" /> Enchondromatosis, multiple, ollier type - CISMeF





Preferred Label : Enchondromatosis, multiple, ollier type;

Type : Phenotype or locus, molecular basis unknown;

Alternative titles and symbols : DYSCHONDROPLASIA; Ollier disease; OSTEOCHONDROMATOSIS;

Description : Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis. When hemangiomata are associated, the condition is known as Maffucci syndrome (614569). Clinical problems caused by enchondromas include skeletal deformity and the potential for malignant change to osteosarcoma (Schwartz et al., 1987). - Classification of the Enchondromatoses In their classification of the enchondromatoses, Spranger et al. (1978) called Ollier disease and Maffucci syndrome types I and II enchondromatosis, respectively; metachondromatosis (156250), type III; and spondyloenchondrodysplasia, also known as spondyloenchondrodysplasia (271550), type IV; enchondromatosis with irregular vertebral lesions, type V; and generalized enchondromatosis, type VI. Halal and Azouz (1991) added 3 tentative categories to the 6 in the classification of Spranger et al. (1978). Pansuriya et al. (2010) suggested a new classification of enchondromatosis (multiple enchondromas).;

Inheritance : ? Autosomal dominant form;

Prefixed ID : %166000;

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27/07/2025


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