9160/0 Giant cell angiofibroma



Definitions

Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

Giant cell angiofibroma (GCA) is a benign, slowly growing neoplasm predominantly arising in the orbital region and eyelids, the nasolacrimal duct and the lacrimal sac region. Extraorbital lesions are generally located subcutaneously. GCAs are well circumscribed, variably encapsulated and small (median 3cm; lesions arising in soft tissues may measure up to 10cm). Haemorrhage and/or cystic changes may be present. Histologically, the tumour is characterized by bland round to spindle shaped cells, multinucleated giant cells, angiectoid spaces, thick-walled blood vessels and a collagenous or myxoid stroma 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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Giant cell angiofibroma most often occurs in middle-aged adults (median age 45 years). Orbital GCA shows a predilection for men, whereas extraorbital lesions predominate in women. Complete excision is usually curative
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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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