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1
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Sapuppo E, Brunetti O, Tessitore D, Brandi G, Di Giovanni N, Fadda G, Luchini C, Martini M, Quaresmini D, Russo A, Santarpia M, Scarpa A, Scartozzi M, Tuccari G, Franchina T, Silvestris N. Rare histotypes of epithelial biliary tract tumors: A literature review. Crit Rev Oncol Hematol 2023; 181:103892. [PMID: 36481306 DOI: 10.1016/j.critrevonc.2022.103892] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2022] [Revised: 11/21/2022] [Accepted: 12/01/2022] [Indexed: 12/12/2022] Open
Abstract
Adenocarcinoma represents the most frequent biliary tract cancer. However, other rare histotypes can be found in the biliary tract, such as cholangiolocellular carcinoma, cholangiocarcinoma with ductal plate malformation pattern, adenosquamous carcinoma, mucinous carcinoma, signet ring cell carcinoma, clear cell carcinoma, mucoepidermoid carcinoma, lymphoepithelioma-like carcinoma, and sarcomatous cholangiocarcinoma. These cancer types account for less than 10 % of all the already rare biliary tract tumors. Yet, they represent a relevant issue in everyday clinical practice, given the lack of therapeutic recommendations and the overall scarcity of data, mainly deriving from isolated small center-specific cohorts of patients.The shifts of such histotypes from the most common ones reflect genetic and molecular differences, determine changes in clinical aggressiveness, and suggest a possible variability in sensitivity to the standard treatments of biliary adenocarcinomas. The consistency and degree of these variables are still to be solidly demonstrated and investigated. Therefore, this paper aims to review the current literature concerning very infrequent and rare epithelial biliary tract cancers, focusing our attention on the clinical, molecular, and immunohistochemical features of these tumors.
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Affiliation(s)
- Elena Sapuppo
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, Messina, Italy
| | - Oronzo Brunetti
- Medical Oncology Unit-IRCCS Istituto Tumori "Giovanni Paolo II" of Bari, 70124 Bari, Italy
| | - Dalila Tessitore
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, Messina, Italy
| | - Giovanni Brandi
- Haematological and Oncological Institute, Department of Experimental, Diagnostic and Specialty Medicine, St. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
| | - Nicola Di Giovanni
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, Messina, Italy
| | - Guido Fadda
- Department of Human Pathology in Adult and Developmental Age "Gaetano Barresi", Section of Pathology, University of Messina, Messina, Italy
| | - Claudio Luchini
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy; ARC-Net Research Center, University and Hospital Trust of Verona, Verona, Italy
| | - Maurizio Martini
- Department of Human Pathology in Adult and Developmental Age "Gaetano Barresi", Section of Pathology, University of Messina, Messina, Italy
| | - Davide Quaresmini
- Medical Oncology Unit-IRCCS Istituto Tumori "Giovanni Paolo II" of Bari, 70124 Bari, Italy
| | - Antonio Russo
- Department of Surgical, Oncological and Oral Sciences, Section of Medical Oncology, University of Palermo, Palermo, Italy
| | - Mariacarmela Santarpia
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, Messina, Italy
| | - Aldo Scarpa
- Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy; ARC-Net Research Center, University and Hospital Trust of Verona, Verona, Italy
| | - Mario Scartozzi
- Azienda Ospedaliera Universitaria (AOU) Cagliari, University Hospital and University of Cagliari, Cagliari, Italy
| | - Giovanni Tuccari
- Department of Human Pathology in Adult and Developmental Age "Gaetano Barresi", Section of Pathology, University of Messina, Messina, Italy
| | - Tindara Franchina
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, Messina, Italy
| | - Nicola Silvestris
- Medical Oncology Unit, Department of Human Pathology "G. Barresi", University of Messina, Messina, Italy.
