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Anysz-Grodzicka A, Podgorska J, Cieszanowski A. State-of-the-art MR Imaging of Uncommon Hepatocellular Tumours: Fibrolamellar Hepatocellular Carcinoma and Combined Hepatocellularcholangiocarcinoma. Curr Med Imaging 2020; 15:269-280. [PMID: 31989878 DOI: 10.2174/1573405614666180927113622] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2017] [Revised: 09/12/2018] [Accepted: 09/14/2018] [Indexed: 12/12/2022]
Abstract
BACKGROUND Fibrolamellar Carcinoma (FLC) and Combined Hepatocellular- Cholangiocarcinoma (CHC) are rare primary liver tumours, which are related to different clinical settings. In both tumours, correlation with clinical data and laboratory tests are extremely important. DISCUSSION Typically, FLC is diagnosed in young patients without any chronic disease and with normal biochemical tests, whereas CHC arises in cirrhotic patients with elevated tumour markers: AFP and/or CA 19-9. The review describes epidemiology, aetiology, pathogenesis, radiological features and treatment of these tumours. Imaging features typical for FLC are: The presence of central scar, calcifications, the large size, heterogeneous and early contrast-enhancement. CONCLUSION The diagnosis of CHC may be suggested in case of elevation of both AFP and CA 19- 9 or inconsistency between elevated tumour markers and imaging findings (i.e., elevated CA 19-9 and radiological features of HCC, or elevated AFP with imaging findings characteristic of ICC).
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Affiliation(s)
- Agnieszka Anysz-Grodzicka
- Department of Radiology, Maria Sklodowska-Curie Memorial Cancer Centre, Institute of Oncology, Warsaw, Poland
| | - Joanna Podgorska
- Department of Clinical Radiology, Medical University of Warsaw, Warsaw, Poland
| | - Andrzej Cieszanowski
- Department of Radiology, Maria Sklodowska-Curie Memorial Cancer Centre, Institute of Oncology, Warsaw, Poland
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2
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Gera S, Ettel M, Acosta-Gonzalez G, Xu R. Clinical features, histology, and histogenesis of combined hepatocellular-cholangiocarcinoma. World J Hepatol 2017; 9:300-309. [PMID: 28293379 PMCID: PMC5332419 DOI: 10.4254/wjh.v9.i6.300] [Citation(s) in RCA: 74] [Impact Index Per Article: 9.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/11/2016] [Revised: 11/08/2016] [Accepted: 01/03/2017] [Indexed: 02/06/2023] Open
Abstract
Combined hepatocellular-cholangiocarcinoma (CHC) is a rare tumor with poor prognosis, with incidence ranging from 1.0%-4.7% of all primary hepatic tumors. This entity will be soon renamed as hepato-cholangiocarcinoma. The known risk factors for hepatocellular carcinoma (HCC) have been implicated for CHC including viral hepatitis and cirrhosis. It is difficult to diagnose this tumor pre-operatively. The predominant histologic component within the tumor largely determines the predominant radiographic features making it a difficult distinction. Heterogeneous and overlapping imaging features of HCC and cholangiocarcinoma should raise the suspicion for CHC and multiple core biopsies (from different areas of tumor) are recommended before administering treatment. Serum tumor markers CA19-9 and alpha-fetoprotein can aid in the diagnosis, but it remains a challenging diagnosis prior to resection. There is sufficient data to support bipotent hepatic progenitor cells as the cell of origin for CHC. The current World Health Organization classification categorizes two main types of CHC based on histo-morphological features: Classical type and CHC with stem cell features. Liver transplant is one of the available treatment modalities with other management options including transarterial chemoembolization, radiofrequency ablation, and percutaneous ethanol injection. We present a review paper on CHC highlighting the risk factors, origin, histological classification and therapeutic modalities.
