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Tsai TC, Su CM. A case of hepatic sarcomatoid cholangiocarcinoma with diaphragmatic and right lower lobe lung invasion. J Surg Case Rep 2025; 2025:rjaf047. [PMID: 39931030 PMCID: PMC11809243 DOI: 10.1093/jscr/rjaf047] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/17/2024] [Accepted: 01/22/2025] [Indexed: 02/13/2025] Open
Abstract
Hepatic sarcomatoid cholangiocarcinoma is a rare, accounting for <1% of cases and aggressive malignant neoplasm. This report presents a case of a 35-year-old female patient who presented with right shoulder pain, general weakness, anorexia, and weight loss. Imaging studies revealed a hepatic mass in segment S8, with invasion into the diaphragm and right lower lobe of the lung. The patient underwent surgical resection, and the pathological diagnosis confirmed hepatic sarcomatoid cholangiocarcinoma. This case report discusses the clinical presentation, diagnosis, and treatment of this rare malignancy, emphasizing the importance of early diagnosis and aggressive management.
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Affiliation(s)
- Tsung Chiao Tsai
- General Surgical Department, National Cheng Kung University Hospital, No. 138, Shengli Rd., North Dist., Tainan City 704, Taiwan (R.O.C.)
| | - Che Min Su
- General Surgical Department, National Cheng Kung University Hospital, No. 138, Shengli Rd., North Dist., Tainan City 704, Taiwan (R.O.C.)
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2
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Tomlinson JL, Li B, Yang J, Loeuillard E, Stumpf HE, Kuipers H, Watkins R, Carlson DM, Willhite J, O'Brien DR, Graham RP, Chen X, Smoot RL, Dong H, Gores GJ, Ilyas SI. Syngeneic murine models with distinct immune microenvironments represent subsets of human intrahepatic cholangiocarcinoma. J Hepatol 2024; 80:892-903. [PMID: 38458319 PMCID: PMC11141161 DOI: 10.1016/j.jhep.2024.02.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/16/2023] [Revised: 01/19/2024] [Accepted: 02/09/2024] [Indexed: 03/10/2024]
Abstract
BACKGROUND & AIMS Cholangiocarcinoma (CCA) is a poorly immunogenic malignancy associated with limited survival. Syngeneic immunocompetent mouse models of CCA are an essential tool to elucidate the tumor immune microenvironment (TIME), understand mechanisms of tumor immune evasion, and test novel immunotherapeutic strategies. The scope of this study was to develop and characterize immunocompetent CCA models with distinct genetic drivers, and correlate tumor genomics, immunobiology, and therapeutic response. METHODS A multifaceted approach including scRNA-seq, CITE-seq, whole exome and bulk RNA sequencing was employed. FDA-approved PD-1/PD-L1 antibodies were tested in humanized PD-1/PD-L1 mice (HuPD-H1). RESULTS A genetic mouse model of intrahepatic CCA (iCCA) driven by intrabiliary transduction of Fbxw7ΔF/Akt that mimics human iCCA was generated. From the Fbxw7ΔF/Akt tumors, a murine cell line (FAC) and syngeneic model with genetic and phenotypic characteristics of human iCCA were developed. Established SB1 (YAPS127A/Akt) and KPPC (KrasG12Dp53L/L) models were compared to the FAC model. Although the models had transcriptomic similarities, they had substantial differences as well. Mutation patterns of FAC, SB1, and KPPC cells matched different mutational signatures in Western and Japanese CCA patient cohorts. KPPC tumors had a high tumor mutation burden. FAC tumors had a T cell-infiltrated TIME, while SB1 tumors had a preponderance of suppressive myeloid cells. FAC, SB1, and KPPC tumors matched different immune signatures in human iCCA cohorts. Moreover, FAC, SB1, and KPPC tumor-bearing HuPD-H1 mice displayed differential responses to nivolumab or durvalumab. CONCLUSIONS Syngeneic iCCA models display a correlation between tumor genotype and TIME phenotype, with differential responses to FDA-approved immunotherapies. This study underscores the importance of leveraging multiple preclinical models to understand responses to immunotherapy in different genetic subsets of human CCA. IMPACT AND IMPLICATIONS Understanding the relationship between tumor genotype and the phenotype of the immune microenvironment is an unmet need in cholangiocarcinoma (CCA). Herein, we use syngeneic murine models of intrahepatic CCA with different genetic drivers to demonstrate a correlation between tumor genotype and immune microenvironment phenotype in murine models, which is associated with differential responses to FDA-approved immunotherapies. This information will help guide other preclinical studies. Additionally, it emphasizes that immune checkpoint inhibition in patients with CCA is not a "one-size-fits-all" approach. Our observations suggest that, as for targeted therapies, patients should be stratified and selected for treatment according to their tumor genetics.
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Affiliation(s)
| | - Binbin Li
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Jingchun Yang
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Emilien Loeuillard
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Hannah E Stumpf
- Mayo Clinic Graduate School of Biomedical Sciences, Rochester, MN, USA
| | | | - Ryan Watkins
- Department of Surgery, Mayo Clinic, Rochester, MN, USA
| | | | - Jessica Willhite
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Daniel R O'Brien
- Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, USA
| | - Rondell P Graham
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
| | - Xin Chen
- Cancer Biology Program, University of Hawaii Cancer Center, Honolulu, HI, USA
| | - Rory L Smoot
- Department of Surgery, Mayo Clinic, Rochester, MN, USA; Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN, USA
| | - Haidong Dong
- Department of Urology, Mayo Clinic, Rochester, MN, USA; Department of Immunology, Mayo Clinic, Rochester, MN, USA
| | - Gregory J Gores
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Sumera I Ilyas
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA; Department of Immunology, Mayo Clinic, Rochester, MN, USA.
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Choi JH, Thung SN. Recent Advances in Pathology of Intrahepatic Cholangiocarcinoma. Cancers (Basel) 2024; 16:1537. [PMID: 38672619 PMCID: PMC11048541 DOI: 10.3390/cancers16081537] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/29/2024] [Revised: 04/10/2024] [Accepted: 04/16/2024] [Indexed: 04/28/2024] Open
Abstract
Intrahepatic cholangiocarcinoma (ICCA) is a malignant epithelial neoplasm characterized by biliary differentiation within the liver. ICCA is molecularly heterogeneous and exhibits a broad spectrum of histopathological features. It is a highly aggressive carcinoma with high mortality and poor survival rates. ICCAs are classified into two main subtypes: the small-duct type and large-duct types. These two tumor types have different cell origins and clinicopathological features. ICCAs are characterized by numerous molecular alterations, including mutations in KRAS, TP53, IDH1/2, ARID1A, BAP1, BRAF, SAMD4, and EGFR, and FGFR2 fusion. Two main molecular subtypes-inflammation and proliferation-have been proposed. Recent advances in high-throughput assays using next-generation sequencing have improved our understanding of ICCA pathogenesis and molecular genetics. The diagnosis of ICCA poses a significant challenge for pathologists because of its varied morphologies and phenotypes. Accurate diagnosis of ICCA is essential for effective patient management and prognostic determination. This article provides an updated overview of ICCA pathology, focusing particularly on molecular features, histological subtypes, and diagnostic approaches.