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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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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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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] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Accepted: 09/28/2020] [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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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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Zhang N, Li Y, Zhao M, Chang X, Qu Q, He X. Sarcomatous carcinoma in biliary system: A retrospective study. Medicine (Baltimore) 2019; 98:e14585. [PMID: 30813178 PMCID: PMC6407967 DOI: 10.1097/md.0000000000014585] [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: 11/25/2022] Open
Abstract
Sarcomatous carcinoma in biliary system, including sarcomatous intrahepatic cholangiocarcinoma (SIC) and sarcomatous choledochal carcinoma (SCC), is extremely rare and malignant.This retrospective study included 5 patients with SIC and 4 patients with SCC. Their basic characteristics, preoperative lab tests, preoperative imaging features, perioperative status, and follow-up information have been collected and analyzed.Lesions at different locations induced various preoperative symptoms. The history of choledocholithiasis or hepatolithiasis was remarkable in patients with SIC. Cancer antigen 19-9 appeared to be a key factor for both SIC and SCC. However, preoperative lab tests or imaging features could not distinguish SIC from intrahepatic cholangiocarcinoma, or SCC from choledochal carcinoma. Surgical treatments for all 9 patients were successful. Efficacy of adjuvant chemotherapy was not ideal. The prognosis of sarcomatous biliary carcinoma was enormously poor.Sarcomatous carcinoma in biliary system is extremely rare and malignant. Chronic inflammation could be critical in the currently unknown occurrence mechanism. Further research is urgently needed to improve the prognosis.
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Affiliation(s)
| | | | | | - Xiaoyan Chang
- Department of Pathology, Peking Union Medical College Hospital, Beijing, China
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Yugawa K, Yoshizumi T, Mano Y, Harada N, Itoh S, Ikegami T, Soejima Y, Fujita N, Kohashi K, Aishima S, Oda Y, Mori M. Primary intrahepatic cholangiocarcinoma with sarcomatous stroma: case report and review of the literature. Surg Case Rep 2018; 4:138. [PMID: 30478801 PMCID: PMC6261094 DOI: 10.1186/s40792-018-0543-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2018] [Accepted: 11/12/2018] [Indexed: 12/16/2022] Open
Abstract
Background Hepatic carcinosarcomas, which include both carcinomatous and sarcomatous elements, are uncommon in adults. Although carcinosarcoma in hepatocellular carcinoma is occasionally reported, carcinosarcoma in intrahepatic cholangiocarcinoma (ICC) is an extremely rare ICC variant. Few such cases have been reported in English and no large study of its clinicopathological features exists. Case presentation Here, we report a 60-year-old man with an asymptomatic hepatic B infection who developed hepatic carcinosarcoma from an otherwise normal liver. The 6.0-cm tumor was accidentally discovered by PET-CT in a cancer examination. Serum examinations showed no elevation of tumor markers. He underwent left and caudate lobectomy of the liver. The diagnosis of intrahepatic cholangiocarcinoma with sarcomatous stroma was based on thorough pathologic examination and immunohistochemical staining. The tumor exhibited adenocarcinomatous and sarcomatous components; the adenocarcinomatous element was positive for epithelial markers, the sarcomatous element was positive for mesenchymal markers, but negative for epithelial markers. The patient made an uneventful recovery after surgery. At present, 14 months after surgery, he remains well with no evidence of tumor recurrence. Conclusions We report an unusual case of hepatic carcinosarcoma (intrahepatic cholangiocarcinoma with sarcomatous stroma) and discuss the etiology and prognosis of this rare disease.
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Affiliation(s)
- Kyohei Yugawa
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan. .,Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan.
