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Kim DK, Kim BR, Jeong JS, Baek YH. Analysis of intrahepatic sarcomatoid cholangiocarcinoma: Experience from 11 cases within 17 years. World J Gastroenterol 2019; 25:608-621. [PMID: 30774275 PMCID: PMC6371010 DOI: 10.3748/wjg.v25.i5.608] [Citation(s) in RCA: 25] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/30/2018] [Revised: 01/03/2019] [Accepted: 01/15/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Intrahepatic sarcomatoid chonalgiocarcinoma (s-CCC) is an extremely rare disease, accounting for less than 1% of hepatobiliary system malignancies, and its pathophysiology is not well known. On the hypothesis that its clinical, serologic, or radiologic diagnosis are not fully understood and its prognosis is poor, we investigated the distinguishing features of s-CCC compared with those of intrahepatic bile duct adenocarcinoma [cholangiocellular carcinoma (CCC)] in patients from a single center.
AIM To analyze the clinical, serologic, imaging, and histopathologic characteristics of intrahepatic s-CCC patients diagnosed in a single center.
METHODS The clinical, serologic, imaging, and histopathologic features of 227 patients diagnosed with intrahepatic cholangiocarcinoma (IHCC) in a single medical center during the last 17 years were analyzed. The characteristics of 11 patients with s-CCC were compared with those of 216 patients with CCC.
RESULTS The number of patients with s-CCC who presented fever and abdominal pain and past history of chronic viral hepatitis or liver cirrhosis (LC) was higher than that of patients with CCC. In imaging studies, patients with s-CCC showed relatively aggressive features. However, no clear distinction was observed between s-CCC and CCC based on other clinical, serologic or radiologic examination results. An accurate diagnosis could be made only via a histopathologic examination through immunohistochemical staining. The clinical course of s-CCC was generally aggressive, and patients had a relatively poor prognosis.
CONCLUSION In patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.
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Affiliation(s)
- Dong Kyun Kim
- Department of Internal Medicine, Dong-A University College of Medicine, Busan 49201, South Korea
| | - Bo Ra Kim
- Department of Radiology, Dong-A University College of Medicine, Busan 49201, South Korea
| | - Jin Sook Jeong
- Department of Pathology, Dong-A University College of Medicine, Busan 49201, South Korea
| | - Yang Hyun Baek
- Department of Internal Medicine, Dong-A University College of Medicine, Busan 49201, South Korea
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Liao SH, Su TH, Jeng YM, Liang PC, Chen DS, Chen CH, Kao JH. Clinical Manifestations and Outcomes of Patients with Sarcomatoid Hepatocellular Carcinoma. Hepatology 2019; 69:209-221. [PMID: 30014620 DOI: 10.1002/hep.30162] [Citation(s) in RCA: 52] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/06/2018] [Accepted: 05/20/2018] [Indexed: 12/15/2022]
Abstract
Hepatocellular carcinoma (HCC) is the fifth most common cancer and the second leading cause of cancer-related deaths worldwide. Sarcomatoid HCC is a rare histological subtype of HCC with largely unclear clinical manifestations and outcomes. We evaluated the clinical manifestations and outcomes of patients with sarcomatoid HCC. We identified 5,047 patients with histologically proven HCC from the Cancer Registry Database (1996-2016) of National Taiwan University Hospital. Among them, 40 patients with sarcomatoid HCC were identified from the pathology database of National Taiwan University Hospital. We included 160 patients with nonsarcomatoid HCC through propensity score matching according to sex, age, and Barcelona Clinic Liver Cancer stage. The majority of these patients with sarcomatoid HCC were men (75%); their median age was 58 years. Only 47.5% of the patients with sarcomatoid HCC presented with typical image patterns of HCC. The pathological grading of sarcomatoid HCC was more advanced compared with that of nonsarcomatoid HCC (42.5% vs. 23.8% in grade III and IV, P < 0.0001). The sarcomatoid group had significantly shorter median recurrence-free (13.3 vs. 84.2 months, log-rank P < 0.0001) and overall (8.3 vs. 69.3 months, log-rank P < 0.0001) survival than did the nonsarcomatoid group. The results of the multivariable Cox proportional hazard model revealed histological sarcomatoid subtype as an independent factor for all-cause mortality (hazard ratio [HR], 6.47; 95% confidence interval [CI], 3.12-13.43; P < 0.0001) and tumor recurrence (HR, 4.08; 95% CI, 1.72-9.66; P = 0.001). Conclusion: Compared with nonsarcomatoid HCC, sarcomatoid HCC was associated with more advanced histological grades and atypical image patterns. Histological sarcomatoid subtype is an independent predictor of tumor recurrence after curative treatment and all-cause mortality in patients with HCC.