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Sergi CM. Hepatocellular Carcinoma, Fibrolamellar Variant: Diagnostic Pathologic Criteria and Molecular Pathology Update. A Primer. Diagnostics (Basel) 2015; 6:3. [PMID: 26838800 PMCID: PMC4808818 DOI: 10.3390/diagnostics6010003] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2015] [Revised: 12/21/2015] [Accepted: 12/23/2015] [Indexed: 12/13/2022] Open
Abstract
Fibrolamellar hepatocellular carcinoma (FL-HCC) is generally a fairly rare event in routine pathology practice. This variant of hepatocellular carcinoma (HCC) is peculiarly intriguing and,in addition, poorly understood. Young people or children are often the target individuals with this type of cancer. Previously, I highlighted some pathology aspects of FL-HCC, but in this review, the distinctive clinico-pathologic features of FL-HCC and the diagnostic pathologic criteria of FL-HCC are fractionally reviewed and expanded upon. Further, molecular genetics update data with reference to this specific tumor are particularly highlighted as a primer for general pathologists and pediatric histopathologists. FL-HCC may present with metastases, and regional lymph nodes may be sites of metastatic spread. However, peritoneal and pulmonary metastatic foci have also been reported. To the best of our knowledge, FL-HCC was initially considered having an indolent course, but survival outcomes have recently been updated reconsidering the prognosis of this tumor. Patients seem to respond well to surgical resection, but recurrences are common. Thus, alternative therapies, such as chemotherapy and radiation, are ongoing. Overall, it seems that this aspect has not been well-studied for this variant of HCC and should be considered as target for future clinical trials. Remarkably, FL-HCC data seem to point to a liver neoplasm of uncertain origin and unveiled outcome. A functional chimeric transcript incorporating DNAJB1 and PRKACA was recently added to FL-HCC. This sensational result may give remarkable insights into the understanding of this rare disease and potentially provide the basis for its specific diagnostic marker. Detection of DNAJB1-PRKACA seems to be, indeed, a very sensitive and specific finding in supporting the diagnosis of FL-HCC. In a quite diffuse opinion, prognosis of this tumor should be reconsidered following the potentially mandatory application of new molecular biological tools.
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Affiliation(s)
- Consolato M Sergi
- Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, 8440 112 St., AB T6G2B7, Canada.
- Department of Pediatrics, Stollery Children's Hospital, Edmonton, AB T6G2B7, Canada.
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Nayyar M, Imagawa DK, Tirkes T, Demirjian AN, Houshyar R, Sandrasegaran K, Nangia CS, Seery T, Bhargava P, Choi JI, Lall C. Composite liver tumors: a radiologic-pathologic correlation. Clin Mol Hepatol 2015; 20:406-10. [PMID: 25548749 PMCID: PMC4278074 DOI: 10.3350/cmh.2014.20.4.406] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/05/2023] Open
Abstract
Bi-phenotypic neoplasm refers to tumors derived from a common cancer stem cell with unique capability to differentiate histologically into two distinct tumor types. Bi-phenotypic hepatocellular carcinoma-cholangiocarcinoma (HCC-CC), although a rare tumor, is important for clinicians to recognize, since treatment options targeting both elements of the tumor are crucial. Imaging findings of bi-phenotypic HCC-CC are not specific and include features of both HCC and CC. A combination of imaging and immuno-histochemical analysis is usually needed to make the diagnosis.
