:[1]

  • Fracture.
  • Bone pain.
  • Bony deformity.
  • Deafness.
  • Incidental finding - radiologic or biochemical.

Serology:

  • Elevated ALP.

Clinical features - mnemonic PANICS:[7]

  • Pain (bone).
  • Arthralgia and ALP elevated.
  • Nerve compression - deafness.
  • Increased bone density.
  • Cardiac failure (high output) - due to AVM formation in bone.
  • Sunburst skull on X-ray.

Stage

Classically divided into three phases:[8][9]

  1. Lytic (predominantly osteoclasts).
  2. Mixed lytic (osteoclastic) and blastic (osteoblastic).
  3. Sclerotic (burned-out).

Microscopic

Features:[8]

  • Early Paget disease is cellular and fibro-osseous (bone and fibrous components)
  • Mature Paget disease is sclerotic with thickened bone trabeculae.
  • Bone matrix has jigsaw-puzzle like pattern.
    • Jigsaw-puzzle pieces each ~ 100-500 micrometres in size (largest dimension).
  • Increased osteoclast activity.
    • Osteoclast = macrophage that reabsorbs bone matrix.

Images

See also

References

  1. 1.0 1.1 1.2 Britton, C.; Walsh, J. (Mar 2012). "Paget disease of bone - an update.". Aust Fam Physician 41 (3): 100-3. PMID 22396921.
  2. Hoch, B.; Hermann, G.; Klein, MJ.; Abdelwahab, IF.; Springfield, D. (Oct 2007). "Giant cell tumor complicating Paget disease of long bone.". Skeletal Radiol 36 (10): 973-8. doi:10.1007/s00256-007-0310-x. PMID 17437100.
  3. Karakida, K.; Ota, Y.; Aoki, T.; Akamatsu, T.; Kajiwara, H.; Hirabayashi, K. (Sep 2010). "Multiple giant cell tumors in maxilla and skull complicating Paget's disease of bone.". Tokai J Exp Clin Med 35 (3): 112-7. PMID 21319038.
  4. Online 'Mendelian Inheritance in Man' (OMIM) 602080
  5. Online 'Mendelian Inheritance in Man' (OMIM) 603499
  6. Online 'Mendelian Inheritance in Man' (OMIM) 606263
  7. URL: http://www.medicalgeek.com/orthopedics/2743-orthopedics-mnemonics.html. Accessed on: 30 April 2012.
  8. 8.0 8.1 URL: http://emedicine.medscape.com/article/311688-overview. Accessed on: 25 December 2010.
  9. URL: http://radiopaedia.org/articles/paget-disease-of-bone-1. Accessed on: 25 December 2010.