| Background: Malignant fibrous histiocytoma (MFH) has the potential of occurringalmost everywhere, owning to its mesenchymal origin. It has been recognized to themost common sarcoma in soft tissue, however, primary MFH of liver is extremely rareand characterized clinically by a poor prognosis. The histopathologic examination revealed a typical MFH pattem composed of spindle-shaped, pleomorphic, malignantcells in a storiform pattern associated with histiocyte-like cells and bizarre giant cells.The objective of this study is to investigate the clinicopathological and prognosticfeatures of the MFH. To analyze the clinical manifestations, so the diagnosis of MFHmay be made and treatment instituted as early as possible.Methods: We had systematically searched for either published on the MFH all over theworld from January 1985 to December 2005, by electronic and manual retrieving, andother means such as related experts, etc. The Kaplan-Meier method was used toestimate clinicopathological features and patient survival, and differences in theprobabilities were evaluated using the log-rank test. Cox's proportional hazards modelwas performed to identify factors that were independently associated with the prognosis.Statistical analysis was carried out using SPSS 13.0. In the same time, a total of 3 ofMFH patients undergoing surgical resection at Tianjin Cancer Hospital between 1954and 2005 were included in this study. Routine pathological and immunohistochemcaldata were studied in the 3 cases of MFH.Results: From January 1985 to December 2005, Totally 26 pieces of literatures and 33cases were retrieved. There were 18 men and 15 women among the 33 patients withages ranging from 27 to 87 years. The average age of the patients was 56.15 years, andthe male-to-female ratio was 1.2:1. In most of these patients, the tumors occurredmainly at the right lobe. Almost all the patients had a normal laboratory work-up andthe negative tumoral markers such as Alpha-fetoprotein. The average maximal tumordiameter was 11.68cm (range, 6-23.5cm). Among the 33 patients, the metastases ofprimary liver MFH appeared in 5 patients, and recurrence in 2 patients after operation,only 5 patients survived following surgery without any evidence of local recurrence ordistant metastasis.In the literature, 31 cases described the survival status, and the median follow-upwas 6.66 months (range, 0.2-64 months). The one, two, three and five year over-allsurvival rates of the patients were 18.18%, 15.15%, 9.09% and 3.03%. A total of 21patients were died. The exploratory analysis was used to identify risk factors forsurvival in patients with MFH. Factors including the patient's age, sex, tumor location, serum AFP level, type of histology, operation, chemotherapy, the maximal tumordiameter, metastases were analyzed for their association with survival. Among them, themaximal tumor diameter and tumor location were factors associated with survival inunivariate analysis. In multivariate analysis, only the maximal tumor diameter were thedeterminants for survival in patients with MFH. However, there were no statisticallysignificant impacts on recurrence for serum AFP and the other tumor markers.Routine pathological and immunohistochemcal data were studied in the 3 cases ofMFH at Tianjin Cancer Hospital. The histopathologic examination revealed a typicalMFH pattern composed of spindle-shaped, pleomorphic, malignant cells in a storiformpattern associated with histiocyte-like cells and bizarre giant cells. Most of the MFHswere pleomorphic; only one inflammatory type and two myxoid have been reported.Immunohistochemical positivity for Vimentin,α-ACT,α-AT in almost all the cases.Conclusions: Malignant fibrous histiocytoma (MFH) has the potential of occurringalmost everywhere, owning to its mesenchymal origin. It has been recognized to themost common sarcoma in soft tissue, however, primary MFH of liver is extremely rareand characterized clinically by a poor prognosis. Although primary MFH of the liver isan extremely rare aggressive neoplasm with a grim prognosis, it should be considered inthe differential diagnosis of large lesions and sarcomas of the liver. Particular attentionmust be paid to those large exophytic lesions whose anatomical origin is uncertain. |
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