Two Cases of Primary Malignant Fibrous Histiocytoma of the Liver: Immunohistochemical Expression of Ezrin and Its Relationship with Prognosis

Abstract

Malignant fibrous histiocytoma (MFH) as soft tissue sarcoma would not be especially noteworthy, but primary hepatic MFH reports are extremely rare. Herein, we report ezrin expression in tumor tissues from two primary hepatic MFH cases with different prognoses. Cases 1 and 2 were both women, ages 45 and 70 years, respectively. Case 1 had an 11×10 cm liver tumor in segment (S) 3, and case 2 had two liver tumors, 12×8 cm in S5 and 10×7 cm in S8. Neither had any other systemic tumors. Cases 1 and 2 survived for two year and ten months and for eight and a half months, respectively, after the initial tumor resection. Microscopically, the tumors of these two cases were similar and showed proliferation of atypical cells, including spindle, pleomorphic and multi-nucleated giant cells arranged in storiform, sheet and/or fascicle patterns, with scattered foci of inflammatory cells, indicating MFH. Ezrin expression in tumor tissue from case 1 was sparse, whereas that of case 2 showed strong ezrin expression in many tumor cells. The present results indicate ezrin immunoreactivity in primary hepatic MFH to correlate possible with prognosis, which is consistent with reports on some other types of malignancies.

Clinical characteristics of the primary hepatic malignant fibrous histiocytoma in China: case report and review of the literature

“…Primary hepatic MFH is exceedingly rare, especially in comparison with MFH of other sites, or with the more common primary malignancies of the liver [ 2 , 33 ]. Since its original description in 1985[ 5 ], to date, only 45 cases have been described in the English literature, including recent reports by Li et al [ 27 ], Sugitani M et al [ 28 ], Kim HS et al [ 29 ], Caldeira A et al [ 30 ] and Cong Z et al [ 31 ]. Among the 45 cases, 8 cases were from China [ 24 - 26 , 31 ].…”

“…So, early diagnosis and effective treatment might be an effective method. Furthermore, repeated surgery after recurrence might be also effective [ 15 , 28 ]. Besides, consistent with previous studies, our study also suggests that adjunctive therapies, such as chemotherapy chemoembolization or radiation therapy, are not effective treatment for this rare tumor.…”

Abdominal bleeding due to ruptured primary hepatic malignant fibrous histiocytoma

“…Some efforts have to be done to determine clinicopathological factors related to prognosis in hepato-MFH suffering patients, such as high Bcl-2 expression, high proliferation index, vascular invasion, bone morphogenic protein-2, without adequate credibleness. However, the expression of ezrin protein in pleomorphic MFH is related to poor survival [6,17].…”

“…The vast majority of the patients have very limited survival rate. Prognostic factors for the patient survival are the "profile" of the tumor, which corresponds to the tumor size, grade, depth, sufficiency of primary excision, metastasis and the histopathological grade [6,13,18]. Regarding the adjunctive therapies, i.e.…”

“…It is a relatively uncommon entity in the posterior retroperitoneum, the abdominal cavity, or other sites such as the skin and the head. Primary MFH of the liver is rare accounting for less than 1% of primary hepatic malignancies according to Liver Cancer Study Group of Japan and since the first report in the literature in 1985, a limited number of cases have been published [1][2][3][4][5][6][7][8][9][10][11][12][13]. The clinical presentation of primary hepatic MFH miscellaneous and nonspecific varies as there are asymptomatic cases while others present with symptoms which are common in a variety of diseases [8,13].…”