The term 'fibrohistiocytic' tumour is a descriptive designation without histogenetic connotation for a group of heterogeneous lesions that share morphological features of histiocytes and fibroblasts on light microscopy. However, over the years it has become apparent that many so-called 'fibrohistiocytic' tumours are largely composed of relatively undifferentiated mesenchymal cells, but can also show areas of myofibroblastic differentiation. This review focuses on the clinical and histological features as well as differential diagnosis of so-called fibrohistiocytic tumours. Special emphasis is given to more recently described histological variants of fibrous histiocytoma, e.g. cellular, epithelioid, aneurysmal and atypical fibrous histiocytoma, to angiomatous and plexiform fibrous histocytoma (plexiform fibrohistiocytic tumour), lesions that are not true variants of fibrous histiocytomas but have erroneously been designated such, and to atypical fibroxanthoma. The literature on metastasizing fibrous histiocytoma is also reviewed.
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