8835/1 Plexiform fibrohistiocytic tumor


Definitions

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

Plexiform fibrohistiocytic tumour (PFT) is a slowly growing intermediate cutaneous neoplasm. Most frequently affected sites are the upper extremities, in particular hands and wrists, followed by the lower extremities. The tumours are small (rarely >3cm), multinodular and poorly circumscribed; histologically, they are characterized by the presence of multinucleated giant cells, mononuclear histiocyte-like cells, and spindle fibroblast-like cells arranged in a plexiform pattern. The nodules arise in subcutaneous tissue and deep dermis, but extension into skeletal muscle is common.
PFT mainly affects children and young adults and shows a predilection for females. Local recurrence is frequent, but metastases develop rarely 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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