Encondromatosis múltiple [Sindrome de Ollier] / Multiple enchondromatosis

ABSTRACT

Enchondromatosis or Ollier syndrome is defined by the presence of multiple enchondromas with an asymmetrical distribution of low prevalence. Enchondromas are common intra osseous benign cartilage tumors cartilaginous which develop to close proximity growth plate cartilage. Cartilage injuries can be very variable in terms of size, number, location, evolution of enchondroma, age of onset and of diagnosis, requirement for surgery. Clinical problems caused by enchondromas include skeletal malformations, an asymetrics hortening of extremity with limping, and potential risk of malignant change to chondrosarcoma. The condition in which multiple enchondromatosis is associated to soft tissue hemangiomas is known as Maffucci syndrome. So far, both Ollier disease and Maffucci syndrome have occurred only in isolated cases. It has not been established if the disease depends on a single gene or combination of several mutations. The diagnosis is based on clinical and radiological conventional analysis. Histological analysis has a limited role and is used if malignancy is suspected. There is no medical treatment for enchondromatosis. Surgical treatment is recommended only in case of complications. Though, it is difficult to establish a prognosis for Ollier disease, it is found that the early onset forms are usually more severe.