| | - Tomoharu Yoshizumi
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Yohei Mano
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Noboru Harada
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Shinji Itoh
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Toru Ikegami
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Yuji Soejima
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Nobuhiro Fujita
- Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Kenichi Kohashi
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Shinichi Aishima
- Department of Pathology and Microbiology, Faculty of Medicine, Saga University Hospital, Saga, Japan
| | - Yoshinao Oda
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Masaki Mori
- Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan
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8
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Zhang N, Li Y, Zhao M, Chang X, Tian F, Qu Q, He X. Sarcomatous intrahepatic cholangiocarcinoma: Case report and literature review. Medicine (Baltimore) 2018; 97:e12549. [PMID: 30278551 PMCID: PMC6181610 DOI: 10.1097/md.0000000000012549] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Accepted: 09/03/2018] [Indexed: 02/06/2023] Open
Abstract
RATIONALE Sarcomatous intrahepatic cholangiocarcinoma is a rare histological variant of cholangiocarcinoma (ICC). Previous medical literature has not mentioned the prevalence of this kind of disease, but a poorer prognosis than that of ordinary ICC was indicated. The diagnosis of the sarcomatous ICC is established on histopathological and immunohistochemical examinations. In this article, we present a new case of a patient with sarcomatous ICC who had no radiographic sign of intrahepatic tumor preoperatively. PATIENT CONCERNS A 63-year-old man was noted with cholecystolithiasis and right upper abdominal pain. Liver function was within normal limits, although the gamma-glutamyl transpeptidase level was elevated. Serum carbohydrate antigen 19-9 level was elevated. Radiography showed atrophy of the left lobe of the liver, high-intensity signals on T1 weighted images, and low/high-intensity signals on T2 weighted images in hepatic ducts. DIAGNOSES The preoperative diagnoses were hepatolithiasis, choledocholithiasis, and cholecystolithiasis. INTERVENTIONS Exploratory laparotomy, adhesion release, cholecystectomy, choledocholithotomy, and T tube drainage were performed. During the surgery, an ill-defined tumor was detected on the atrophic left lateral lobe of the liver. Hepatic left lateral lobectomy was performed to remove the mass. OUTCOMES The final diagnosis of sarcomatous ICC was made by histopathology after surgery. No evidence of local recurrence or distant metastasis was noted on imaging during follow-up. LESSONS Although rare, sarcomatous ICC does exist in patients presented with cholecystolithiasis and liver atrophy. Surgeons should be aware of the existence of sarcomatous ICC due to the poor prognosis. We recommend that multidisciplinary approaches may be key to improve prognosis, including adjuvant chemotherapy or radiotherapy.
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Affiliation(s)
| | | | | | - Xiaoyan Chang
- Department of Pathology, Peking Union Medical College Hospital, Beijing, China
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9
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Watanabe G, Uchinami H, Yoshioka M, Nanjo H, Yamamoto Y. Prognosis analysis of sarcomatous intrahepatic cholangiocarcinoma from a review of the literature. Int J Clin Oncol 2013; 19:490-6. [PMID: 23824556 DOI: 10.1007/s10147-013-0586-x] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2012] [Accepted: 06/06/2013] [Indexed: 02/06/2023]
Abstract
BACKGROUND Sarcomatous intrahepatic cholangiocarcinoma (ICC) is a rare histological variant of ICC. The prognosis of sarcomatous ICC is poorly understood. METHODS We analyzed the prognosis of sarcomatous ICC by reviewing the previous reports and our own case. RESULTS Only 15 cases of sarcomatous ICC have been reported in the English-language literature so far. Median survival time of patients with sarcomatous ICC with and without surgery was 11 and 3 months, respectively. Survival rate of patients operated on for sarcomatous ICC was similar to that of patients with ordinary ICC without surgery in the early postoperative period. In the long-term view, however, the prognosis for the patients with sarcomatous ICC receiving surgery was better than that for the patients with ordinary ICC without surgery. CONCLUSION Although the prognosis for the patients with sarcomatous ICC was poor even after curative resection, surgery would be justified as the primary treatment for sarcomatous ICC.
