9193/3 Periosteal osteosarcoma


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

Periosteal osteosarcoma is an intermediate grade chondroblastic osteosarcomaarising on the surface of bone. The tumour chiefly affects the diaphysis or diaphyseal-metaphyseal area of the long bones. Calcified spiculations perpendicular to the cortex give the tumour a sunburst appearance. The cartilaginous component predominates within the lesion. The cortex commonly appears thickened as a result of the production of a heavily ossified matrix, while the periphery tends to be uncalcified or only minimally calcified. The advancing margin is usually well demarcated by a capsule/pseudocapsule.
Periosteal osteosarcoma shows a peak incidence in the second and third decades of life, with slight male predominance. It is associated with a better prognosis than conventional osteosarcoma 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