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Affiliation(s)
- Joon Hyuk Choi
- Department of Pathology, Yeungnam University College of Medicine, Daegu 42415, Republic of Korea
| | - Swan N. Thung
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, 1468 Madison Avenue, New York, NY 10029, USA;
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Zhao Q, Ji Z, Chen Y, Wang K, Qiu Y, Tian X, Zhu Y, Qin H, Han H, Yuan H, Dong Y, Wang W. Contrast-enhanced ultrasound features of hepatic sarcomatoid carcinoma different from hepatocellular carcinoma. Clin Hemorheol Microcirc 2024; 87:55-65. [PMID: 38160348 DOI: 10.3233/ch-231944] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2024]
Abstract
BACKGROUND Hepatic sarcomatoid carcinoma (HSC) is a rare malignancy of the liver. The ultrasound and clinical features of HSC have not been determined. OBJECTIVE To investigate and compare the ultrasound and clinical features of HSC and hepatocellular carcinoma (HCC), and to reveal the valuable features of HSC. METHODS The ultrasound features and clinical data of pathologically proven HSC (n = 37) were compared with HCC (n = 92) in a matching ratio of 1:4 using the propensity score (age, gender and tumor size). RESULTS The HSC patients were more likely to accompany with clinical symptoms and vascular invasion than HCC patients (40.5% vs 17.4%, 24.3% vs 6.5%, P < 0.05). The size of HSCs was significantly larger than that of HCCs (P < 0.05). The proportion of patients with elevated alpha-fetoprotein was significantly lower in HSC (35.1% vs 54.3%, P < 0.05). On gray-scale ultrasound images, the HSCs were more likely to demonstrate as indistinct margin and irregular shape lesions compared to HCCs (78.4% vs 48.8%; 70.3% vs 23.9%, P < 0.05). Under color Doppler flow imaging (CDFI), the blood flow signals were more frequently detected in HSC lesions (75.7% vs 56.5%, P < 0.05). Resistance index (RI) was higher in HSCs than in HCCs [0.78 (0.70,0.82) vs 0.70 (0.62,0.76), P < 0.05]. On contrast-enhanced ultrasound (CEUS), HSCs mainly showed entirety heterogeneous hyper-enhancement (48.6%), entirety homogeneous enhancement (18.9%), peripheral and internal septal enhancement (18.9%). The incidence of non-enhanced areas inside HSC lesions was higher than that inside HCC lesions (56.8% vs 31.5%, P < 0.05). During the portal venous and late phases, most of the lesions revealed hypo-enhancement in both groups, whereas earlier washout was observed in HSCs [43.0 s (30.5,58.0) vs 60.0 s (46.3,100.0), P < 0.05]. CONCLUSIONS CEUS features are useful in preoperative and non-invasive differentiation of hepatic sarcomatoid carcinoma and hepatocellular carcinoma.
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Affiliation(s)
- Qiannan Zhao
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Zhengbiao Ji
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Yanling Chen
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Kun Wang
- Department of Ultrasound, Binzhou Medical University Hospital, Binzhou, China
| | - Yijie Qiu
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Xiaofan Tian
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Yuli Zhu
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Hong Qin
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Hong Han
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Haixia Yuan
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
- Department of Ultrasound, Zhongshan Hospital, Fudan University (Qingpu Branch), Shanghai, China
| | - Yi Dong
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
- Department of Ultrasound, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Wenping Wang
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
- Shanghai Institute of Imaging Medicine, Shanghai, China
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Liang B, Huang T, Kuang SL, Xie GY, Liu TQ, Chen YY. Surgery after combination therapy with a tyrosine kinase inhibitor and anti-PD-1 antibody in sarcomatoid hepatocellular carcinoma: case report and literature review. Front Oncol 2023; 13:1212013. [PMID: 37869099 PMCID: PMC10585252 DOI: 10.3389/fonc.2023.1212013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2023] [Accepted: 09/18/2023] [Indexed: 10/24/2023] Open
Abstract
Introduction Although surgery is the preferred treatment for sarcomatoid hepatocellular carcinoma (SHC), the prognosis remains considerably poor due to early postoperative recurrence and metastasis. Reports on surgery after combined treatment with a tyrosine kinase inhibitor and anti-programmed cell death (PD)-1 antibody are unavailable. Case presentation A 69-year-old male patient with SHC was admitted to our hospital for treatment of a liver tumor that was detected on ultrasonography. Abdominal computed tomography with triple-phase enhancement revealed a lesion in the right hepatic lobe that measured 86.0 mm × 75.0 mm × 71.0 mm. Biopsy revealed a pathological diagnosis of liver sarcoma or sarcomatoid carcinoma. The patient subsequently received transcatheter arterial chemo-embolization, as he did not consent to surgery. More than two months later, he received a combination of lenvatinib with camrelizumab, as computed tomography showed an increase in the lesion size (to 123.0 mm × 90.0 mm × 80.0 mm) and lateral growth posterior to the upper pole of the right kidney. Liver resection was performed after 6 months of systemic therapy; pathological examination confirmed a diagnosis of SHC and showed extensive necrosis of tumor cells. Combined treatment with lenvatinib and camrelizumab was continued for 6 months after surgery. The patient has survived for over 24 months after initial diagnosis and is currently tumor-free. Conclusion Combined systemic therapy with a tyrosine kinase inhibitor and anti-PD-1 antibody may represent a feasible treatment strategy for improving resectability in cases of unresectable SHC. The outcomes with this combination may also be explored in cases of resectable SHC that have a high-risk of recurrence; this may improve the therapeutic effect.
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Affiliation(s)
- Bin Liang
- Department of Hepatobiliary, Pancreatic and Spleen Surgery, Guangxi Academy of Medical Sciences, the People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, China
| | - Tao Huang
- Department of Hepatobiliary, Pancreatic and Spleen Surgery, Guangxi Academy of Medical Sciences, the People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, China
| | - Shao-Lei Kuang
- Department of Hepatobiliary, Pancreatic and Spleen Surgery, Guangxi Academy of Medical Sciences, the People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, China
| | - Guang-Yuan Xie
- Department of Hepatobiliary, Pancreatic and Spleen Surgery, Guangxi Academy of Medical Sciences, the People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, China
| | - Tian-Qi Liu
- Department of General Surgery, the Jiangbin Hospital of Guangxi Zhuang Autonomous Region, Nanning, China
| | - Yuan-Yuan Chen
- Department of Hepatobiliary, Pancreatic and Spleen Surgery, Guangxi Academy of Medical Sciences, the People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, China
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Zaher EA, Patel P, Gotimukul A, Sqour H. Sarcomatoid Intrahepatic Cholangiocarcinoma: A Rare and Aggressive Primary Liver Cancer. Cureus 2023; 15:e39520. [PMID: 37366443 PMCID: PMC10290760 DOI: 10.7759/cureus.39520] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 05/26/2023] [Indexed: 06/28/2023] Open
Abstract
Sarcomatoid intrahepatic cholangiocarcinoma (S-iCCA) is a rare variant of primary liver cancer with a poor prognosis due to local aggressive expansion and frequent metastases. The pathogenesis remains unclear, but theories suggest epithelial-mesenchymal transition, biphasic differentiation of pluripotent stem cells, or sarcomatoid re-differentiation of immature multipotent carcinoma cells. Chronic hepatitis B and C, cirrhosis, and age above 40 are plausible contributors. Diagnosis of S-iCCA requires immunohistochemical evidence of both mesenchymal and epithelial molecular expression. Early detection and total resection are the current mainstay approach. We report a case of metastatic S-iCCA in a 53-year-old male with alcohol use disorder who underwent en bloc right hepatic lobectomy, right adrenalectomy, and cholecystectomy.
