Table of ContentsDefinition / general
Cite this page: Polyostotic. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bonefibrousdysplasiapolyostotic.html. Accessed August 24th, 2019.
Definition / general
- 20% of cases, slightly younger than monostotic patients, may persist into adulthood
- Involvement of shoulder and pelvic girdles causes crippling deformities, fractures
- Sites: femur, skull, tibia; craniofacial involvement in 100% with extensive skeletal disease, 50% otherwise; usually unilateral
- Associated with progressive disease (recurring fractures, long bone deformities, facial deformities), worse if earlier age of onset; rare malignant transformation into sarcoma, more likely after radiation
- 50% have abnormal cutaneous pigmentation