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Affiliation(s)
- Megha Nayyar
- Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
| | | | | | | | | | | | | | - Tara Seery
- University of California, Irvine, Orange, CA, USA
| | - P Bhargava
- University of Washington, Seattle, WA, USA
| | - Joon Ii Choi
- Department of Radiology, College of Medicine, Seoul St.Mary's Hospital, the Catholic University of Korea, Seoul, Korea
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5
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Ganeshan D, Szklaruk J, Kundra V, Kaseb A, Rashid A, Elsayes KM. Imaging features of fibrolamellar hepatocellular carcinoma. AJR Am J Roentgenol 2014; 202:544-552. [PMID: 24555590 DOI: 10.2214/ajr.13.11117] [Citation(s) in RCA: 73] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
OBJECTIVE Fibrolamellar hepatocellular carcinoma (HCC) is a rare primary liver tumor, which significantly differs from conventional HCC. This article reviews the molecular cytogenetics, pathology, imaging features, and management of this relatively rare tumor. CONCLUSION Fibrolamellar HCC predominantly occurs in young patients without underlying hepatitis or cirrhosis. Serum α-fetoproteins are not elevated in most cases, and hence imaging plays an important role in diagnosis, staging, and surveillance.
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Affiliation(s)
- Dhakshinamoorthy Ganeshan
- 1 Division of Diagnostic Imaging, Body Imaging Section, Unit 1473, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030-4009
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6
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Abstract
The fibrolamellar variant of hepatocellular carcinoma is a rare primary liver cancer occurring in adolescents and young adults without chronic liver disease or known risk factors. Histologically, it is defined by lamellar bands of fibrosis surrounding well-differentiated tumor cells. Radiologic imaging typically demonstrates a large, solitary mass with calcifications and a central scar. Lymph node metastases in the porta hepatis are frequently diagnosed upon presentation. More patients with fibrolamellar carcinoma are candidates for surgical resection than those with conventional hepatocellular carcinoma, owing to their young age and absence of cirrhosis. The most important prognostic factor is surgical resection, which results in 5-year overall survival rates ranging between 50 and 76 %. Despite complete surgical resection, relapse rates are high, and novel therapies are needed to prevent and treat recurrent disease.
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Affiliation(s)
- Yun Shin Chun
- Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA 19111, USA.
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Rosa M, Mohammadi A. Cytologic features of fibrolamellar carcinoma with mucin production: a rare variant of combined hepatocellular-cholangiocarcinoma. Diagn Cytopathol 2012; 42:431-5. [PMID: 23008149 DOI: 10.1002/dc.22931] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2012] [Revised: 07/06/2012] [Accepted: 08/29/2012] [Indexed: 11/07/2022]
Abstract
Fibrolamellar carcinoma (FC) is an uncommon tumor that usually arises in non-cirrhotic livers of adolescents or young adults. It differs clinically from hepatocellular carcinoma in its better prognosis and lack of gender predilection. Cytologically, the tumor is composed of large polygonal cells with abundant cytoplasm, pleomorphic nuclei, and prominent large nucleoli. A variant of FC with mucinous differentiation has been previously described. These tumors have been regarded as combined hepatocellular cholangiocarcinomas. Herein, we report the case of a 44-year-old Asian female with a large liver mass present for approximately two years. Core needle biopsy with imprint cytology demonstrated FC with areas of intracellular mucin. To our knowledge, cytological features of this rare tumor have not been described before.
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Affiliation(s)
- Marilin Rosa
- Department of Pathology and Laboratory Medicine, University of Florida, College of Medicine, Jacksonville, Florida
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Torbenson M. Fibrolamellar carcinoma: 2012 update. SCIENTIFICA 2012; 2012:743790. [PMID: 24278737 PMCID: PMC3820672 DOI: 10.6064/2012/743790] [Citation(s) in RCA: 65] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 07/26/2012] [Accepted: 08/22/2012] [Indexed: 06/02/2023]
Abstract
Fibrolamellar carcinomas are a unique type of primary liver cancer. They occur most commonly in children and young adults. Their etiology remains a mystery, as they are not associated with chronic liver disease. Fibrolamellar carcinomas are not indolent tumors, but have an overall better prognosis than typical hepatocellular carcinomas, in part because of the younger age at presentation and the lack of cirrhosis. The most important prognostic feature is whether the tumor is resectable. Histologically, the tumor is made up of large cells that contain abundant mitochondria. The nuclei of the tumor cells have prominent nucleoli. The tumor cells induce the formation of extensive intratumoral fibrosis, which often grows in parallel, or lamellar bands. The tumor cells clearly show hepatocellular features but are also unique in showing both biliary and neuroendocrine differentiation. The uniqueness of fibrolamellar carcinoma extends to their molecular findings. While the genetic abnormalities that lead to fibrolamellar carcinomas are not yet known, studies have shown that they lack mutations in the genes most commonly mutated in typical hepatocellular carcinoma (TP53 and CTNNB1). In this paper, the clinical, pathological, and basic science literature on fibrolamellar carcinoma is comprehensively reviewed. Key areas of needed research are also discussed.