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Affiliation(s)
- Eli A Zaher
- Department of Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Parth Patel
- Department of Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Ashwini Gotimukul
- Department of Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
| | - Hasan Sqour
- Department of Internal Medicine, Ascension Saint Joseph Hospital, Chicago, USA
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Lieu DQ, Anh TN, Hoa TT, Phuong VT, Duc NM. An extremely rare case of intrahepatic sarcomatoid cholangiocarcinoma. Radiol Case Rep 2023; 18:968-973. [PMID: 36636481 PMCID: PMC9829551 DOI: 10.1016/j.radcr.2022.12.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2022] [Accepted: 12/04/2022] [Indexed: 01/07/2023] Open
Abstract
One uncommon histological subtype of intrahepatic cholangiocarcinoma is sarcomatoid intrahepatic cholangiocarcinoma. Histopathological and immunohistochemical tests are used to diagnose sarcomatoid intrahepatic cholangiocarcinoma, which frequently has a worse prognosis than regular intrahepatic cholangiocarcinoma. The example of a 65-year-old female with sarcomatoid intrahepatic cholangiocarcinoma, who presented with sporadic right upper abdomen discomfort, is discussed in this paper. This case study and literature analysis aims to improve physicians' comprehension of sarcomatoid intrahepatic cholangiocarcinoma and lower the frequency of missed clinical diagnoses.
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Affiliation(s)
- Dau Quang Lieu
- Department of Internal Medicine, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Tran Ngoc Anh
- Department of Internal Medicine, Hanoi Medical University Hospital, Hanoi, Vietnam,Department of Internal Medicine, Hanoi Medical University, Hanoi, Vietnam
| | - Tran-Thi Hoa
- Department of Pathology, Hanoi Medical University, Hanoi, Vietnam
| | - Vu-Thi Phuong
- Department of Pathology, Vinmec Healthcare System, Hanoi, Vietnam
| | - Nguyen Minh Duc
- Department of Radiology, Pham Ngoc Thach University of Medicine, 2 Duong Quang Trung Ward 12 District 10, Ho Chi Minh City, Vietnam,Corresponding author.
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Yoshuantari N, Jeng YM, Liau JY, Lee CH, Tsai JH. Hepatic Sarcomatoid Carcinoma Is an Aggressive Hepatic Neoplasm Sharing Common Molecular Features With Its Conventional Carcinomatous Counterparts. Mod Pathol 2023; 36:100042. [PMID: 36788061 DOI: 10.1016/j.modpat.2022.100042] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2022] [Revised: 10/11/2022] [Accepted: 10/11/2022] [Indexed: 01/19/2023]
Abstract
Hepatic sarcomatoid carcinoma is a rare hepatic tumor with an aggressive clinical behavior and dismal outcome. However, the molecular pathogenesis is incompletely defined. In this study, we analyzed 59 hepatic sarcomatoid carcinomas using targeted next-generation sequencing and immunohistochemistry. A panel of 14 genes commonly mutated in primary liver carcinomas was examined. PD-L1 and loss of expression for switch/sucrose nonfermenting complexes, including BAP1, ARID1A, ARID2, and PBRM1, were detected by immunohistochemistry. The 59 hepatic sarcomatoid carcinomas encompass various carcinomatous subtypes and tumors with complete sarcomatoid transformation. Mutations in TP53 and promoter of TERT (pTERT) were frequently identified in sarcomatoid hepatocellular carcinoma, sarcomatoid combined hepatocellular cholangiocarcinoma, and hepatic sarcomatoid carcinomas with complete sarcomatoid transformation but rarely in sarcomatoid cholangiocarcinoma. Alterations involving switch/sucrose nonfermenting complexes were uncommon in hepatic sarcomatoid carcinoma (n = 2). PD-L1 expressed in tumor-associated immune cells in 67% of the tumors and in tumor cells in 33% of the tumors. A multivariate survival analysis indicated that PD-L1 expression in immune cells served as an independent favorable predictive factor of patient survival (P = .036). In conclusion, hepatic sarcomatoid carcinoma displays molecular similarity with its conventional carcinomatous counterparts. This finding suggests persistent genetic characteristics during sarcomatous evolution. PD-L1 expression in immune cells is a favorable prognostic factor for patient outcomes and may be a potential biomarker for immunotherapeutic treatment.
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Affiliation(s)
- Naomi Yoshuantari
- Department of Anatomical Pathology, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada/Dr Sardjito General Hospital, Yogyakarta, Indonesia
| | - Yung-Ming Jeng
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan; Graduate Institute of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Jau-Yu Liau
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan; Graduate Institute of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan
| | - Chia-Hsiang Lee
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
| | - Jia-Huei Tsai
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan; Graduate Institute of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan.
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Zeng Z, Liu Y, Yu J, Xu Q, Wang Y, Zhao C, Jiang O. Sarcomatoid Intrahepatic Cholangiocarcinoma After Immunotherapy: A Case Report and Review of the Literature. J Clin Transl Hepatol 2022; 10:1240-1249. [PMID: 36381099 PMCID: PMC9634775 DOI: 10.14218/jcth.2021.00395] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/07/2021] [Revised: 12/14/2021] [Accepted: 01/29/2022] [Indexed: 12/04/2022] Open
Abstract
Sarcomatoid carcinoma is a rare tumor that is composed of a mixture of malignant epithelial cells and mesenchymal cells. Many studies have reported that sarcomatoid carcinoma occurs in multiple organs including the liver. Sarcomatoid intrahepatic cholangiocarcinoma (S-iCCA) is an extremely rare tumor that primarily occurs in the liver. This case occurred in a middle-aged man who was admitted to our hospital with abdominal pain. Enhanced computed tomography of the abdomen showed a low-density mass in the upper right posterior lobe of the liver with enhancement in the periphery. Histological and immunohistochemical examination indicated that the tumor was malignant, with both cancer and sarcoma components, and was positive for cytokeratin and vimentin. The patient was diagnosed with S-iCCA. Metastases appeared in the liver and lung 4 months after surgery. Two cycles of chemotherapy were administered. Because of enlargement of the tumor, anti-angiogenic agents combined with immunotherapy were subsequently given to achieve disease control. To the best of our knowledge, this is the first reported case of a programmed cell death-1 inhibitor used in a S-iCCA patient. The purpose of this case report and literature review is to enhance clinician understanding of S-iCCA and to explore safe and effective treatment methods.
