Primary Hepatic Malignant Fibrous Histiocytoma

Abstract

Primary hepatic malignant fibrous histiocytoma (MFH) is a rare entity with ill-defined clinicopathologic characteristics. We present the largest series to date (7 cases) of primary hepatic MFH, a systematic review of 27 cases published in the English literature, and an analysis of prognostic factors including ezrin expression. The 2 men and 5 women in our series were 34 to 80 years old (average 61 y) and presented with abdominal pain (6/7), systemic symptoms of low-grade fever, malaise, anorexia and weight loss (4/7), and spiking fever (1/7). One case was an incidental finding. Computed tomography and ultrasound revealed a well-demarcated solitary heterogeneous low-density mass ranging from 5.5 to 20 cm (average 12 cm) in largest dimension (5/7), or multiple small nodules up to 2 cm in diameter (1/7) or a 10 cm multiloculated cystic lesion owing to extensive necrosis (1/7). Histologically, a storiform-pleomorphic pattern with variable degrees of necrosis predominated (6/7). A myxoid pattern was seen in only 1 case and this case lacked necrosis. Lymphoplasmacytic infiltration was seen in 3/7 of the cases. Tumors were grade I (1/7), grade II (2/7), grade III (4/7), stage pT1 (4/7), pT2 (1/7), pT4 (1/7), and pT4, cM1 (1/7) at diagnosis. Three patients with tumor cell ezrin immunoreactivity score >or=1 died of tumor within 1 year. Two patients with tumor cell ezrin immunoreactivity score <1 survived >4 years after surgery. Combined analysis of our series and previously reported cases suggest that primary hepatic MFH possess certain characteristic clinicopathologic features that may help in making the diagnosis and in differentiating it from other more common neoplasms in the liver. The prognosis of hepatic MFH depends primarily on tumor size and stage at the time of diagnosis. Ezrin expression in tumor cells can provide additional prognostic information and may be a potential target for new adjuvant therapies.

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

“…In the previous study in the English literature, patients were all older than 34, whereas in our study it is found that there are also 4 patients younger than 15 years old, including 1 boy and 3 girls, suggesting that the primary hepatic MFH could also occur among young persons. In addition, in a recent study of 34 cases, Li and colleagues showed that there was no sex predilection in the hepatic MFH[27], while in our study, 50 of 76 patients in China were men, with a male to female ratio of 1.9:1, suggesting that there might be a slight male predominance for the incidence of this rare malignant tumor. Certainly, these results still waits further confirmed in the future study.…”

“…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].…”

Primary hepatic malignant fibrous histiocytoma combined with invasion of inferior vena cava

“…Meanwhile, CD-68, KP-1, and α1-antichymotrypsin were positive, conforming to the feature of MFH. [4,9,10] Thus, MFH with margin negative was confirmed pathologically.…”

“…[4,9–10] However, the histopathologic concept of MFH including storiform-pleomorphic, myxoid, inflammatory, giant cell, and angiomatoid variants had been eliminated; concomitantly World Health Organization denominated most of the MFH as undifferentiated pleomorphic sarcomas in 2002. [17] …”

A case of primary gastric undifferentiated high-grade pleomorphic sarcoma diagnosed with chief complaint of fever: a case report and literature review

“…However, as the fever and CRP level dramatically improved after the surgery, the gastric tumors were clearly related to the main complaints. There are reports about cases of UPS and MFH presenting initially with fever [21–23], and reports about cases of MFH producing actively cytokines such as G-CSF [24–27]. But.…”