9183/3 Telangiectatic osteosarcoma


Definitions

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

Telangietactic osteosarcoma is an osteosarcoma histologically characterized by a cystic architecture with large, blood-filled spaces ("bags of blood"). The cystic spaces are lined by benign-looking giant cells. The septa are cellular and contain highly malignant atypical mononuclear tumour cells. The amount of osteoid varies, but usually fine, lacelike osteoid is observed in minimal amount 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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Telangiectatic osteosarcoma usually arises in the metaphysis of long tubular bones; extension into the epyphysis and into soft tissues is typical. The lesions show purely lytic, large bone destruction without distinct surrounding bone sclerosis.
Telangiectatic osteosarcoma is rare. It most frequently occurs in the second decade of life and has a male predominance (1.5:1). The prognosis is similar to that of conventional osteosarcoma
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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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