Paget disease CHORUS

Description: disease of unknown etiology involving destruction and reparation
* age> 40y; M:F 2:1
* skull
o osteoporosis circumscripta (outer table destroyed only)
o "cotton wool" appearance
o increased hat size!
* spine
o monostotic vertebral involvment usually
* pelvis
o affected in 2/3 cases
o consists of cortical thickening
o enlargement of pubis/ischium
o coarse trabecular pattern
* extremities
o "blade of grass" appearance
* elevation of alk phos (up to 20X), normal calcium, normal phosphorus
* complications:
o path fractures in 8% cases (usu. femur = "shepard's crook")
o malignant degeneration (usu. osteosarcoma-50%, fibrosarcoma-25%)