principles management

Multiple Hereditary Exostosis

AKA

 

Diaphyseal Aclasis / Multiple Osteochondromas

 

Definition

 

Heritable skeletal dysplasia

 

Epidemiology

 

AD with variable penetrance (96%)

 

Malignant Transformation

 

Incidence of malignant transformation much higher 

- 10% overall

- 1 % / year

 

Chondrosarcoma (CS) > Osteosarcoma (OS)