Infrequent chromosome allele loss in fibrolamellar carcinoma

Ding, Shi-Fa; Delhanty, Jda; Bowles, L.; Dooley, JS; Wood, C. B.

doi:10.1038/bjc.1993.47

Cited by 18 publications

(7 citation statements)

References 15 publications

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“…Ding et al examined five cases of fibrolamellar carcinoma for loss of heterozygosity at 18 sites and found only two allele losses in 55 informative loci, both occurring in the same patient. 45 This allele loss rate of 3.6% was significantly lower than the rate of 16.1% that they previously described in 16 cases of classic hepatocellular carcinoma using the same methods. 46 Orsatti et al examined the ploidy of 12 cases of fibrolamellar carcinoma and 10 cases of hepatocellular carcinoma.…”

Section: Genetics and Epigeneticscontrasting

confidence: 51%

Fibrolamellar Carcinoma: A Review with Focus on Genetics and Comparison to Other Malignant Primary Liver Tumors

Ward

Waxman

2011

Semin Liver Dis

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Fibrolamellar carcinoma is a rare primary malignant liver neoplasm that usually affects adolescents and young adults with no underlying liver disease. Morphologically, the tumor cells resemble oncocytic hepatocytes arranged in cords with a stroma of lamellated collagen fibers. Immunohistochemical studies have found that fibrolamellar carcinomas express markers associated with both biliary (CK7 and epithelial membrane antigen) and hepatocytic (heppar-1and glypican-3) differentiation, as well as markers associated with hepatic progenitor cells (CK19 and EpCAM) and stem cells (CD133 and CD44). Genetic studies show fewer alterations compared with classic hepatocellular carcinoma. Pooled data from comparative genomic hybridization studies show that fibrolamellar carcinomas have fewer and less frequent genomic alterations when compared with classic hepatocellular carcinoma, cholangiocarcinoma, and hepatoblastoma. Of the alterations seen in fibrolamellar carcinoma, the most frequent are gains in 1q and 8q (also frequently seen in other hepatic tumors) and loss of 18q. Fibrolamellar carcinoma also has less frequent methylation of tumor suppressor promoters compared with hepatocellular carcinoma and minimal alterations in mitochondrial DNA. Fibrolamellar carcinoma is associated with better survival than hepatocellular carcinoma and cholangiocarcinoma, presumably due to the young age of the patients and the lack of cirrhosis. These features make more aggressive surgical therapy possible. There is currently very little information on the effectiveness of chemotherapy for fibrolamellar carcinoma.

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Section: Genetics and Epigeneticscontrasting

confidence: 51%

Fibrolamellar Carcinoma: A Review with Focus on Genetics and Comparison to Other Malignant Primary Liver Tumors

Ward

Waxman

2011

Semin Liver Dis

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“…Cytogenetic studies and other methodologies for examining large cytogenetic abnormalities have shown relative chromosomal stability in FLC, compared with typical HCC [2,4,13], and have identified a limited number of areas of interest, principally gains on chromosome 1q [2,4]. Positional mapping of typical HCC has further narrowed the regions of interest for the 1q oncogene:1q21-q22 [14], 1q23.1-q24.2 [5], 1q32.1 [5], and 1q43-q44 [5].…”

Section: Discussionmentioning

confidence: 97%

Fibrolamellar carcinomas show overexpression of genes in the RAS, MAPK, PIK3, and xenobiotic degradation pathways

et al. 2007

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“…The frequency of allelic loss evaluated by polymerase chain reaction has been shown to be lower in fibrolamellar carcinoma compared with conventional hepatocellular carcinoma. 41 These tumors have also been shown to lack intratumor heterogeneity as detected by DNA fingerprinting Figure 3 Kaplan-Meier curve demonstrating 5-year survival in fibrolamellar carcinomas with cytogenetic abnormalities versus those without cytogenetic abnormalities (33% vs 80%, P ¼ 0.1).…”

Section: Discussionmentioning

confidence: 99%

Chromosomal changes in fibrolamellar hepatocellular carcinoma detected by array comparative genomic hybridization

Kakar

Chen

et al. 2009

Modern Pathology

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Fibrolamellar hepatocellular carcinoma is a rare subtype of hepatocellular carcinoma with distinct clinical and histological features, and better survival compared with conventional hepatocellular carcinoma in some but not all series. We performed a comparative genomic hybridization analysis on 11 fibrolamellar carcinomas and correlated the findings with clinicopathologic features and survival. Chromosomal imbalances were identified in six cases (55%), whereas the other five (45%) yielded normal results. The mean number of aberrations per case was 3.9 for all cases and 7.2 in abnormal cases. Among the six abnormal cases, gains or losses were observed at 3 loci in two cases, 7 loci in one case, 8 loci in two cases and 14 loci in one case. The most common abnormalities were observed in chromosomes 7, 8 and 18, with 7q gain in five cases and 7p gain in four cases. Aberrations associated with intermediate or advanced conventional hepatocellular carcinomas, including losses at 3q, 4q and 13q were identified in 17-33% of fibrolamellar carcinomas. There was no correlation of chromosomal changes with age, gender and tumor size. The 5-year survival among the six patients with no chromosomal abnormalities was 80% (4/5) compared with 33% (2/6) in patients with chromosomal abnormalities (P=0.1). In conclusion, fibrolamellar carcinomas show fewer chromosomal abnormalities compared with those reported in literature for conventional hepatocellular carcinoma. The most common abnormalities occur in chromosomes 7 and 8. Fibrolamellar carcinomas with chromosomal changes appear to behave more aggressively compared with cases with no cytogenetic abnormalities. The favorable outcome in some fibrolamellar carcinomas may be due to absent or low number of cytogenetic aberrations.

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Infrequent chromosome allele loss in fibrolamellar carcinoma

Cited by 18 publications

References 15 publications

Fibrolamellar Carcinoma: A Review with Focus on Genetics and Comparison to Other Malignant Primary Liver Tumors

Fibrolamellar Carcinoma: A Review with Focus on Genetics and Comparison to Other Malignant Primary Liver Tumors

Fibrolamellar carcinomas show overexpression of genes in the RAS, MAPK, PIK3, and xenobiotic degradation pathways

Chromosomal changes in fibrolamellar hepatocellular carcinoma detected by array comparative genomic hybridization

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