Paget's disease of the bone

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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

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

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]

  • Lytic and mixed Paget disease are cellular and fibro-osseous (bone and fibrous components)
  • Sclerotic Paget disease has 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.