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Affiliation(s)
- Go Watanabe
- Department of Gastroenterological Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, 010-8543, Japan,
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10
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Primary liver sarcomatous carcinoma: report of two cases and review of the literature. Pathol Res Pract 2013; 209:249-54. [PMID: 23484778 DOI: 10.1016/j.prp.2013.01.005] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/17/2012] [Revised: 11/28/2012] [Accepted: 01/02/2013] [Indexed: 01/12/2023]
Abstract
Primary liver sarcomatous carcinomas (PLSCs) are very aggressive tumors. They are characterized by a fast clinical course, and therefore need a prompt histological diagnosis. Here, we report two cases of PLSC. One arises in a non-cirrhotic liver and the other in cirrhosis, with differences in onset and histological features. Special emphasis is put on the expression of albumin and HCC markers, and their possible usefulness in the diagnosis. The English literature of the last 20 years was revised (92 cases). Immunohistochemistry was performed manually or automatically; in situ hybridization (ISH) technique for albumin mRNA detection was carried out. The sarcomatoid components in both cases were immunoreactive for K8/18, Glutamine Synthetase and EZH2, and negative for Glypican 3, SMA, caldesmon, desmin, DOG-1, CD34, CD31, CD117, CD56, and alpha-fetoprotein. The detection of albumin mRNA by ISH was negative in the sarcomatoid component in both cases. PLSC represents a diagnostic challenge for pathologists, especially in its "pure" form: neither albumin mRNA detection nor HCC markers are useful for the diagnosis: positivity for K8/18 and the negativity for the mesenchymal markers seem to represent the main tools for the histological diagnosis.
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Zhan Q, Shen BY, Deng XX, Zhu ZC, Chen H, Peng CH, Li HW. Clinical and pathological analysis of 27 patients with combined hepatocellular-cholangiocarcinoma in an Asian center. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2013; 19:361-9. [PMID: 21744084 DOI: 10.1007/s00534-011-0417-2] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
BACKGROUND Our purpose was to assess the clinicopathological features and surgical outcomes of combined hepatocellular-cholangiocarcinoma (HCC-CC) in an Asian center. METHODS Between 1998 and 2009, 27 patients were diagnosed with combined HCC-CC at our hospital. Their medical records were reviewed and clinicopathological data retrospectively analyzed. RESULTS The 27 patients included 24 (88.9%) males and 3 (11.1%) females with a mean age of 58.26 ± 11.18 years. Cirrhosis was present in 10 patients (37.0%), and 12 patients had hepatitis C or hepatitis B virus infection. Serum alpha fetoprotein was >20 ng/ml in 7 of the 19 patients in whom it was measured (36.8%). Twenty-five patients underwent hepatic resections and 2 received liver transplantations. Five (18.5%) patients had separate HCC and CC within the same liver (type I), 21 (77.8%) had tumors with mixed components (type II), and 1 patient had a type III tumor (3.7%). Of 22 patients with immunohistochemical data, 19 (86.4%) were cytokeratin (CK) 7-positive, 20 (90.9%) were CK19-positive, and 4 (18.2%) were CK20-positive. Mean follow-up was 25.8 months. The 1- and 2-year survival rates were 72.5 and 49.4%, respectively. The 1- and 2-year disease-free survival rates were 54.2 and 41.3%, respectively. Symptoms at the time of diagnosis, and regional lymph node metastases, were associated with higher mortality and recurrence. CONCLUSIONS Lymph node metastasis and positive resection margins are important factors affecting HCC-CC surgical outcomes.
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Affiliation(s)
- Qian Zhan
- General Surgical Department, Ruijin Hospital Affiliated to Shanghai Jiaotong University, 197 Ruijin Er Road, Shanghai 200025, China
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12
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Hu X, Chen H, Jin M, Wang X, Lee J, Xu W, Zhang R, Li S, Niu J. Molecular cytogenetic characterization of undifferentiated embryonal sarcoma of the liver: a case report and literature review. Mol Cytogenet 2012; 5:26. [PMID: 22551002 PMCID: PMC3478990 DOI: 10.1186/1755-8166-5-26] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2011] [Accepted: 02/26/2012] [Indexed: 12/14/2022] Open
Abstract
Undifferentiated embryonal sarcoma of the liver (UESL) represents a heterogeneous group of tumors derived from mesenchymal tissues. Earlier cytogenetic studies in limited cases demonstrated that UESL is associated with a recurrent translocation t(11;19)(q11;q13.3-q13.4) or add(19)(q13.4). In this report, we present our array comparative genomic hybridization (aCGH), fluorescence in situ hybridization (FISH) findings, and a missense mutation of TP53 gene by DNA sequencing in a 19-year-old patient with UESL. The data were compared to laboratory findings reported by previous studies.