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Affiliation(s)
- Zheng Zeng
- Department of Abdominal Oncology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
| | - Yu Liu
- Department of Abdominal Oncology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
| | - Jing Yu
- Department of Abdominal Oncology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
| | - Qiang Xu
- Department of Abdominal Oncology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
| | - Yong Wang
- Department of Abdominal Oncology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
| | - Chang Zhao
- Department of Pathology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
| | - Ou Jiang
- Department of Abdominal Oncology, The Second People’s Hospital of Neijiang, Neijiang, Sichuan, China
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Peng K, Li X, Wang Z, Li M, Yang Y. Association of low-density lipoprotein cholesterol levels with the risk of mortality and cardiovascular events: A meta-analysis of cohort studies with 1,232,694 participants. Medicine (Baltimore) 2022; 101:e32003. [PMID: 36482567 PMCID: PMC9726298 DOI: 10.1097/md.0000000000032003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
BACKGROUND Lowering elevated low-density lipoprotein cholesterol (LDL-C) is an important strategy to prevent cardiovascular disease (CVD), while some studies report low LDL-C increases all-cause mortality. Our study aimed to explore the appropriate low LDL-C level with the lower CVD risk but with no excess risk for all-cause mortality. METHODS PubMed, Embase, Cochrane Library, and Web of Science were searched until April 7, 2021. Twenty cohort studies with 1232,694 adults were obtained. Effect size index was evaluated using pooled relative risk (RR) with 95% confidence interval (CI). Heterogeneity was assessed using the Cochran's Q test and I2 statistic, and heterogeneity sources was investigated using meta-regression. Publication bias was assessed and sensitivity analysis was performed. RESULTS The risks of all-cause mortality (RR: 1.34, 95%CI: 1.00-1.80), CVD death (RR: 1.79, 95%CI: 1.26-2.54), CHD death (RR: 2.03, 95%CI: 1.36-3.03) were higher in LDL-C ≥ 160 mg/dL than LDL-C of 70-129 mg/dL. Both LDL-C of 130-159 mg/dL and ≥ 160 mg/dL were associated with higher CVD risk than LDL-C of 70-129 mg/dL, with RR of 1.26 (95%CI: 1.08-1.47) and 1.70 (95%CI: 1.35-2.14), respectively. Compared to LDL-C of 70-129 mg/dL, no association was found between LDL < 70 mg/dL and all-cause mortality and CVD events. CONCLUSION Our results found LDL-C ≥ 130 mg/dL was associated with the higher risk of all-cause mortality and CVD risk, indicating that adults with high LDL-C should take interventions to regulate the LDL-C level lower than 130 mg/dL.
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Affiliation(s)
- Ke Peng
- Department of Cardiology, The General Hospital of Western Theater Command, Chengdu, P.R. China
| | - Xingyue Li
- School of Materials Science and Engineering, Southwest Jiaotong University, Chengdu, P.R. China
| | - Zhen Wang
- Department of Cardiology, The General Hospital of Western Theater Command, Chengdu, P.R. China
| | - Meiling Li
- Department of Cardiology, The General Hospital of Western Theater Command, Chengdu, P.R. China
| | - Yongjian Yang
- Department of Cardiology, The General Hospital of Western Theater Command, Chengdu, P.R. China
- * Correspondence: Yongjian Yang, Department of Cardiology, The General Hospital of Western Theater Command, No.270 Rongdu Road, Jinniu District, Chengdu 610083, P.R. China (e-mail: )
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11
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Xi LF, Jin Y, Li JT. Intrahepatic sarcomatoid cholangiocarcinoma: A case report of the youngest patient on record and a review of the condition's characteristics. Front Surg 2022; 9:963952. [PMID: 36117828 PMCID: PMC9476318 DOI: 10.3389/fsurg.2022.963952] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2022] [Accepted: 08/15/2022] [Indexed: 11/13/2022] Open
Abstract
As a rare disease, intrahepatic sarcomatoid cholangiocarcinoma (s-CCC) represents less than 1% of malignancies of the hepatobiliary system and its main clinical symptoms include abdominal pain and fever. Results of pathological examinations, despite being the "gold standard", can easily be confused with hepatocellular carcinoma (HCC). This report is about a 32-year-old male patient who was hospitalized due to occupancy of segment V of the liver for three days and had a history of chronic hepatitis B (CHB) over a 20-year span. Magnetic resonance imaging (MRI) showed a 43 mm × 52 mm-sized liver mass in the V segment, with patchy peripheral enhancement during the arterial phase and rapid wash-out during the portal and late phases. A laparoscopic hepatectomy of segment V, along with cholecystectomy, was performed. Histopathological and immunohistochemical examinations indicated a malignant neoplasm that was positive for vimentin and cytokeratin, with these features providing a positive diagnosis for intrahepatic sarcomatoid cholangiocarcinoma. After surgery, an adjuvant therapy of albumin-paclitaxel combined with gemcitabine regimen was given. No recurrence was found six months after the surgery, with follow-up still ongoing. This report aims to improve the awareness, diagnosis, and treatment of s-CCC.
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Affiliation(s)
| | | | - Jiang-Tao Li
- Department of Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
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12
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Ma S, Li C, Ma Y, Wang X, Zhang D, Lu Z. A retrospective study on the clinical and pathological features of hepatic sarcomatoid carcinoma: Fourteen cases of a rare tumor. Medicine (Baltimore) 2022; 101:e30005. [PMID: 35945746 PMCID: PMC9351887 DOI: 10.1097/md.0000000000030005] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/05/2023] Open
Abstract
Hepatic sarcomatoid carcinoma is a rare liver malignancy with atypical clinical symptoms and a high degree of malignancy. To improve the understanding of this disease, we collected the clinical and pathological data of 14 patients with hepatic sarcomatoid carcinoma admitted to the First Affiliated Hospital and Second Affiliated Hospital of Bengbu Medical College from 2011 to 2021 and reviewed the relevant literature. The clinical and pathological data of 14 patients with hepatic sarcomatoid carcinoma were collected from the electronic medical record system of the 2 hospitals. All clinical data were independently reviewed by 2 clinicians, and all pathological data were independently reviewed by 2 pathologists. At the same time, we reviewed the related literature on hepatic sarcomatoid carcinoma in Pubmed and CNKI. This group of 14 patients, 10 males and 4 females, aged 50-77 years. The main symptoms of the patients were abdominal pain, bloating, anorexia, fatigue or weight loss in the upper abdomen, and 3 patients were asymptomatic. On imaging, hepatic sarcomatoid carcinoma manifests as heterogeneous mass with irregular shape and unclear boundary, and computed tomography (CT)/magnetic resonance imaging (MRI) enhanced scan shows progressive or persistent heterogeneous enhancement, marginal enhancement or annular enhancement, and central necrosis. The pathological features of hepatic sarcomatoid carcinoma are the proliferation of spindle cells and pleomorphic cells, which alternate with acinar cells. Hepatic sarcomatoid carcinoma is more common in middle-aged and elderly patients, especially men, and has no characteristic clinical manifestations. Imaging examination and B-ultrasound-guided liver biopsy + immunohistochemistry can help diagnose. Radical surgery is the preferred method for hepatic sarcomatoid carcinoma, and postoperative adjuvant chemotherapy is expected to prolong patient survival.
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Affiliation(s)
- Shuoshuo Ma
- Department of General Surgery, The First Affiliated Hospital of Bengbu Medical Collage
| | - Chunshuang Li
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College
| | - Yang Ma
- Department of General Surgery, The First Affiliated Hospital of Bengbu Medical Collage
| | - Xiaolei Wang
- Department of Imaging, The First Affiliated Hospital of Bengbu Medical College, Bengbu, Anhui, China
| | - Dengyong Zhang
- Department of General Surgery, The First Affiliated Hospital of Bengbu Medical Collage
| | - Zheng Lu
- Department of General Surgery, The First Affiliated Hospital of Bengbu Medical Collage
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13
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Sheng X, Qin JM. Clinical diagnosis and treatment strategies for sarcomatoid intrahepatic cholangiocarcinoma. Shijie Huaren Xiaohua Zazhi 2022; 30:614-622. [DOI: 10.11569/wcjd.v30.i14.614] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Sarcomatoid intrahepatic cholangiocarcinoma (SiCCA) is a malignant tumor composed of mixed epithelial cells and mesenchymal cells derived from malignant bile duct cells. SiCCA has a low incidence and has no specific clinical manifestations and serological and imaging examinations. The diagnosis of SiCCA relies on histopathology and immunohistochemistry. The very high malignancy of SiCCA makes it prone to liver and other organ metastases. SiCCA has a poor prognosis because it has a low surgical resection rate, is easy to relapse shortly after surgery, and has no effective prevention and treatment measures. Comprehensive analysis integrating imaging, serum tumor markers, and histopathological examination technology is an important measure to improve the diagnosis of SiCCA and reduce the misdiagnosis rate. Early diagnosis, surgical treatment, and comprehensive postoperative treatment based on chemotherapy are the keys to improving the survival and prognosis of patients with SiCCA.
