9243/3 Dedifferentiated chondrosarcoma


ICD-O-3 topography code: C40-C41
ICD10: C40-C41

Dedifferentiated chondrosarcoma is an aggressive variant of chondrosarcoma, containing two clearly defined components, a well differentiated cartilage tumour, either an enchondroma or a low grade chondrosarcoma, and a high grade noncartilaginous sarcoma. The most commonly affected sites are pelvis, femur and humerus. The tumour usually produces an ill defined, lytic, intraosseous lesion, often associated with cortical perforation and extraosseous extension. The lobulated low grade cartilaginous component is usually located centrally, while the higher grade component is predominantly extraosseous.
Dedifferentiated chondrosarcoma makes up 10% of all reported chondrosarcomas. The mean age of presentation is between 50 and 60 years. Despite aggressive therapy, approximately 90% of patients die within two years 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