8823/0 Desmoplastic fibroma


Definitions

Skin
ICD-O-3 topography code: C44

Sclerotic fibroma is a rare, benign soft tissue tumour composed of eosinophilic collagen bundles arranged in a storiform pattern with minimal cellular atypia. It is typically a small (<1cm diameter) translucent, white nodule and occurs in both sexes at any age. Sites of involvement include the face, upper and lower extremities and trunk.
Multiple tumours are typical of the inherited Cowden syndrome .



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

Sclerotic (desmoplastic) fibroma is a rare, benign osteolytic tumour histologically composed of spindle cells with minimal cytological atypia and abundant collagen production. It most frequently affects the mandible. Tumours are usually firm and well delineated. Intralesional trabeculation is frequent. Desmoplastic fibromas are locally aggressive; larger lesions may extend beyond the periosteum into soft tissue.
Desmoplastic fibroma is a very rare bone tumour. It mainly affects adolescents and young adults. Recurrence rates following curettage and resection are 72% and 17%, respectively 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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