8830/0 Xanthofibroma



Definitions

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

Benign fibrous histiocytoma (BFH) is a benign neoplasm composed of fibroblastic spindle cells in a whorled storiform pattern. It is characterized by the presence of foam cells, inflammatory cells, hemosiderin deposition and stromal haemorrhage.

Deep benign fibrous histiocytoma develops within subcutaneous or deep soft tissues and in parenchymal organs. Most commonly affected sites are the lower limbs and the head and neck region. The lesions are generally well circumscribed and pseudo-encapsulated.
Deep benign fibrous histiocytoma can develop at any age, but most patients are adults >25 years old. Males are more frequently affected. The tumour may recur locally but does not metastasize 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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Bone
ICD-O-3 topography code: C40-C41

Benign fibrous histiocytoma (BFH) is a benign neoplasm composed of fibroblastic spindle cells in a whorled storiform pattern. It is characterized by the presence of foam cells, inflammatory cells, hemosiderin deposition and stromal haemorrhage.

Approximately 40% of benign fibrous histiocytoma of bone involve the long bones (most frequently femur and tibia), where the tumour is centred in the epiphysis or diaphysis. The lesion is sharply demarcated from the adjacent uninvolved bone. BFH is a well defined medullary defect without matrix formation; internal trabeculation or pseudoseptations may be present. Approximately two-thirds of the lesions have sclerotic margins; soft tissue extension does not occur. Typically, tumours are firm, <3 cm in diameter. Scattered among the spindle-shaped fibroblasts are multinucleated, osteoclast-type giant cells.
Benign fibrous histiocytoma of the bone is rare and occurs in all age groups. The prognosis is excellent; surgical curettage/resection is usually curative
2
 
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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