9184/3 Osteosarcoma in Paget disease of bone


Definitions

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

Paget osteosarcomas result from a sarcomatous transformation of pre-existing Paget disease of the bone. These bone tumours are high grade lesions, usually have a poor prognosis and occur in up tp 1% of patents with Paget disease 1
 
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



. Large bones of the limbs and the skull are preferentially affected. Multiple Paget osteosarcomas may occur.