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Affiliation(s)
- Xia Sheng
- Department of Pathology, Minhang Hospital Affiliated to Fudan University, Shanghai 201100, China
| | - Jian-Min Qin
- Department of General Surgery, The Third Hospital Affiliated to Naval Military Medical University, Shanghai 201805, China
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14
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Lv TR, Hu HJ, Regmi P, Liu F, Li FY. Sarcomatoid hepatocellular carcinoma versus conventional hepatocellular carcinoma: a systematic review and meta-analysis. J Cancer Res Clin Oncol 2022; 148:1685-1696. [PMID: 35201426 DOI: 10.1007/s00432-022-03949-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2021] [Accepted: 02/06/2022] [Indexed: 02/05/2023]
Abstract
BACKGROUND Sarcomatoid hepatocellular carcinoma (SHCC) is a rare subtype of primary liver malignancies and is still ill-defined and poorly understood. Therefore, our study was performed to have a comprehensive evaluation SHCC versus conventional hepatocellular carcinoma (HCC). METHODS A thorough database searching was performed in PubMed, EMBASE and the Cochrane Library. RevMan5.3 and Stata 13.0 software were used for statistical analyses. The primary endpoint of our analysis is the long-term survival and the secondary endpoint is clinical and pathological features. RESULTS Four studies with a relative large cohort were finally identified. Compared with patients with pure HCC, patients with SHCC had a significantly worse overall survival (P < 0.00001) and disease-free survival (P < 0.0001). Moreover, a larger tumor size (P = 0.003), a higher incidence of node metastasis (P < 0.00001) and a higher proportion of advanced lesions (P = 0.04) were more frequently detected in patients with SHCC. Higher levels of serum ALT (P = 0.02) and TB (P = 0.005) were detected in patients with HCC rather than SHCC, while serum ALB (P = 0.02) level was relatively higher in patients with SHCC. For other measured outcomes, including concurrent viral hepatitis, liver cirrhosis, liver storage (Child A/B), multifocal tumors, vascular invasion and preoperative AFP level, the results showed no significant difference (P > 0.05). CONCLUSION SHCC has a worse prognosis and exhibits more aggressively than conventional HCC. Future large well-designed studies are demanded for further validation.
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Affiliation(s)
- Tian-Run Lv
- Department of Biliary Surgery, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China
| | - Hai-Jie Hu
- Department of Biliary Surgery, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China
| | - Parbatraj Regmi
- Department of Biliary Surgery, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China
| | - Fei Liu
- Department of Biliary Surgery, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China
| | - Fu-Yu Li
- Department of Biliary Surgery, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China.
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Younger NT, Wilson ML, Martinez Lyons A, Jarman EJ, Meynert AM, Grimes GR, Gournopanos K, Waddell SH, Tennant PA, Wilson DH, Guest RV, Wigmore SJ, Acosta JC, Kendall TJ, Taylor MS, Sproul D, Mill P, Boulter L. In Vivo Modeling of Patient Genetic Heterogeneity Identifies New Ways to Target Cholangiocarcinoma. Cancer Res 2022; 82:1548-1559. [PMID: 35074757 PMCID: PMC9359731 DOI: 10.1158/0008-5472.can-21-2556] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2021] [Revised: 12/14/2021] [Accepted: 01/18/2022] [Indexed: 01/07/2023]
Abstract
Intrahepatic cholangiocarcinoma (ICC) is an aggressive malignancy of the bile ducts within the liver characterized by high levels of genetic heterogeneity. In the context of such genetic variability, determining which oncogenic mutations drive ICC growth has been difficult, and developing modes of patient stratification and targeted therapies remains challenging. Here we model the interactions between rare mutations with more common driver genes and combine in silico analysis of patient data with highly multiplexed in vivo CRISPR-spCas9 screens to perform a functional in vivo study into the role genetic heterogeneity plays in driving ICC. Novel tumor suppressors were uncovered, which, when lost, cooperate with the RAS oncoprotein to drive ICC growth. Focusing on a set of driver mutations that interact with KRAS to initiate aggressive, sarcomatoid-type ICC revealed that tumor growth relies on Wnt and PI3K signaling. Pharmacologic coinhibition of Wnt and PI3K in vivo impeded ICC growth regardless of mutational profile. Therefore, Wnt and PI3K activity should be considered as a signature by which patients can be stratified for treatment independent of tumor genotype, and inhibitors of these pathways should be levied to treat ICC. SIGNIFICANCE This work shows that, despite significant genetic heterogeneity, intrahepatic cholangiocarcinoma relies on a limited number of signaling pathways to grow, suggesting common therapeutic vulnerabilities across patients.
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Affiliation(s)
- Nicholas T. Younger
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Mollie L. Wilson
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Anabel Martinez Lyons
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Edward J. Jarman
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Alison M. Meynert
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Graeme R. Grimes
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Konstantinos Gournopanos
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Scott H. Waddell
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Peter A. Tennant
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - David H. Wilson
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Rachel V. Guest
- Clinical Surgery, University of Edinburgh, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom
| | - Stephen J. Wigmore
- Clinical Surgery, University of Edinburgh, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom
| | - Juan Carlos Acosta
- Cancer Research UK Edinburgh Centre, Institute of Genetics and Cancer, Crewe Road South, Edinburgh, United Kingdom
| | - Timothy J. Kendall
- Centre for Inflammation Research, University of Edinburgh, Edinburgh, United Kingdom
| | - Martin S. Taylor
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Duncan Sproul
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Pleasantine Mill
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
| | - Luke Boulter
- MRC Human Genetics Unit, Institute of Genetics and Cancer, University of Edinburgh, United Kingdom
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Feng JY, Li XP, Wu ZY, Ying LP, Xin C, Dai ZZ, Shen Y, Wu YF. Sarcomatoid intrahepatic cholangiocarcinoma with good patient prognosis after treatment with Huaier granules following hepatectomy: A case report. World J Clin Cases 2022; 10:2829-2835. [PMID: 35434085 PMCID: PMC8968806 DOI: 10.12998/wjcc.v10.i9.2829] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Revised: 12/06/2021] [Accepted: 02/20/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Sarcomatoid intrahepatic cholangiocarcinoma (SICC) is an extremely rare and highly invasive malignant tumor of the liver. The precise pathologic mechanism of SICC has not been clearly identified, and the prognosis is very poor. The effectiveness of the treatment strategy of radical hepatectomy combined with Huaier granules has not yet been reported.
CASE SUMMARY The patient was a 69-year-old male who presented with intermittent right upper abdominal pain for one month and 4-pound weight loss before admission. Abdominal magnetic resonance imaging and magnetic resonance cholangiopancreatography showed multiple stones in the bile ducts accompanied by dilatation of the intrahepatic and extrahepatic bile ducts. The preoperative diagnoses were right intrahepatic bile duct stones and extrahepatic bile duct stones; thus, surgical resection was performed. Choledochoscopy showed that the bile duct wall of the right anterior lobe was thickened, and a mass was visible in the duct. Then, a biopsy was performed, and rapid frozen-section biopsy analysis indicated that the tumor was malignant. The final diagnosis was SICC (T1aN0M0). Huaier granules were taken by the patient as anticancer therapy after surgery. The patient attended follow-up for 72 mo with no tumor recurrence or metastasis.