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Affiliation(s)
- Xiaoxia Hu
- Department of Internal Medicine, The First Hospital of Jilin University, Jilin 130021, China.
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Takashimizu S, Kojima SI, Nagata J, Nishizaki Y, Kagawa T, Shiraishi K, Mine T, Kaneko JI, Kokudo N, Watanabe N. Intrahepatic cholangiocellular carcinoma and hepatocellular carcinoma developed after a 6-year sustained virological response to interferon therapy for chronic hepatitis C. Clin J Gastroenterol 2011; 4:167-173. [PMID: 26189349 DOI: 10.1007/s12328-011-0216-y] [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] [Received: 01/24/2011] [Accepted: 02/21/2011] [Indexed: 10/18/2022]
Abstract
A 67-year-old male patient presenting with chronic hepatitis C (CHC) achieved a sustained virological response (SVR) following 6 months of treatment with 6 million units of beta-interferon (IFN). The SVR state continued for 6 years. Hepatocellular carcinoma (HCC) developed in liver segments 4 and 5, and was treated with transcatheter arterial chemoembolization, followed by radiofrequency ablation of the tumors. A recurrence of HCC occurred in segment 4 one and a half years after the initial treatment for HCC and a new tumor also developed in segment 8. These tumors were diagnosed to be recurrent HCC, and the three hepatic segments were resected. The pathological examination and immunostaining of the tumors revealed the tumor in segment 4 to be a well to moderately differentiated typical HCC. On the other hand, the tumor in segment 8 was a moderately to poorly differentiated adenocarcinoma and was diagnosed as an intrahepatic cholangiocellular carcinoma (ICC). HCC developed from CHC in a patient who achieved a 6-year SVR after IFN therapy, followed one and a half years later by the development of a heterochronous ICC at a different site, thus indicating the presence of HCC-ICC double cancer. This was an exceedingly rare and clinically important case in terms of the carcinogenic mechanism of HCC and ICC from a post-SVR CHC patient. We have to be aware of the possible development not only of HCC but of ICC after SVR in CHC patients.
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Affiliation(s)
- Shinji Takashimizu
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Sei-Ichiro Kojima
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Junko Nagata
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Yasuhiro Nishizaki
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Tatehiro Kagawa
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Koichi Shiraishi
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Tetsuya Mine
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan
| | - Jun-Ichi Kaneko
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
| | - Norihiro Kokudo
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
| | - Norihito Watanabe
- Division of Gastroenterology, Department of Internal Medicine, Tokai University School of Medicine, Bohseidai, Isehara, Kanagawa, 259-1193, Japan.
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14
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Watanabe T, Sakata J, Ishikawa T, Shirai Y, Suda T, Hirono H, Hasegawa K, Soga K, Shibasaki K, Saito Y, Umezu H. Synchronous development of HCC and CCC in the same subsegment of the liver in a patient with type C liver cirrhosis. World J Hepatol 2009; 1:103-109. [PMID: 21160972 PMCID: PMC2998951 DOI: 10.4254/wjh.v1.i1.103] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2009] [Revised: 09/02/2009] [Accepted: 09/09/2009] [Indexed: 02/06/2023] Open
Abstract
As a result of having undergone computed tomography (CT), a 75-year-old woman with type-C liver cirrhosiswas shown to have two tumors on the ventral and dorsal sides of subsegment 3 (S3). The tumor on the ventral side was diagnosed as a classic hepatocellular carcinoma (HCC), while that on the dorsal side was considered atypical for a HCC. Although the indocyanine green (ICG) findings indicated poor hepatic reserve, the prothrombin time (PT) was relatively good. An operation was performed in February 2007; however, this resulted in exploratory laparotomy. Dynamic CT performed 12 mo after the operation revealed that the tumor on the dorsal side of S3 had apparently increased. The marginal portion of the tumor was shown to be in the early and parenchymal phases, while the internal portion was found to have grown only slightly in the delayed phase. We diagnosed this tumor as a cholangiocellular carcinoma (CCC). S3 subsegmentectomy was performed in April 2008. The tumor on the ventral side was pathologically diagnosed as a moderately differentiated HCC, and that on the dorsal side was diagnosed as a CCC. We can therefore report a rare case of synchronous development of HCC and CCC in the same subsegment of the liver in a patient with type-C liver cirrhosis. We also add a literature review for all the reported cases published in Japan and around the world, and summarize the features of double cancer exhibiting both HCC and CCC.