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Affiliation(s)
- Sih-Han Liao
- National Taiwan University Cancer Center, Taipei, Taiwan.,Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Tung-Hung Su
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
| | - Yung-Ming Jeng
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
| | - Po-Chin Liang
- Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan
| | - Ding-Shinn Chen
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.,Genomics Research Center, Academia Sinica, Taipei, Taiwan
| | - Chien-Hung Chen
- Department of Internal Medicine, National Taiwan University Hospital Yunlin Branch, Yunlin County, Taiwan.,National Taiwan University College of Medicine, Taipei, Taiwan
| | - Jia-Horng Kao
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.,Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan
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Leng Q, Xiang XI, Tang Y, Yang Y, Qiu LI. Primary hepatic sarcomatoid carcinoma: A case report. Exp Ther Med 2015; 10:1145-1148. [PMID: 26622454 DOI: 10.3892/etm.2015.2599] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2014] [Accepted: 06/01/2015] [Indexed: 02/05/2023] Open
Abstract
Sarcomatoid carcinoma (SC) is a malignant tumor type of unclear pathogenesis, which rarely occurs in the liver. The present study reports the case of a 60-year-old woman who presented with multiple masses in her liver, with no fever, abdominal pain or jaundice. Serological markers for hepatitis B and C virus were negative, as was the test for α fetoprotein. Abdominal enhanced magnetic resonance imaging examination confirmed multiple lesions in the liver, and liquefactive necrosis was observed. The patient underwent a right hepatectomy, in addition to a cholecystectomy. Cytokeratin 8 (CK8), cluster of differentiation 117, pancytokeratin and vimentin were positively detected using immunohistochemistry, and thus the diagnosis of primary hepatic SC (PHSC) was established. During the follow-up period, characteristic imaging manifestations of tumor recurrence were detected, including peripheral enhancement, central necrosis, variable enhancement, venous invasion and intrahepatic metastasis. The immunohistochemical detection of CK, epithelial membrane antigen and vimentin, with CK8 as a critical indicator, may be useful for the diagnosis of PHSC. The preferred treatment for PHSC is surgical resection and the prognosis is poor.
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Affiliation(s)
- Qianying Leng
- Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China ; Department of Ultrasound, No.4 West China Teaching Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - X I Xiang
- Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - Yuanjiao Tang
- Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - Yujia Yang
- Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China
| | - L I Qiu
- Department of Ultrasound, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, P.R. China
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Okabayashi T, Shima Y, Iwata J, Iiyama T, Sumiyoshi T, Kozuki A, Tokumaru T, Hata Y, Noda Y, Morita M. Surgical outcomes for 131 cases of carcinosarcoma of the hepatobiliary tract. J Gastroenterol 2014; 49:982-91. [PMID: 24162331 DOI: 10.1007/s00535-013-0882-2] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2013] [Accepted: 08/18/2013] [Indexed: 02/07/2023]
Abstract
Carcinosarcoma of the hepatobiliary tract is highly aggressive and has a poor prognosis even after curative resection. The purpose of this study was to collate and analyze published data to clarify the surgical outcome of carcinosarcoma of the hepatobiliary tract and the relationships between potential prognostic factors and survival after surgery. We surveyed worldwide literature from 1970 to 2012 and obtained clinicopathological data for 131 patients who had undergone surgical resection for carcinosarcoma of the hepatobiliary tract, including one patient from our clinic. The relationships between potential prognostic factors and survival rates were examined using the Kaplan-Meier method and the log-rank test. The overall 1-, 3-, and 5-year survival rates for patients with carcinosarcoma of the hepatobiliary tract after surgery were 44.0, 29.3, and 27.0 %, respectively. In univariate analyses, age and gender were not significant prognostic factors; however, advanced stage according to the classification of the Union for International Cancer Control in resected specimens was significantly associated with a shorter survival time after surgery. Although carcinosarcoma of the hepatobiliary tract remains a rare disease worldwide, its poor prognosis, even after curative resection, demands further epidemiological and pathological study that could lead to the development of new management strategies.
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Affiliation(s)
- Takehiro Okabayashi
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, 2125-1 Ike, Kochi, Kochi, 781-8555, Japan,
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Carcinosarcoma of the liver: report of a case. Surg Today 2013; 44:1161-70. [PMID: 23645292 DOI: 10.1007/s00595-013-0612-7] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2012] [Accepted: 12/09/2012] [Indexed: 02/07/2023]
Abstract
A 64-year-old Japanese woman without a history of viral hepatitis was admitted for investigation of a huge liver mass. The tumor, measuring 14 × 12 × 22 cm, had invaded the diaphragm, right lung, and inferior vena cava. Serum examinations demonstrated high levels of carbohydrate antigen 19-9 (CA19-9), and the Child-Pugh score was A. She underwent right lobectomy of the liver and partial resection of the right diaphragm, right lung, and inferior vena cava. Radio- and chemotherapy were also given, but she died of recurrence 3 months after surgery. Microscopically, the tumor exhibited intermingled adenocarcinomatous and atypical mesenchymal components. The carcinomatous component was positive for cytokeratins 7, 19, and 20, chromogranin A, epithelial membrane antigen, c-KIT, and vimentin. The sarcomatous component was positive for vimentin and c-KIT. A review of 36 cases of hepatic carcinosarcoma revealed the following: chronic hepatitis or cirrhosis in 57 % of the patients; increased serum CA19-9 levels in 30 %; a mean tumor diameter of 10 cm; invasion of the adjacent organs or metastasis to distant organs in 47 %; wide intrahepatic infiltration in 44 %; and 50 % survival of only 5 months. Significant differences were seen according to tumor diameter (diameter >5 cm; p < 0.05), wide intrahepatic infiltration (p < 0.05), and extrahepatic invasion/metastasis (p < 0.01). Neither chemotherapy nor radiotherapy contributed to prognosis, but surgical resection resulted in some improvement (p < 0.05).
