Malignant Fibrous Histiocytoma of the Liver: A Case Report and Review of the Literature Youichi Akifuji, Ichiro Honjo, Syoushi Katayama, Seiichi Ishitobi, Juichi Ueki, Kiyoshi Kishi*, Kazuyoshi Kato* and Shu Nakamoto** Primary malignant fibrous histiocytoma (MFH) of the liver is reported in a 79-year-old man. The tumor, measuring 8.0 X 8.0 x 6.0cm, was located in the left lobe of the liver and consisted of spindle cells in a storiform pattern intermingled with bizarre giant cells. Immuno histochemically, most of the tumor cells expressed vimentin. Cytoplasmic immunoreactivity for Oi-antichymotrypsin was documented in the giant cells. However, epithelial expression could (Internal Medicine 31: 284-288, 1992) not be demonstrated. Key words: MFH, liver tumor, immunocytochemistry


alkaline phosphatase, and lactate dehydrogenase levels were normal. Tests for tf-fetoprotein, carcinoembryonic Malignant fibrous histiocytoma (MFH) is a common antigen, 19-9 carbohydrate antigenic determinant, pro sarcoma of the soft tissue, occurring usually in the ex tein induced by vitamin K absence II and hepatitis B tremities and less commonly in the retroperitoneal space, virus surface antigen were negative, but hepatitis C virus the abdominal cavity, or other sites such as the skin, and antibody was positive. Abdominal ultrasonic examin the head and neck region (1, 2). MFH of the liver is ation (Fig. la) revealed a hypoechoic mass localized in extremely rare, and only ll cases (3-13) have been the left lobe of the liver, which by enhanced computed reported to date. Here, a case of primary MFH is re tomography scan (Fig. lb) appeared to be a well ported, including examination of the excised liver. The circumscribed mass. There were no retroperitoneal literature is also reviewed. Case Report masses or adenopathy, and the pancreas was normal. A selective hepatic arteriogram (Fig. lc) demonstrated that A 79-year-old man was admitted to our hospital on the mass was hypovascular. Embolization (lipiodol) with February 20, 1990 with complaints of anorexia and general chemotherapy (Mitomycin C, 30 mg) was performed on fatigue. Four years previously at another hospital, he this mass, but the treatment was not effective. Based had been diagnosed as having liver dysfunction. On on these findings, a cholangiocarcinoma was strongly admission, the patient appeared somewhat emaciated. There was slight anemia, but no jaundice. Cardio suspected. A laparotomy was carried out on April 4, 1990. A hard mass that invaded the diaphragm was pulmonary examination was unremarkable. The liver palpated in the left lateral segment of the liver. The rest span was 8 cm on the right clavicular line. A hard tender of the intraabdominal examination was unremarkable. A mass (10cm in diameter) which was palpable in the left hepatectomy with partial resection of the diaphragm epigastrium, was displaced by respiration. The laboratory Pathological was then performed. Features The of the postoperative Tumor course was satis data on admission are listed in Table 1. Blood examin factory, but a recurrence was confirmed in the right lobe 34.9%; hemoglobin, 12.3g/dl; and leukocytes, 7,500/ ation revealed erythrocytes, 377 x 104/mm3; hematocrit, mm3. Total serum protein, bilirubin, transaminases, Macroscopicfindings of the liver and the scapula 5 months after the operation. From Department of Internal Medicine, * Surgery and ** Laboratory Medicine, Tottori Prefectural Central Hospital, 730 Ezu, Tottori Japan Received for publication November 21, 1990; Accepted for publication July 31, 1991 Reprint requests should be addressed to Youichi AKIFUJI, MD, Tottori Prefectural Central Hospital, 730 Ezu, Tottori 680, Japan 284



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MFHof the Liver The surgical specimen (Fig. 2a) consisted of a left lobe of the liver (ll.0 X ll.0 x 9.0cm). The cut surface of the specimen showed a single round tumor mass, measuring 8.0 X 8.0 X 6.0cm (Fig. 2b). The tumor was well circumscribed, of rubbery consistency, and gray in

color. Scalloped borders were located within the liver parenchyma. The tumor penetrated through the upper part of the liver capsule and extended into the diaphragm.

Table 1. Examination Data on Admission 377 x 104/m m 3

RBC Ht Hb W BC Pit T Z T D G O

34 .9% 12 .3 g/dl 7,500/m m ,3 27 .8 x 104/m m 3

TT TT .B il .B il OT PT

3.9 U 15.6 U 0 .4 m g /d l 0.2 m g /d l 17 U 9U 191 U 0.29 A pH 163 U 28 IU /L


T .P A lb O iG lob a2 p

7.4 g/dl 39.3 % 5.3 % 13.4 % 10.4 % 31.3%

r BU N Cr Na K a

24 m g /dl 1 .7 m g /d l 137 m E q /l 4 .6 m E q /l 103 m E q /l

C A 19-9 C EA A FP P IV K A II

5 .3 U /m 1.3 n g/m 3 .0 n g/m < 0 .07 A U /m

l l l l

10 .4 U

S-A m ylase T .C h oi T .G .

