Primary liver carcinoma arising in people younger than 30 years.

Department of Pathology, the Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA.
American Journal of Clinical Pathology (Impact Factor: 3.01). 11/2005; 124(4):512-8. DOI: 10.1309/TT0R7KAL32228E99
Source: PubMed

ABSTRACT Primary liver carcinomas in children and young adults are uncommon and poorly described. We examined primary liver carcinomas in people younger than 30 years and performed immunostains for markers of biliary (cytokeratin [CK] 7, CK19, CD56) and hepatocellular (HepPar) differentiation. We found 23 primary liver carcinomas were found: 13 hepatocellular carcinomas (HCCs), 9 fibrolamellar carcinomas (FLCs), and 1 cholangiocarcinoma. Most HCCs showed compact (n = 7) or trabecular (n = 4) growth patterns. The Edmondson grades were as follows: 1, 3 tumors; 2, 8 tumors; and 3, 2 tumors). All HCCs and FLCs were HepPar(+). All FLCs and 7 of 9 HCCs were CK7(+). In contrast, a control group of 65 adult HCCs showed less CK7 positivity (24 [37%]; P = .03). CK19 was positive in 2 HCCs and CD56 in 1 HCC. No chronic background liver disease was seen, although 3 cases showed foci of altered hepatocytes. HCCs are the most common primary liver carcinoma in children and young adults followed by FLCs. They are morphologically similar to adult HCC, but more likely to be CK7(+).

  • Source
    [Show abstract] [Hide abstract]
    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.
    09/2012; 2012:743790. DOI:10.6064/2012/743790
  • Source
  • [Show abstract] [Hide abstract]
    ABSTRACT: Hepatocellular tumors are pathologically divided into a limited number of entities such as focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma and its variants, and hepatoblastoma. Recent advances in immunophenotypic and molecular characterization have led to an increased appreciation of the complexities of these growths. For example, subtypes of hepatocellular adenomas with differing premalignant potentials have been defined, our ability to differentiate hepatocellular carcinoma from high-grade dysplasia continues to improve, and molecular similarities of histologically discordant elements of combined hepatocellular/cholangiocellular carcinoma have been reported. This chapter describes pathologic, immunophenotypic, and molecular features of hepatocellular tumors. Continued progress in our understanding of these growths at the cellular and subcellular levels suggests that categorization of these tumors may continue to evolve as additional significant clinicopathologic correlates are discovered.

Full-text (2 Sources)

Available from
Jun 25, 2014