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Affiliation(s)
- Michael Torbenson
- Department of Pathology, The Johns Hopkins University School of Medicine, Room B314, 1503 E. Jefferson, Bond Street Building, Baltimore, MD 21231, USA
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9
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Lee CW, Chan KM, Lee CF, Yu MC, Lee WC, Wu TJ, Chen MF. Hepatic resection for hepatocellular carcinoma with lymph node metastasis: clinicopathological analysis and survival outcome. Asian J Surg 2011; 34:53-62. [PMID: 21723467 DOI: 10.1016/s1015-9584(11)60020-1] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2010] [Revised: 12/13/2010] [Accepted: 04/14/2011] [Indexed: 01/29/2023] Open
Abstract
OBJECTIVE Lymph node metastasis (LNM) rarely occurs in hepatocellular carcinoma (HCC). Few studies have reported the potential risk factors of LNM and the influence of LNM on the progression and prognosis of HCC. The purposes of this study were to explore the clinicopathological characteristics of operable HCC with LNM and to demonstrate the effects of LNM on HCC prognosis. METHODS A retrospective review of 2,034 HCC patients undergoing surgery from 1982 to 2005 was performed. The influence of LNM was assessed by clinicopathological factors, tumour recurrence, and overall survival. A total of 66 randomly selected patients matched for clinicopathological variables were used to analyse the difference in survival. RESULTS A total of 25 patients (1.23%) were reported to have LNM. Higher preoperative carcinoembryonic antigen levels (> 10 ng/mL) were significantly associated with a higher incidence of LNM than were low preoperative carcinoembryonic antigen levels (≤ = 10 ng/mL) (15.38%vs. 3.79%, p = 0.042). Furthermore, HCC with LNM (N1 disease) was larger in size (mean, 9.44 vs. 5.85 cm, p = 0.016) and significantly associated with vascular invasion, worse histological grade, and nonencapsulation (p = 0.002, < 0.001, and < 0.001, respectively). Finally, patients with HCC accompanied by LNM had shorter mean disease-free survival and overall survival (p = 0.001 and < 0.001, respectively). CONCLUSION This study identified the worst prognosis of HCC in a population with LNM. HCC with LNM tends to be the infiltrating type with larger tumour size (> 5 cm), presence of microvascular invasion, and worse histological grade. Liver resection with lymphadenectomy is possibly beneficial for patients with HCC accompanied by LNM.
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Affiliation(s)
- Chao-Wei Lee
- Department of Surgery, Chang Gung Memorial Hospital, Linkou, Taoyuan, Taiwan
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Abstract
Combined hepatocellular-cholangiocarcinoma is a rare primary neoplasm in the liver. It has gained increasing recognition recently, which in part may be due to more extensive sampling of the explants and surgical resection specimens, the diagnostic challenges encountered in the clinical practice, and the yet to be determined clinical outcome, but partly may be attributed to its intriguing histogenesis/cells of origin. This review aims to update combined hepatocellular-cholangiocarcinoma with an emphasis on the pathological diagnosis, including the differential diagnosis and its diagnostic pitfalls, the possible cell of origin of this neoplasm, and its clinical outcome.