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Affiliation(s)
- Takuya Watanabe
- Takuya Watanabe, Haruka Hirono, Katsuhiko Hasegawa, Kenji Soga, Koichi Shibasaki, Department of Internal Medicine and Gastroenterology, Medical Hospital, The Nippon Dental University School of Life Dentistry at Niigata, 1-8, Hamauracho, Chuo-ku, Niigata 951-8580, Japan
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15
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Undifferentiated embryonal sarcoma of the liver: case report and literature survey. ACTA ACUST UNITED AC 2008; 15:536-44. [PMID: 18836810 DOI: 10.1007/s00534-007-1265-y] [Citation(s) in RCA: 41] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2007] [Accepted: 08/24/2007] [Indexed: 12/15/2022]
Abstract
Undifferentiated embryonal sarcoma (UES) of the liver was first identified as an independent clinicopathologic type of sarcoma in 1978. It is an uncommon hepatic tumor, of mesenchymal origin, usually observed in children, and cases in adults are rare: to our best knowledge, reports of only 51 cases have been published in the past 50 years. We present a case of UES of the liver in a previously healthy 22 year-old woman, admitted to our hospital due to a palpable mass in the right upper abdomen. On admission, laboratory studies showed mildly elevated aspartate aminotransferase, alkaline phosphatase, and gamma-GPT. Hepatitis and tumor markers were negative. Ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) showed a large mass involving the right lobe and the medial segment of the liver, with compression of the bile duct. Right trisectionectomy with bile duct resection and reconstruction was performed. Microscopically, the tumor was composed of pleomorphic spindle cells in a myxoid stroma with focal staining of S-100 by immunohistochemistry. The histologic diagnosis was UES. Adjuvant therapy with vincristine, actinomycin-D, and cyclophosphamide was performed, and at 14 months of follow-up, the patient is alive without any evidence of recurrence. The clinical and histopathological features, as well as the therapeutic choices for adult UES, are described for this patient and in the literature of the past 50 years.
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16
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Inaba K, Suzuki S, Sakaguchi T, Kobayasi Y, Takehara Y, Miura K, Baba S, Nakamura S, Konno H. Double primary liver cancer (intrahepatic cholangiocarcinoma and hepatocellular carcinoma) in a patient with hepatitis C virus-related cirrhosis. ACTA ACUST UNITED AC 2007; 14:204-9. [PMID: 17384916 DOI: 10.1007/s00534-006-1134-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2005] [Accepted: 05/08/2006] [Indexed: 01/16/2023]
Abstract
We herein report a rare case of double primary liver cancer, consisting of intrahepatic cholangiocarcinoma (ICC) and hepatocellular carcinoma (HCC). A 67-year-old Japanese man with hepatitis C virus-related liver cirrhosis was diagnosed with multiple HCC in S7 and S8 of the liver. We performed a hepatic resection of S7 and S8. The liver tumors in S7 and S8 were pathologically diagnosed as HCC and ICC, respectively. Multiple recurrence of the HCC found 7 years after the surgery was successfully treated with transcatheter arterial chemoembolization. Subsequently, this patient has been doing well, without HCC recurrence. Double primary liver cancer is very rare, and only 21 resected cases have been reported, including that in our patient. We reviewed at all cases in the Japanese- and English-language literature to investigate the clinicopathological features. Our literature review revealed that the present patient is the longest survivor among patients who have undergone hepatectomy for double primary liver cancer.
