Intrahepatic Sarcomatoid Cholangiocarcinoma with Portal Vein Thrombosis: A Case Report 1

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1 Intrahepatic Sarcomatoid Cholangiocarcinoma with Portal Vein Thrombosis: A Case Report 1 Jae-Hoon Lim, M.D., Jin Woong Kim, M.D., Suk Hee Heo, M.D., Yong Yeon Jeong, M.D., Heoung Keun Kang, M.D. A 53-year-old man was admitted to our hospital with a liver mass. CT and MR images showed the presence of a multilobulated mass with hemorrhagic and necrotic foci in the left hepatic lobe. In addition, thrombosis of the left portal vein was noted. The patient underwent surgery for total removal of the mass. A pathological examination revealed the presence of an intrahepatic sarcomatoid cholangiocarcinoma with portal vein thrombosis. The patient underwent a left hepatectomy and chemotherapy. However, the disease recurred with metastases after five months. The patient underwent surgery and chemotherapy for the recurred tumor. Index words : Liver neoplasms Thrombosis Tomography Magnetic resonance (MR) A liver carcinosarcoma is a malignant tumor containing a mixture of carcinomatous and sarcomatous elements (1). Liver carcinosarcomas are classified as two types including sarcomatoid hepatocellular carcinoma (HCC) and cholangiocarcinoma. A sarcomatoid HCC is a carcinoma that most resembles a sarcoma found in the liver. Only a few cases of intrahepatic sarcomatoid cholangiocarcinomas have been reported (1-6). To the best of our knowledge, an intrahepatic sarcomatous cholangiocarcinoma with portal vein thrombosis had not been reported. We report here the first case of a sarcomatoid cholangiocarcinoma with portal vein thrombosis in the English language literature. 1 Department of Radiology, Chonnam National University Hwasun Hospital, Chonnam National University Hospital, Chonnam National University, Medical School Received October 22, 2008 ; Accepted December 29, 2008 Address reprint requests to : Yong Yeon Jeong, M.D., Department of Radiology, Chonnam National University Hwasun Hospital, Chonnam National University, Medical School, 160 Ilsimri Hwasun-eup, Hwasungun, Chonnam , Korea. Tel Fax yjeong@chonnam.ac kr 333 Case Report A 53-year-old man was incidentally found to have a liver tumor. Biochemical indices of liver function showed mild elevation of bilirubin, with a total bilirubin level of 1.4 mg/dl (< 1.2 mg/dl) and direct bilirubin level of 0.5 mg/dl (< 0.4 mg/dl). The levels of tumor markers were the following: alpha-fetoprotein level, 8.57 IU/mL (< 5.8 IU/mL); carcinoembryonic antigen level, 1.65 ng/ml (< 3.4 ng/ml); serum carbohydrate antigen 19-9 level, U/mL (< 39 U/mL). Serum markers for hepatitis B were positive. A CT examination was performed. A precontrast scan showed surface nodularity of the liver, suggestive of liver cirrhosis. As seen on contrast-enhanced CT images, the mass in the medial segment of liver had thick peripheral enhancement as seen on an arterial phase image and concentric filling of contrast media on portal and delayed phase images. A more caudal axial CT scan showed the presence of thrombi within the left portal

2 Jae-Hoon Lim, et al : Intrahepatic Sarcomatoid Cholangiocarcinoma with Portal Vein Thrombosis vein that had the same enhancement pattern as compared to the mass (Fig. 1). As depicted on MR imaging, the mass was observed as hypointense on a T1-weighted image and was observed as hyperintense on a T2-weighted image (Fig. 2). Several hemorrhagic foci were hyperintense within the mass as seen on a T1-weighted image. For contrast-enhanced MR imaging, thick contrast enhancement around the tumor as seen on an arterial phase image and concentric filling of contrast material was seen on portal and delayed phase images. The tumor thrombi of the left portal vein were also noted as hypointense as seen on a T1- weighted image and were noted as hyperintense as seen on a T2-weighted image. The tumor thrombi had thin rim-like enhancement as seen on an arterial phase image and concentric filling of contrast media as seen on portal and delayed phase images. There was no intrahepatic bile duct dilatation. The preoperative differential diagnosis was cholangiocarcinoma based on the enhancement pattern. However, we could not rule out a hepatocellular carcinoma as the patient had underlying liver cirrhosis and portal vein thrombosis. The patient underwent a left hepatectomy with a portal vein thrombectomy. The cut surface showed the presence of a multilobulated, white to yellowish solid mass that measured cm at the greatest dimension. Several small areas of necrosis and hemorrhage were detected in the intrahepatic tumor mass. In addition, tumor thrombi were present within the left portal vein. The surrounding parenchyma showed cirrhotic change. A histological examination of the tumor showed a malignant neoplasm with poorly differentiated adenocarcinoma and sarcomatous components. An immunohistochemical examination of the sarcomatous components showed positive staining for vimentin, cytokeratin and low molecular cytokeratin (Fig. 3). Based on these histological and immunohistochemical findings, the tumor was diagnosed as a cholangiocarcinoma with sarcomatous changes. Fig. 1. A mass in the left hepatic lobe is shown on abdominal dynamic CT (A) arterial, (B) portal and (C) delayed phase images. The mass has thick peripheral enhancement as shown on an arterial phase image and concentric filling of contrast media as shown on portal and delayed phase images. D. As shown on a portal phase image, tumor thrombi within the left portal vein are noted (arrow). A B C D 334