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Goto H, Tanaka A, Kondo F, Takeshita K, Nagashima I, Hanawa N, Aiso M, Takamori Y, Kato KI, Takahashi Y, Fukushima JI, Furui S, Fukusato T, Asano T, Takikawa H. Carcinosarcoma of the liver. Intern Med 2010; 49:2577-82. [PMID: 21139295 DOI: 10.2169/internalmedicine.49.3581] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022] Open
Abstract
Herein we present a 73-year-old man with primary carcinosarcoma of the liver, a rare malignant tumor of the liver. The case was followed up due to HBV-related liver cirrhosis. Regular check-up by ultrasound demonstrated a hyperechoic tumor in the left lobe of the liver, and he was referred and admitted to our hospital. Dynamic CT studies revealed a mostly hypoenhancing hepatic mass with a peripheral ring enhancement. Surgical resection was performed, and the resected tumor was macroscopically a simple nodular type, 3 cm in diameter, with a dense fibrous capsule. Microscopically, undifferentiated cells were dominant in the tumor, while moderately differentiated hepatocellular carcinoma (HCC) were also observed. A transitional zone was noted between the undifferentiated tumor and HCC. Tumor tissue with adenocarcinoma, osteosarcoma and chondrosarcoma were also detected. Immunohistochemical studies demonstrated that tumor cells were HepPar 1 positive in hepatocellular carcinoma, and CK19 and partly CK7 positive in adenocarcinoma. Moreover, CD56, chromogranin A and c-kit were occasionally positive in undifferentiated tumor cells. The diagnosis of carcinosarcoma was made based on the concomitant presence of HCC and sarcomatous components, yet it is noteworthy that various types of tumor cells were observed.
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Affiliation(s)
- Hideaki Goto
- Department of Medicine, Teikyo University School of Medicine, Tokyo
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Sumiyoshi S, Kikuyama M, Matsubayashi Y, Kageyama F, Ide Y, Kobayashi Y, Nakamura H. Carcinosarcoma of the liver with mesenchymal differentiation. World J Gastroenterol 2007; 13:809-12. [PMID: 17278210 PMCID: PMC4066020 DOI: 10.3748/wjg.v13.i5.809] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
We report an extremely rare case where a mesenchymal differentiation, especially embryonal sarcoma, was demonstrated in cholangiocarcinoma. At autopsy, a yellowish-white tumor (15 cm x 12 cm) was found in the right hepatic lobe, and there were several daughter nodules in both hepatic lobes. Histologically, most of the main tumor and all of the daughter nodules examined showed sarcomatous changes (spindle cells, pleomorphic cells and hyalization). Histologic examination of a part of the main tumor disclosed a focus of adenocarcinoma within the tumor. The frequent transitions between the adenocarcinomatous areas and the sarcomatous areas suggested that sarcomatous transformation occurred in the cholangiocarcinoma and then spread rapidly. Immunohistochemically, the adenocarcinomatous elements were positive for cytokeratin, carcinoembryonic antigen (CEA) and epithelial membrane antigen, and negative in the sarcomatous cells. Vimentin was positive only in the sarcomatous elements. The findings of the present case support the view that carcinosarcomas represent carcinomas that develop sarcomatous elements via metaplasia of the epithelial element.
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Garcez-Silva MH, Gonzalez AM, Moura RAM, Linhares MM, Lanzoni VP, Trivino T. Carcinosarcoma of the Liver: A Case Report. Transplant Proc 2006; 38:1918-9. [PMID: 16908322 DOI: 10.1016/j.transproceed.2006.06.057] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Primary hepatic carcinosarcoma is a rare malignant hepatic tumor containing both carcinomatous and sarcomatous elements. A 40-year-old man referred to our liver transplant team because of hepatic cirrhosis was on the waiting list, having undergone all liver tests, ultrasonography, and with normal serum alpha fetoprotein markers every 6 months to search for a tumor. He underwent a liver transplantation without complication. The pathologic findings of the original liver indicated carcinosarcoma. We have reviewed the literature on this subject.
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Affiliation(s)
- M H Garcez-Silva
- Liver Transplantation Surgeons, Department of Surgery, Surgical Gastroenterology-Transplantation Ward, São Paulo Federal University, Paulista School of Medicine, Rua dos Otonis 899, São Paulo, Brazil.
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