88 U 118 m g/dl 56 m g/dl


(- ) (+ ) (+ ) 29.5%

Microscop ic findings The tumor was composed of fibroblast-like spindle cells in a storiform pattern (Fig. 3a) intermingled with bizarre giant cells (Fig. 3b), and massive central necrosis with fibrosis was observed. The giant cells had single or multiple hyperchromatic and irregular nuclei and eosinophilic cytoplasm. The mitotic figures were infre quent. No cross-striations could be observed in the neoplastic cells. Special staining procedures demon strated a fine reticulin and/or collagenous mesh, and there were strands of collagen. The surrounding hepatic tissue did not show anyfindings evidence of cirrhosis. Immunohistochemical Most of the fibroblast-like cells, histiocyte-like cells, and giant cells were vimentin-positive (Fig. 3c). Several pleomorphic tumor cells, including giant cells, showed granular cytoplasm positive for ^-antichymotrypsin (Fig. 3d). Stains for lysozyme, keratin, epithelial mem brane and S-100toprotein were negative. These antigen, features desmin were considered be diagnostic of the storiform-pleomorphic type of MFH.

Fig. 1. a) Ultrasound examination showing the presence of a hypoechoic mass (arrows) localized in the left lobe of the liver, b) Enhanced computed tomography showing a large mass (arrows) in volving the entire region of the left lobe of the liver, c) Hepatic arteriogram. Note the presence of the large hypovascular mass (arrows) in the left lobe of the liver. Internal


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et al Discussion MFH was previously known as malignant fibrous xanthoma (14) and fibroxanthosarcoma (15, 16). The typical location of MFH is in the soft tissues of the lower or upper extremities. Weiss and Enzinger (1) recently reviewed 200 cases of MFH, 16% of which involved the abdomen and retroperitoneum. While the tissue of origin is uncertain, development from a primitive mesenchymal Microscopically, cell (15) or tissuethe histiocyte tumor is(16) composed has been of suggested. pleomorphic spindle cells arranged in sheets, and fasicles with a prominent storiform pattern (1, 16). MFH of the liver is extremely rare. Table 2 lists the ll cases of primary hepatic MFH previously reported in the literature, and the present case. The average age of these patients was 55.3 years. There was no predilection for one lobe to be the site of origin. The reported tumors were large, 6cm or more in diameter, and diaphragm invasion was revealed in five cases. Thus, most of the tumors were diagnosed in the advanced stage. In general, MFH manifests a broad range of histological patterns and Fig. 2. a) The surgical specimen consisted of a left lobe of the liver, measuring ll.0 x ll.0 x 9.0cm. b) Cut surface of the specimen showing a single, well circumscribed tumor mass (8.0 x 8.0 x 6.0cm)

Fig. 3. a) Low power view of the tumor demonstrating spindle cells in a stri form pattern intermingled with giant cells (HE, x20). b) Hig power view of the tumor showing plemorphic bizarre giant cells (arrows) (HE, x 100). c) Cytoplasmic immunoreactivity for vimentin is most tumor cells (arrows) (x lOO). d) Some of the bizarre giant cells express cytoplasmic immunoreactivity for arantichymotrypsin (arr (xlOO). 286 Internal Medicine Vol. 31, No. 2 (February 1992)

MFHof the Liver Table 2.

Reported Case of Malignant

A ge Sex

L ob e T u m o r S iz e (c m )

1 C o n ra n (19 8 5 )(3) 2 A lb er ti-F lo r ( 19 8 5 )i 3 N ak a za w a (19 8 5 )1,(5 ) 4 F uk uyam a (19 8 6 )',(6)

61 M 59 M 61 F 38 F

L e ft & R ig h t (2 2 .4 x 2 3,5 x 12 ) R ig h t & L e ft R o u n d (1 8 x 1 5 x 1 4 ) R ig h t R o u n d (12 x 1 4 x 1 8 ) L e ft R o u n d (6 x 6 x 7 )

S to rifo rm / p le o m o rp h ic

5 L e n gy el ( 1 9 8 6 Vl

Malignant fibrous histiocytoma of the liver: a case report and review of the literature.

Primary malignant fibrous histiocytoma (MFH) of the liver is reported in a 79-year-old man. The tumor, measuring 8.0 x 8.0 x 6.0 cm, was located in th...
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