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Affiliation(s)
- Matthew M Yeh
- Department of Pathology, University of Washington School of Medicine, Seattle, Washington 98195, USA.
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Abstract
Fibrolamellar hepatocellular carcinoma (FHLCC) generally occurs in young individuals lacking a background of chronic liver disease and other risk factors for hepatocellular carcinoma. The clinical presentations of FLHCC are generally nonspecific, and the alpha-fetoprotein level is typically within the normal range in most cases. Imaging studies have a major role in clinical diagnosis, but pathology is the gold standard in confirming diagnosis. Pathological characteristics of FLHCC include the presence of tumor cells with a deeply eosinophilic cytoplasm and macronucleoli surrounded by abundant fibrous bands. The most effective treatment for FLHCC is aggressive surgical resection. This comprehensive literature review gives a full account of the clinical, pathological, and molecular features of FLHCC.
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Zhang T, Chen XD, He S, Wang QC. Significance of hepatocyte paraffin-1, cluster of differentiation 34 and cytokeratin expression in the diagnosis of alpha-fetoprotein-negative hepatocellular carcinoma. Shijie Huaren Xiaohua Zazhi 2009; 17:2791-2797. [DOI: 10.11569/wcjd.v17.i27.2791] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To investigate the significance of hepatocyte paraffin-1 (Hep Par-1), cluster of differentiation 34 (CD34) and cytokeratin (CK) expression in the diagnosis of alpha-fetoprotein-negative hepatocellular carcinoma (AFP(-)HCC) and in the differential diagnosis among AFP(-) HCC, intrahepatic cholangiocellular carcinoma (ICC) and metastatic adenocarcinoma (MAC).
METHODS: Paraffin-embedded surgical specimens were collected from 70 AFP(-)HCC patients, 6 ICC patients and 24 MAC patients at Nantong Tumor Hospital from 1989 to 2007. All specimens were stained immunohistochemically for Hep Par 1, CD34 and CK.
RESULTS: There were statistically significant differences in the positive expression rates of Hep Par 1 and CD34 in AFP(-)HCC, ICC and MAC (Hep Par 1: χ2 = 50.7937, 9.5745 and 37.4532, respectively; CD34: χ2 = 67.0330, 9.9836 and 49.3927, respectively; all P < 0.01). Significant differences were also noted in the positive expression rates of Hep Par 1, CD34, CK20 and CK19 between poorly and well differentiated AFP(-)HCC (all P < 0.01 or 0.05). The accuracy, sensitivity and specificity of combined immunohistochemical detection of Hep Par 1, CD34 and CK in the differential diagnosis among AFP(-) HCC, ICC and MAC were 90.7%, 89.8% and 93.3%, respectively.
CONCLUSION: Combined detection of Hep Par 1, CD34 and CK can improve the accuracy of diagnosis of AFP(-)HCC and of differential diagnosis among AFP(-)HCC, ICC and MAC.
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Primary liver carcinoma exhibiting dual hepatocellular-biliary epithelial differentiations associated with citrin deficiency: a case report. J Clin Gastroenterol 2008; 42:855-60. [PMID: 18385606 DOI: 10.1097/01.mcg.0000225683.29841.9c] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
We report a 50-year-old male patient with primary liver carcinoma exhibiting dual hepatocellular and biliary epithelial differentiations associated with citrin deficiency (asymptomatic adult-onset type II citrullinemia, CTLN2). Although so far 14 CTLN2 patients with hepatocellular carcinoma have been reported, this report describes a unique case of liver carcinoma showing the features of both hepatocellular and cholangiocellular carcinoma. In addition to the clinical data of the 14 patients reported previously, the findings in our patient suggest that the citrin deficiency might be one of the key disorders causing hepatocellular carcinoma especially at younger ages and can also play an important role in hepatocarcinogenesis of the hepatic progenitor cells, which have the bipotential to differentiate into both hepatocytes and cholangiocytes.