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Affiliation(s)
- Keisuke Inaba
- Second Department of Surgery, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu 431-3192, Japan
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17
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Sotiropoulos GC, Molmenti EP, Frilling A, Paul A, Malamutmann E, Broelsch CE, Malagó M. Liver transplantation for double primary hepatic cancer-hepatocellular carcinoma and intrahepatic cholangiocarcinoma. Transplantation 2006; 82:718-9. [PMID: 16969301 DOI: 10.1097/01.tp.0000234929.56209.8e] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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18
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Lódi C, Szabó E, Holczbauer A, Batmunkh E, Szíjártó A, Kupcsulik P, Kovalszky I, Paku S, Illyés G, Kiss A, Schaff Z. Claudin-4 differentiates biliary tract cancers from hepatocellular carcinomas. Mod Pathol 2006; 19:460-9. [PMID: 16439986 DOI: 10.1038/modpathol.3800549] [Citation(s) in RCA: 70] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
The recently identified claudins are dominant components of tight junctions, responsible for cell adhesion, polarity and paracellular permeability. Certain claudins have been shown to have relevance in tumor development, with some of them, especially claudin-4, even suggested as future therapeutic target. The aim of the present study was to analyze the expression of claudin-4 in the biliary tree, biliary tract cancers and hepatocellular carcinomas. A total of 107 cases were studied: 53 biliary tract cancers, 50 hepatocellular carcinomas, 10 normal liver and 10 normal extrahepatic biliary duct samples. Immunohistochemical analysis was performed on conventional specimens and on tissue microarrays as well. Claudin-4 was further investigated by Western blot analysis and real-time RT-PCR. Intense membranous immunolabeling was found for claudin-4 in all biliary tract cancers unrelated to the primary site of origin, namely intrahepatic, extrahepatic or gallbladder cancers. Normal biliary epithelium showed weak positivity for claudin-4. In contrast, normal hepatocytes and tumor cells of hepatocellular carcinomas did not express claudin-4. The results of Western immunoblot analysis and real-time RT-PCR were in correlation with the immunohistochemical findings. Cytokeratins, as CK7 (92%) and CK19 (83%) were mostly positive in biliary tract cancers, however, one-third of hepatocellular carcinomas also expressed CK7 (34%). HSA antibody (HepPar1) reacted with the majority of hepatocellular carcinomas (86%), while being positive in a low percentage of the biliary tract cancers (8%). In conclusion, this is the first report of a significantly increased claudin-4 expression in biliary tract cancers, which represents a novel feature of tumors of biliary tract origin. Claudin-4 expression seems to be a useful marker in differentiating biliary tract cancers from hepatocellular carcinomas and could well become a potential diagnostic tool.
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Affiliation(s)
- Csaba Lódi
- 2nd Department of Pathology, Semmelweis University, Budapest, Hungary
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Zhang Z, Nonaka H, Nagayama T, Hatori T, Ihara F, Zhang L, Akima M, Shiba T. Double cancer consisting of adenosquamous and hepatocellular carcinomas of the liver. Pathol Int 2001; 51:961-4. [PMID: 11844070 DOI: 10.1046/j.1440-1827.2001.01302.x] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
A very rare case of a double cancer consisting of adenosquamous and hepatocellular carcinomas of the liver in a 65-year-old-man is discussed. The patient was hospitalized with epigastralgia in May 1997. Abdominal computed axial tomography revealed a tumor located in the left lobe of the liver and a left hepatic lobectomy was performed. The tumor recurred several months after surgery and the patient died on 4 June 1999. At autopsy, both a major tumor mass with extensive involvement, located in the surgical margin, and a small mass located in S7 were discovered. Microscopically, the major tumor was diagnosed as adenosquamous carcinoma and the small one in S7 as hepatocellular carcinoma. To our knowledge, this is the first case of a double cancer consisting of adenosquamous and hepatocellular carcinomas of the liver. The pathological findings support the hypothesis that this tumor developed as a squamous transformation of adenocarcinoma.
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Affiliation(s)
- Z Zhang
- First Department of Pathology, Toho University School of Medicine, Tokyo, Japan.
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