Bone & joints

Other nonneoplastic

Paget disease



Last author update: 1 August 2013
Last staff update: 31 October 2022 (update in progress)

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PubMed Search: Paget disease of bone[title]

Nat Pernick, M.D.
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Cite this page: Pernick N Paget disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonepagets.html. Accessed December 3rd, 2022.
Definition / general
  • Also called osteitis deformans
  • "Collage of matrix madness," with furious osteoclastic bone resorption (osteolytic phase), hectic bone formation (mixed osteoclastic/osteoblastic phase), burnt out osteosclerotic stage (gain in bone mass, but bone is disordered)
  • 90% are over age 55, rare before age 40
  • More common in whites in U.S., England (3% at autopsy), France, Austria, Germany, Australia, New Zealand (5 - 11%); rare in blacks, Scandinavia, China, Japan, Africa
  • May be due to slow virus infection of paramyxovirus, similar to subacute sclerosis leukoencephalitis (virus identified in osteoblasts)
  • Sites: 85% of presenting patients are polyostotic (pelvis, spine, skull), 15% monostotic (tibia, ilium, femur, skull, vertebrae, humerus); rare in hands / feet, ribs, fibula [note: polyostotic patients are more likely to seek medical attention; monostotic patients are often asymptomatic, but actually are more common]
  • Symptoms: often mild; localized pain due to microfractures and nerve compression; may have secondary osteoarthritis due to weak femur or tibia, chalk-like fractures of tibia, fibula, femur, spinal cord injuries due to spinal fractures; also associated with high output congestive heart failure due to shunting of blood through warm skin (bone is hypervascular and hot)
  • Leontiasis ossea: cranium too heavy to lift
  • Platybasia: invagination of base of skull due to weak bone, compression of posterior fossa structures
  • Associated neoplasms: sarcoma (5% with severe polyostotic disease), giant cell tumor, giant cell granuloma
  • NOT related to Paget disease of breast or vulva, although both discovered by Sir James Paget, one of the founders of pathology
Diagnosis
  • Xray, elevated serum alkaline phosphatase and urinary hydroxyproline (normal serum calcium and phosphorus)
Radiology description
  • Early - radiolucency
  • Late - increased bone density, increased microfractures, loss of distinction between cortex and medulla; may have sharp demarcation between normal and affected bone; may extend into soft tissue if florid disease
Radiology images

Contributed by Mark R. Wick, M.D. and AFIP images
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Pseudotumoral (proliferative) type, Xray

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Sarcoma arising in Paget disease

Treatment
  • Calcitonin, diphosphonates
Microscopic (histologic) description
  • Diagnostic features are increased osteoclastic and osteoblastic activity with supportive radiologic findings
  • Acute: primarily woven bone; focal mosaic pattern of lamellar bone, resembles jigsaw puzzle with prominent irregular cement lines; osteoclasts present at surface of bone but don't tunnel; in osteolytic phase, osteoclasts may have up to 100 nuclei
  • Chronic: thick trabeculae and thicker bones; fine fibrosis of marrow
Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.
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Paget disease of bone

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Proliferative phase

Positive stains
  • Reticulin (highlights disorganization of lamellar bone)
Differential diagnosis
Of cement lines:
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