Malignant fibrous histiocytoma of visceral organs: clinicopathologic features and diagnostic value of ezrin and HMG-CoA reductase

Malignant fibrous histiocytoma (MFH) of the breast and visceral organs is extremely rare. There is an incomplete understanding of the clinical pathology of the primary MFH originating from the breast and visceral organs, especially in comparison with other soft tissue sarcomas. As a consequence we searched and analyzed the clinical and pathological records of all the nine patients with diagnosed breast and visceral MFH in our hospital. Immunohistochemical staining was performed for ezrin and HMG-CoA reductase in these MFH cases and relevant mesenchymal sarcomas. The 9 MFH cases presented with nonspecific symptoms and imaging manifestations. 6 cases were classified as storiform-pleomorphic MFH, 2 cases as inflammatory MFH, and the remaining 1 case as giant cell MFH. The results showed that ezrin expression, as well as HMG-CoA reductase expression, was significantly stronger in MFH cases than other non-MFH sarcomas. Poor prognosis seemed to be associated with younger age. Certain characteristics and clinicopathologic features can help us making the diagnosis of MFH. In conclusion, our study provided the potential value of ezrin and HMG-CoA reductase for diagnosis and differential diagnosis of MFH located in the breast and visceral organs. More accurate prognostic information of this rare disease needed to be further investigated.