9220/1 Chondromatosis, NOS


Definitions

Bone
ICD-O-3 topography code: C40-C41

Multiple chondromatosis is a multifocal benign neoplasm arising from soft tissue or bone. It is characterized by the presence of chondrocytes and is composed of hyaline cartilage. Any part of a tubular bone can be affected; the most common site is the metaphysis, in particular that of the hand.
Failure of normal enchondral ossification during development and the production of cartilaginous masses (enchondromas) in the metaphysis and adjacent regions of the shafts and flat bones (Ollier disease ) lead to bone deformities of varying degrees, ranging from a few small lesions in the hand or foot to multiple, widely distributed, sizeable lesions and marked skeletal deformation. Pathological fractures, limb length discrepancies, and bowing deformities are common in severe cases.
Maffucci syndrome combines the features of Ollier disease associated with angioma of the soft tissue.
Multiple chondromatosis is rare; tumours appear in childhood and patients usually present during the first two decades of life without sex predilection. Malignant progression occurs in 25-30% of cases, usually to low grade chondrosarcoma 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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