CONCLUSION Sarcomatous intrahepatic cholangiocarcinoma is an extremely rare, aggressive malignancy, and the diagnostic gold standard is pathological diagnosis. We reported the first case of successful treatment with Huaier granules as anticancer therapy after surgery, which indicated that Huaier granules are safe and effective. Further studies are needed to study the anticancer molecular mechanisms of Huaier granules in sarcomatous intrahepatic cholangiocarcinoma.
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Affiliation(s)
- Ji-Ye Feng
- Department of Hepatobiliary & Pancreas Surgery, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Xian-Peng Li
- Department of Infectious Disease, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Zong-Yang Wu
- Department of Hepatobiliary & Pancreas Surgery, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Li-Ping Ying
- Department of Hepatobiliary & Pancreas Surgery, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Chang Xin
- Department of Hepatobiliary & Pancreas Surgery, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Zhen-Zhen Dai
- Department of Pathology, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Yao Shen
- Department of Radiology, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
| | - Yi-Feng Wu
- Department of Hepatobiliary & Pancreas Surgery, The Affiliated People’s Hospital of Ningbo University, Ningbo 315040, Zhejiang Province, China
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Vij M, Puri Y, Rammohan A, G G, Rajalingam R, Kaliamoorthy I, Rela M. Pathological, molecular, and clinical characteristics of cholangiocarcinoma: A comprehensive review. World J Gastrointest Oncol 2022; 14:607-627. [PMID: 35321284 PMCID: PMC8919011 DOI: 10.4251/wjgo.v14.i3.607] [Citation(s) in RCA: 20] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/25/2021] [Revised: 12/13/2021] [Accepted: 02/23/2022] [Indexed: 02/06/2023] Open
Abstract
Cholangiocarcinomas are a heterogeneous group of highly aggressive cancers that may arise anywhere within the biliary tree. There is a wide geographical variation with regards to its incidence, and risk-factor associations which may include liver fluke infection, primary sclerosing cholangitis, and hepatolithiasis amongst others. These tumours are classified into intrahepatic, perihilar and distal based on their anatomical location. Morphologically, intrahepatic cholangiocarcinomas are further sub-classified into small and large duct variants. Perihilar and distal cholangiocarcinomas are usually mucin-producing tubular adenocarcinomas. Cholangiocarcinomas develop through a multistep carcinogenesis and are preceded by dysplastic and in situ lesions. While clinical characteristics and management of these tumours have been extensively elucidated in literature, their ultra-structure and tumour biology remain relatively unknown. This review focuses on the current knowledge of pathological characteristics, molecular alterations of cholangiocarcinoma, and its precursor lesions (including biliary intraepithelial neoplasia, intraductal papillary neoplasms of the bile duct, intraductal tubulopapillary neoplasms and mucinous cystic neoplasm).
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Affiliation(s)
- Mukul Vij
- Department of Pathology, Dr Rela Institute and Medical center, Chennai 600044, Tamil Nadu, India
| | - Yogesh Puri
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
| | - Ashwin Rammohan
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
| | - Gowripriya G
- Department of Pathology, Dr Rela Institute and Medical center, Chennai 600044, Tamil Nadu, India
| | - Rajesh Rajalingam
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
| | - Ilankumaran Kaliamoorthy
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
| | - Mohamed Rela
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Center, Chennai 600044, Tamil Nadu, India
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18
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Ji W, Xing Y, Ma J, Zhao Z, Xu H, Zheng S, Li W, Li X. Primary Liver Sarcomatoid Carcinoma: A Case Series and Literature Review. J Hepatocell Carcinoma 2021; 8:1117-1127. [PMID: 34522692 PMCID: PMC8434859 DOI: 10.2147/jhc.s325182] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2021] [Accepted: 08/25/2021] [Indexed: 12/12/2022] Open
Abstract
Background Primary liver sarcomatoid carcinoma (PLSC) is rare. To improve the understanding of PLSC, cases were described and reviewing the literature. Methods A retrospective analysis was performed on 14 cases of PLSC diagnosed by pathology in Northeastern China from 2010 to 2020. An individual participant data analysis based on reported cases was conducted to determine epidemiological characteristics, clinical characteristics, and prognoses of PLSC. Results A total of 136 cases involved our 14 cases and 122 cases from previous reports. The percentages of sarcomatoid hepatocellular carcinoma, sarcomatoid cholangiolocellular carcinoma, and mixed and unclassified types were 36.8%, 41.9%, 5.9%, and 15.4%, respectively. A total of 95.6% PLSC was found in Asia. There was a lower percentage of hepatitis-infected patients in Japan, when compared with the Republic of Korea (38.5% vs 70.0%, P<0.05). Five cases were initially misdiagnosed as a hepatic abscess by imaging. A total of 36.7% cases had metastases when being diagnosed, and 68.9% cases relapsed during the follow-up. The median disease-free survival and overall survival (OS) were 3 months and 5 months, respectively. Only radical surgery (hazard ratio = 0.308, 95% confidence interval 0.186–0.512, P<0.001) indicated a better OS. Conclusion PLSC was more prevalent in Asia and there were possibilities of misdiagnoses. Surgery is still an effective treatment and can significantly prolong the OS. Only limited strategies for recurrent or advanced PLSC, immunotherapy may be possible treatment.
