Classification of pleomorphic sarcomas: where are we now?

Until a decade ago, so-called pleomorphic and storiform malignant fibrous histiocytoma (MFH) represented the most frequently diagnosed sarcoma, accounting for approximately 40% of adult mesenchymal malignancies. However, the latest World Health Organization classification of soft tissue tumours considers MFH a synonym for undifferentiated pleomorphic sarcoma. Historically, the term MFH was introduced in the medical literature in 1963 by Ozzello, O'Brien and Stout, on the basis of the acquisition of phagocytic properties observed in cultured fibroblasts. The existence of MFH as a well-defined clinicopathological entity became rapidly very popular and by the mid 1980s MFH represented the most common sarcoma in adults. With the advent of electron microscopy, immunohistochemistry and molecular genetics, it became clear that the so-called 'facultative fibroblast' theory had no scientific grounds and, in 1992, a milestone paper eventually brought attention to the concept that MFH merely represented a morphological pattern shared by a wide variety of poorly differentiated malignant neoplasms, which include specific subtypes of pleomorphic sarcomas. Currently, accurate subclassification of pleomorphic sarcomas is mandatory as it enables recognition of non-sarcomatous lesions as well as pleomorphic neoplasms not associated with aggressive behaviour. Furthermore, as myogenic differentiation predicts aggressive clinical behaviour among pleomorphic sarcomas, precise histotyping allows prognostic stratification of patients.