Classification of pleomorphic sarcomas: where are we now?

摘要: 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, latest World Health Organization classification soft tissue tumours considers MFH synonym undifferentiated sarcoma. Historically, term was introduced in medical literature 1963 by Ozzello, O'Brien Stout, on basis acquisition phagocytic properties observed cultured fibroblasts. The existence as well-defined clinicopathological entity became rapidly very popular mid 1980s common sarcoma adults. With advent electron microscopy, immunohistochemistry molecular genetics, it clear that "facultative fibroblast" theory had no scientific grounds and, 1992, milestone paper eventually brought attention to concept merely morphological pattern shared wide variety poorly differentiated neoplasms, which include specific subtypes sarcomas. Currently, accurate subclassification sarcomas is mandatory enables recognition non-sarcomatous lesions well neoplasms not associated with aggressive behaviour. Furthermore, myogenic differentiation predicts clinical behaviour among sarcomas, precise histotyping allows prognostic stratification patients.