Bone & joints
Other nonneoplastic
Paget disease
Author: Nat Pernick, M.D.
Topic Completed: 1 August 2013
Revised: 27 December 2018
Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.
PubMed Search: Paget disease of bone[title]
Table of Contents
Definition / general | Diagnosis | Radiology description | Radiology images | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosisCite this page: Pernick N Paget disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonepagets.html. Accessed June 19th, 2021.
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 Dr. Mark R. Wick
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Pseudotumoral (proliferative) type, Xray
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 Dr. Mark R. Wick
Paget disease of bone
Proliferative phase
Positive stains
- Reticulin (highlights disorganization of lamellar bone)
Differential diagnosis
Of cement lines:
- Chronic osteomyelitis
- Polyostotic fibrous dysplasia: cortical bone has eccentric atrophy
- Radiation therapy
- Reactive bone adjacent to carcinoma