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Affiliation(s)
- Wei Ji
- Cancer Center, The First Hospital of Jilin University, Changchun, People's Republic of China
| | - Yunlong Xing
- Plastic Surgery, China-Japan Union Hospital, Jilin University, Changchun, People's Republic of China
| | - Jinshu Ma
- Department of Pathology, China-Japan Union Hospital, Jilin University, Changchun, People's Republic of China
| | - Zhuo Zhao
- Cardiology Department, The Second Hospital of Jilin University, Changchun, People's Republic of China
| | - Hongqin Xu
- Department of Hepatology, The First Hospital of Jilin University, Changchun, People's Republic of China
| | - Shuang Zheng
- Department of Radiology, The First Hospital of Jilin University, Changchun, People's Republic of China
| | - Wei Li
- Cancer Center, The First Hospital of Jilin University, Changchun, People's Republic of China
| | - Xu Li
- Department of Hepatology, The First Hospital of Jilin University, Changchun, People's Republic of China
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19
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Zhang H, Chai S, Chen L, Wang Y, Cheng Y, Fang Q, Wu G, Wang X, Liang W, Xiao W. MRI Features of Hepatic Sarcomatoid Carcinoma Different From Hepatocellular Carcinoma and Intrahepatic Cholangiocarcinoma. Front Oncol 2021; 11:611738. [PMID: 34221954 PMCID: PMC8247642 DOI: 10.3389/fonc.2021.611738] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2020] [Accepted: 05/25/2021] [Indexed: 12/25/2022] Open
Abstract
Introduction Hepatic sarcomatoid carcinoma (HSC) is a rare type of liver cancer with a high malignant grade and poor prognosis. This study compared the clinical characteristics and magnetic resonance imaging (MRI) features of HSCs with those of hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC), aiming to identify valuable features for HSC diagnosis. Methods In total, 17 pathologically confirmed HSC cases, 50 HCC cases and 50 common ICC cases were enrolled from two hospitals. The clinical characteristics and MRI features of all cases were summarized and statistically analyzed. Results On the one hand, the incidence rates of elevated carbohydrate antigen (CA) 19-9 and elevated carcinoembryonic antigen (CEA) were significantly higher in the HSC cases than in the HCC cases (29.4% vs. 0%; 17.6% vs. 0%). The HSC enhancement patterns, primarily including progressive enhancement, were also significantly different from HCC cases. The incidence rates of heterogeneous signals on T2-weighted imaging and during the arterial phase were significantly higher in the HSC cases than in the HCC cases (94.1% vs. 66.0%; 100.0% vs. 72.0%). The diameter of HSCs was significantly larger than that in the HCC cases (6.12 cm vs. 4.21 cm), and the incidence rates of adjacent cholangiectasis, intrahepatic metastasis and lymph node enlargement were considerably higher in the HSC cases than in the HCC cases (52.9% vs. 6.0%; 47.1% vs. 12.0%; 41.2% vs. 2.0%). On the other hand, the incidence rate of elevated CA199 was significantly lower in the HSC cases than in the ICC cases (29.4% vs. 60.0%). The incidence rates of intratumoral necrosis and pseudocapsules were significantly higher in the HSC cases than in the HCC cases (35.3% vs. 8.0%; 47.1% vs. 12.0%). However, the incidence rates of target signs were significantly lower in the HSC cases than in the HCC cases (11.8% vs. 42.0%). In addition, there was no significant difference in the enhancement patterns between HSC cases and ICC cases. Conclusions HSCs were frequently seen in elderly men with clinical symptoms and elevated CA199 levels. The MRI features, including large size, obvious heterogeneity, hemorrhage, progressive enhancement, pseudocapsule and lymph node enlargement, contributed to the diagnosis of HSC.
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Affiliation(s)
- Hongbin Zhang
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Siyuan Chai
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Lintao Chen
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Yubizhuo Wang
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Yongna Cheng
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Quan Fang
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Guosen Wu
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Xiangming Wang
- Department of Radiology, Yiwu Central Hospital, Yiwu, China
| | - Wenjie Liang
- Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Wenbo Xiao
- Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
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Tang Y, Zhang T, Zhao Y, Chen Z, Ma X. Development and validation of a comprehensive radiomics nomogram for prognostic prediction of primary hepatic sarcomatoid carcinoma after surgical resection. Int J Med Sci 2021; 18:1711-1720. [PMID: 33746587 PMCID: PMC7976557 DOI: 10.7150/ijms.53602] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2020] [Accepted: 01/06/2021] [Indexed: 02/05/2023] Open
Abstract
Objective: This study aimed to establish and validate a radiomics nomogram comprised of clinical factors and radiomics signatures to predict prognosis of primary hepatic sarcomatoid carcinoma (PHSC) patients after surgical resection. Methods: In this retrospective study, 79 patients with pathological confirmation of PHSC and underwent surgical resection were recruited. A radiomics nomogram was developed by radiomics signatures and independent clinical risk factors selecting from multivariate Cox regression. All patients were stratified as high risk and low risk by nomogram. Model performance and clinical usefulness were assessed by C-index, calibration curve, decision curve analysis (DCA) and survival curve. Results: A total of 79 PHSC were included with 1-year and 3-year overall survival rates of 63.3% and 35.4%, respectively. The least absolute shrinkage and selection operator (LASSO) method selected 3 features. Multivariate Cox analysis found six independent prognostic factors. The radiomics nomogram showed a significant prediction value with overall survival (HR: 7.111, 95%CI: 3.933-12.858, P<0.001). C-index of nomogram was 0.855 and 0.829 in training and validation set, respectively. Decision curve analysis validated the clinical utility of this nomogram. There was a significant difference in the 1-year and 3-year survival rates of stratified high-risk and low-risk patients in the whole cohort (30.6% vs. 90.1% and 5.6% vs. 62.4%, respectively, P < 0.001). Conclusion: This radiomics nomogram serve as a potential tool for predicting prognosis of PHSC after surgical resection, and help to identify high risk patients who may obtain feeble survival benefit from surgical resection.
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Affiliation(s)
- Youyin Tang
- Department of Liver Surgery, Liver Transplantation Center, West China Hospital of Sichuan University, No. 37 GuoXue Alley, Chengdu 610041, China
| | - Tao Zhang
- West China School of Medicine, West China Hospital, Sichuan University, No. 37 GuoXue Alley, Chengdu 610041, China
| | - Yunuo Zhao
- Department of Biotherapy, West China Hospital and State Key Laboratory of Biotherapy, Sichuan University, No. 37 GuoXue Alley, Chengdu 610041, China
| | - Zheyu Chen
- Department of Liver Surgery, Liver Transplantation Center, West China Hospital of Sichuan University, No. 37 GuoXue Alley, Chengdu 610041, China
| | - Xuelei Ma
- Department of Biotherapy, West China Hospital and State Key Laboratory of Biotherapy, Sichuan University, No. 37 GuoXue Alley, Chengdu 610041, China
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21
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Li X, Li J, Liu K, Tan L, Liu Y. Sarcomatoid intrahepatic cholangiocarcinoma in a patient with poor prognosis: a case report and literature review. J Int Med Res 2020; 48:300060520969473. [PMID: 33161814 PMCID: PMC7658526 DOI: 10.1177/0300060520969473] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Sarcomatoid intrahepatic cholangiocarcinoma (S-iCCA) is a rare histological variant of intrahepatic cholangiocarcinoma (iCCA). The diagnosis of S-iCCA is based on histopathological and immunohistochemical examinations, and S-iCCA often has a poorer prognosis than that of ordinary iCCA. In this article, we present the case of a 64-year-old man with S-iCCA who presented with intermittent right upper abdominal pain. The aim of this case report and literature review is to strengthen the understanding of S-iCCA among clinicians and reduce the incidence of missed clinical diagnoses.
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Affiliation(s)
- Xu Li
- Department of Hepatology, The First Hospital of Jilin University, Changchun, China, Jilin Province, China
| | - Jian Li
- Department of Hepatology, The First Hospital of Jilin University, Changchun, China, Jilin Province, China
| | - Kai Liu
- Department II of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun, China, Jilin Province, China
| | - Ludong Tan
- Department II of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun, China, Jilin Province, China
| | - Yahui Liu
- Department II of Hepatobiliary and Pancreatic Surgery, The First Hospital of Jilin University, Changchun, China, Jilin Province, China
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22
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Wang T, Yang X, Wang W. Another case of hepatic alveolar echinococcosis? Asian J Surg 2020; 43:1119-1120. [PMID: 32893118 DOI: 10.1016/j.asjsur.2020.08.013] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Accepted: 08/20/2020] [Indexed: 02/08/2023] Open
Affiliation(s)
- Tao Wang
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, PR China
| | - Xianwei Yang
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, PR China
| | - Wentao Wang
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, PR China.