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Wu XZ. Origin of cancer stem cells: the role of self-renewal and differentiation. Ann Surg Oncol 2007; 15:407-14. [PMID: 18043974 DOI: 10.1245/s10434-007-9695-y] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2007] [Revised: 09/24/2007] [Accepted: 10/19/2007] [Indexed: 12/12/2022]
Abstract
BACKGROUND Self-renewal and differentiation potential is the feature of stem cells. Differentiation is usually considered to be a one-way process of specialization as cells develop the functions of their ultimate fate and lose their immature characteristics, such as self-renewal. Progenitor cells, the products of stem cells losing the activity of self-renewal, could differentiate to mature cells, which have the feature of differentiation and lose the activity of self-renewal. The roles for cancer stem cells have been demonstrated for some cancers. However, the origin of the cancer stem cells remains elusive. METHODS This review focuses on current scientific controversies related to the establishment of the cancer stem cells--in particular, how self-renewal and differentiation block might contribute to the evolution of cancer. RESULTS Cancer stem cells may be caused by transforming mutations occurring in multi-potential stem cells, tissue-specific stem cells, progenitor cells, mature cells and cancer cells. Progenitor cells obtain the self-renewal activity by activating the self-renewal-associated genes rather than dedifferentiate to tissue special stem cells. The transform multi-potential stem cells gain the differentiation feature of special tissue by differentiating to cancer cells. Mature cells and cancer cells may dedifferentiate or reprogram to cancer stem cells by genetic and / or epigenetic events to gain the self-renewal activity and lose some features of differentiation. The cancer-derived stem cells are not the "cause", but the "consequence" of carcinogenesis. The genetic program controlling self-renewal and differentiation is a key unresolved issue. CONCLUSION Cancer stem cells may be caused by disturbance of self-renewal and differentiation occurring in multi-potential stem cells, tissue-specific stem cells, progenitor cells, mature cells and cancer cells.
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Affiliation(s)
- Xiong-Zhi Wu
- Tianjin Medical University Cancer Institute and Hospital, Ti-Yuan-Bei, Huan-Hu-Xi Road, He-Xi District, Tianjin 300060, China.
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Abstract
Since its first description 50 years ago, fibrolamellar carcinomas (FLCs) have been recognized as a unique type of primary liver cancer. FLCs occur principally in children and young adults and are not associated with chronic liver disease. Their etiology is unknown. The tumor is made up of large polygonal cells containing abundant eosinophilic cytoplasm, large vesiculated nuclei, and large nucleoli, with tumor cells that are embedded in lamellar bands of fibrosis. Although rare, the most common variant of FLC shows areas of glandular type differentiation with mucin production. The uniqueness of FLC extends to their molecular findings, as they show no evidence for involvement by many of the major pathways and genes that are dysregulated in typical hepatocellular carcinoma, including alpha-fetoprotein, TP53 mutations, and beta catenin mutations. FLCs are not indolent tumors, but have an overall better prognosis than hepatocellular carcinomas of the usual sort because of the younger age at presentation and lack of cirrhosis. The most important prognostic feature is resectability. Although their morphologic appearance on routine stains is well defined, their etiology is still unknown and much of their molecular biology remains poorly described and awaits future investigation.
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Affiliation(s)
- Michael Torbenson
- Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA.
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Abstract
Fibrolamellar carcinoma (FL-Ca) is a primary malignant liver tumor at unknown etiology, without cirrhosis and usually without an increase at tumor markers, which occurs mainly in young patients. As it can simulate malignant and benign tumors, particularly FNH, diagnosis is difficult. Ultrasound and angiography show mostly uncharacteristic features. The highest specificity has CT, if calcifications are present, because these calcifications in a tumor similar to FNH are pathognomonic for FL-Ca. In MRI the central scars of FL-Ca and FNH have a different signal intensity in T2-weighted images, so that MRI becomes more and more important in the differential diagnosis to FNH.
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