3 A B C D E Fig. 2. A. An axial T1-weighted gradient echo image shows a well-defined hypointense mass with a focal hyperintense lesion. B. An axial T2-weighted single shot fast spin-echo image reveals the hyperintense mass. C. An axial T1-weighted image obtained during the arterial phase shows peripheral ring-like enhancement. D. An axial T1-weighted image obtained during the delayed phase demonstrates concentric filling of contrast media. E. An axial T1-weighted image obtained during the delayed phase demonstrates tumor thrombi with thin rim-like enhancement in the left portal vein (arrow). A B Fig. 3. A. The histological appearance of the sarcomatoid cholangiocarcinoma is shown. Hematoxylin and eosin staining ( 200) shows the cholangiocarcinoma mixed with bundles of atypical spindle cells. B. The appearance of the sarcomatoid cholangiocarcinoma is shown after immunohistochemical staining. The sarcomatous component (arrows) is stained for low molecular cytokeratin ( 400). 335

4 Jae-Hoon Lim, et al : Intrahepatic Sarcomatoid Cholangiocarcinoma with Portal Vein Thrombosis The patient underwent a left hepatectomy and chemotherapy. However, the disease recurred with metastases after five months. The patient underwent surgery and chemotherapy for the recurred tumor. Discussion Most sarcomatoid carcinomas in the liver are thought to be sarcomatoid HCCs. An intrahepatic sarcomatoid cholangiocarcinoma is extremely rare (1-6). Radiological findings for intrahepatic sarcomatoid cholangiocarcinomas have not been established. As depicted on dynamic CT and MR imaging, intrahepatic cholangiocarcinomas have irregular rim-like, band-like contrast enhancement around the tumor as seen on early phase images, with progressive and concentric filling of contrast material as seen on late phase images. The findings for the present case were similar to the findings for the enhancement pattern of a cholangiocarcinoma. The first presumptive diagnosis was an intrahepatic cholangiocarcinoma. However, portal vein thrombosis is not a common manifestation in cholangiocarcinomas. The etiologies of portal vein thrombosis are liver cirrhosis, liver malignancy including HCC, metastases, pancreatic carcinoma and cholangiocarcinoma, pancreatitis, a hypercoagulable state and idiopathy (7). Portal vein thrombosis in patients with liver cirrhosis is usually associated with the presence of an HCC. Most HCCs show early enhancement on hepatic arterial phase images and rapid washout on portal and delayed phase images. In our case, the enhancement pattern was different from an HCC. Portal vein thrombosis and liver cirrhosis are suggestive findings of an HCC. It was difficult to make the differential diagnosis between a cholangiocarcinoma and HCC in this case. It has been reported that the prognosis for an intrahepatic sarcomatoid cholangiocarcinoma is worse as compared to an intrahepatic cholangiocarcinoma (8). The factors that account for the poor prognosis may be ascribed to the remarkable intrahepatic development, especially the high potential of the sarcomatous component to metastasize to a variety of regions (5, 8). The effectiveness of chemotherapy and radiation therapy for intrahepatic sarcomatoid cholangiocarcinomas has not been well investigated well and remains unclear. In conclusion, we have reported the first case of a sarcomatoid cholangiocarcinoma with portal vein thrombosis. A sarcomatoid carcinoma should be included in the etiology of portal vein thromobosis. References 1. Nomura K, Aizawa S, Ushigome S. Carcinosarcoma of the liver. Arch Pathol Lab Med 2000;124: Kaibori M, Kawaguchi Y, Yokoigawa N, Yanagida H, Takai S, Kwon AH, et al. Intrahepatic sarcomatoid cholangiocarcinoma. J Gastroenterol 2003;38: Shimada M, Takenaka K, Rikimaru T, Hamatsu T, Yamashita Y, Kajiyama K, et al. Characteristics of sarcomatous cholangiocarcinoma of the liver. Hepatogastroenterology 2000;47: Matsuo S, Shinozaki T, Yamaguchi S, Takami Y, Obata S, Tsuda N, et al. Intrahepatic cholangiocarcinoma with extensive sarcomatous change: report of a case. Surg Today 1999;29: Kwon JH, Kang YN, Kang KJ. Carcinosarcoma of the liver: a case report. Korean J Radiol 2007;8: Wang XW, Liang P, Li HY. Primary hepatic carcinosarcoma: a case report. Chin Med J 2004;117: Jain P, Nijhawan S. Portal vein thrombosis: etiology and clinical outcome of cirrhosis and malignancy-related non-cirrhotic, non-tumoral extrahepatic portal venous obstruction. World J Gastroenterol 2007;13: Sugihara S, Kojiro M. Pathology of cholangiocarcinoma. In Okuda K, Ishak KG. Neoplasms of the liver. Tokyo: Springer-Verlag, 1987:

5 대한영상의학회지 2009;60: 문맥혈전을동반한간내육종형담관암 : 1 예보고 1 1 전남대학교의과대학화순전남대학교병원영상의학과 임재훈 김진웅 허숙희 정용연 강형근 53세남자가간의종괴를주소로내원하였다. CT와 MRI에서간좌엽에출혈과괴사한부위를포함하는다엽성의종괴가있었으며, 좌측문맥에혈전이관찰되었다. 수술을시행하였으며, 문맥혈전을동반한육종형담관암으로진단되었다. 환자는좌엽절제술을시행받고나서항암치료를받았다. 그러나 5개월후병변이재발하였고전이되어재수술과항암치료를받았다. 337