1278
A rare nonhereditary disorder associated with multiple enchondromas, which are benign cartilaginous tumors in the bone.
Prevalence 1:100,000.
Asymmetrical involvement of the extremities, with one side being involved, or predominantly so.
Discrepancies in leg length or bowing deformities are caused by enchondromas during childhood and adolescence.
Deformities often required surgical correction.
Enchondromas are located in small tubular bones.
Hand and foot function may be disabled depending upon the severity of enlargement and deformities that occur.
Malignant transformation to Congo sarcomas occurs in the range of 5-50% of cases.
Gliomas, acute myelogenous leukemia, and juvenile granulocytic cell tumors may be associated.