IS Case 559: Paget disease
Imaging Sciences URMC 2010
Publication Date: 2011-11-16
Patient is an 81-year-old female involved in a motor vehicle accident. She was an unrestrained passenger and was found to have an open left tibial shaft fracture and a closed left acetabular fracture.
Paget disease is a common disease of unknown etiology. Involvement before age 40 is unusual; after age 40 Paget disease can be seen in up to 3% of the population. Men are affected twice as commonly as women. Nearly any bone can be affected, although involvement of the skull, spine pelvis, femur, tibia, humerus, or scapula is typical. Three stages have been described: an early phase with osteolysis from osteoclastic bone resorption, a second phase with mixed osteolytic/osteoblastic activity with a coarsened and thickened trabecular pattern and cortex, and a third blastic phase with osteosclerosis. Monostotic or polyostotic patterns are seen, but diffuse involvement is less common.
The lesions (as in this patient) are seldom painful. Some estimate that 90% of lesions remain unrecognized. Involvement of the skull, particularly the skull base, and of the spine may be associated with neurological disturbances. Long bones may develop pathological fractures.
Histologically, there is intense osteoclastic resorption of normal bone by giant multinucleated cells. Osteoblasts produce exuberant hypervascular new bone. There is distortion and disruption of the organized bony matrix.
TOP 4 DIFFERENTIAL DIAGNOSIS:
- Whitehouse RW. Paget's disease of bone. Semin Musculoskelet Radiol. 2002 Dec;6(4):313-22. PMID: 12541188
- Mirra JM, Brien EW, Tehranzadeh J. Paget's disease of bone: review with emphasis on radiologic features, Part I. Skeletal Radiol. 1995 Apr;24(3):163-71. PMID: 7610408
- Diel J, Ortiz O, Losada RA, Price DB, Hayt MW, Katz DS. The sacrum: pathologic spectrum, multimodality imaging, and subspecialty approach. Radiographics. 2001 Jan-Feb;21(1):83-104. PMID: 11158646