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Wang JP, Yao ZG, Sun YW, Liu XH, Sun FK, Lin CH, Ren FX, Lv BB, Zhang SJ, Wang Y, Meng FY, Zheng SZ, Gong W, Liu J. Clinicopathological characteristics and surgical outcomes of sarcomatoid hepatocellular carcinoma. World J Gastroenterol 2020; 26:4327-4342. [PMID: 32848337 PMCID: PMC7422543 DOI: 10.3748/wjg.v26.i29.4327] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2020] [Revised: 04/24/2020] [Accepted: 07/14/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Hepatocellular carcinoma (HCC) is the sixth most common type of cancer and the fourth leading cause of cancer-related death worldwide. Sarcomatoid HCC, which contains poorly differentiated carcinomatous and sarcomatous components, is a rare histological subtype of HCC that differs from conventional HCC. It is highly aggressive and has a poor prognosis. Its clinicopathological characteristics, surgical outcomes and underlying mechanisms of its highly aggressive nature have not been fully elucidated.
AIM To examine the clinicopathological characteristics and surgical outcomes of sarcomatoid HCC and explore the histogenesis of sarcomatoid HCC.
METHODS In total, 196 patients [41 sarcomatoid HCC and 155 high-grade (Edmondson-Steiner grade III or IV) HCC] who underwent surgical resection between 2007 and 2017 were retrospectively reviewed. The characteristics and surgical outcomes of sarcomatoid HCC were compared with those of patients with high-grade HCC. The histological composition of invasive and metastatic sarcomatoid HCCs was evaluated.
RESULTS Sarcomatoid HCC was more frequently diagnosed at an advanced stage with a larger tumor and higher rates of nonspecific symptom, adjacent organ invasion and lymph node metastasis than high-grade HCC (all P < 0.05). Compared with high-grade HCC patients, sarcomatoid HCC patients are less likely to have typical dynamic imaging features of HCC (44.4% vs 72.7%, P = 0.001) and elevated serum alpha-fetoprotein levels (> 20 ng/mL; 36.6% vs 78.7%, P < 0.001). The sarcomatoid group had a significantly shorter median recurrence-free survival (5.6 mo vs 16.4 mo, log-rank P < 0.0001) and overall survival (10.5 mo vs 48.1 mo, log-rank P < 0.0001) than the high-grade group. After controlling for confounding factors, the sarcomatoid subtype was identified as an independent predictor of poor prognosis. Pathological analyses indicated that invasive and metastatic lesions were mainly composed of carcinomatous components.
CONCLUSION Sarcomatoid HCC was associated with a more advanced stage, atypical dynamic imaging, lower serum alpha-fetoprotein levels and a worse prognosis. The highly aggressive nature of sarcomatoid HCC is perhaps mediated by carcinomatous components.
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Affiliation(s)
- Jian-Ping Wang
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong Province, China
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Zhi-Gang Yao
- Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Ya-Wen Sun
- Cancer Prevention and Control Research Office of Shandong Province, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan 250117, Shandong Province, China
| | - Xi-Han Liu
- Cheeloo College of Medicine, Shandong University, Jinan 250012, Shandong Province, China
| | - Feng-Kai Sun
- Department of Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Cun-Hu Lin
- Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Fu-Xin Ren
- Shandong Medical Imaging Research Institute, Shandong University, Jinan 250021, Shandong Province, China
| | - Bei-Bei Lv
- Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Shuai-Jun Zhang
- Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Yang Wang
- Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Fan-Ying Meng
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong Province, China
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Shun-Zhen Zheng
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong Province, China
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Wei Gong
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong Province, China
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Jun Liu
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong Province, China
- Department of Liver Transplantation and Hepatobiliary Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
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Wang Y, Ming JL, Ren XY, Qiu L, Zhou LJ, Yang SD, Fang XM. Sarcomatoid intrahepatic cholangiocarcinoma mimicking liver abscess: A case report. World J Clin Cases 2020; 8:208-216. [PMID: 31970189 PMCID: PMC6962087 DOI: 10.12998/wjcc.v8.i1.208] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2019] [Revised: 11/26/2019] [Accepted: 11/30/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Sarcomatoid intrahepatic cholangiocarcinoma (SICC) is an extremely rare and highly invasive malignant tumor of the liver. To our knowledge, the imaging findings of sarcomatous cholangiocarcinoma have been rarely reported; and radiological features of this tumor mimicking liver abscess have not yet been reported.
CASE SUMMARY We present a case of SICC mimicking liver abscess. The patient, a 43-year-old male, complained of repeated upper right abdominal discomfort and intermittent distension over a period of one month. Radiology examination revealed a huge focal lesion in the right liver. The lesion was hypointense on computed tomography with honeycomb enhancement surrounded by enhanced peripheral areas. It showed a hypo-signal on non-contrast T1-weighted images and a hyper-signal on non-contrast T2-weighted images. Radiologists diagnosed the lesion as an atypical liver abscess. The patient underwent a hepatectomy. After surgery, he survived another 2.5 mo before passing away. A search of PubMed and Google revealed 43 non-repeated cases of SICC reported in 20 published studies. The following is a short review in order to improve the diagnostic and therapeutic skills in cases of SICC.
CONCLUSION This report presents the clinical and radiological features of SICC and imaging features which showed hypovascularity and progressive enhancement. SICC can present as a multilocular cyst on radiological images and it is necessary to distinguish this lesion from an atypical abscess. Simple surgical treatment is not the best treatment option for this disease.
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Affiliation(s)
- Yan Wang
- Department of Radiology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
| | - Jia-Lei Ming
- Department of Radiology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
| | - Xing-Yu Ren
- Department of Radiology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
| | - Lu Qiu
- Department of Radiology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
| | - Li-Juan Zhou
- Department of Radiology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
| | - Shu-Dong Yang
- Department of Pathology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
| | - Xiang-Ming Fang
- Department of Radiology, The Affiliated Wuxi People’s Hospital of Nanjing Medical University, Wuxi 214023, Jiangsu Province, China
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Wang T, Kong J, Yang X, Shen S, Zhang M, Wang W. Clinical features of sarcomatoid change in patients with intrahepatic cholangiocarcinoma and prognosis after surgical liver resection: A Propensity Score Matching analysis. J Surg Oncol 2019; 121:524-537. [PMID: 31867746 DOI: 10.1002/jso.25815] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2019] [Accepted: 12/10/2019] [Indexed: 02/06/2023]
Abstract
BACKGROUND Intrahepatic cholangiocarcinoma with sarcomatoid change (iCCA-SC) is a rare histological subtype of iCCA, the clinical features and outcomes after surgical resection on the prognosis is still unknown. METHODS We retrospectively reviewed the clinical data of patients with histologically proven iCCA who underwent curative liver resection at our hospital between January 2008 and December 2018. Propensity score matching analysis was used to match patients with and without sarcomatoid change at a ratio of 1:4. The nomogram integrating all significant independent factors for overall survival (OS) and recurrence-free survival (RFS) was constructed to predict prognosis for iCCA. The predictive accuracy ability of the nomogram was determined by Harrell's index (C-index). RESULTS A total of 40 iCCA-SC and 160 ordinary iCCA patients were included in this study. RFS and OS in the iCCA-SC group were significantly lower than those in the ordinary iCCA group (P<.001 and P = .002, respectively). The calibration curve for the probability of survival showed good agreement between the nomogram prediction and actual observation. CONCLUSION The histological sarcomatoid subtype is an independent predictor of tumor recurrence and shorter OS in iCCA patients. The nomogram we established could provide more accurate prognostic prediction for iCCA patients.
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Affiliation(s)
- Tao Wang
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, China
| | - Junjie Kong
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, China
| | - Xianwei Yang
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, China
| | - Shu Shen
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, China
| | - Menglan Zhang
- Department of Pathology, West China Hospital of Sichuan University, Chengdu, China
| | - Wentao Wang
- Department of Liver Surgery and Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, China
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