Primary Liver Carcinoma Arising in People Younger Than 30 Years

Department of Pediatrics, Johns Hopkins University, Baltimore, Maryland, United States
American Journal of Clinical Pathology (Impact Factor: 2.51). 11/2005; 124(4):512-8. DOI: 10.1309/TT0R7KAL32228E99
Source: PubMed


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(+).

Download full-text


Available from: Kathleen B Schwarz, Jun 25, 2014
  • Source
    • "Meanwhile, cases of HCV-Ab-positivity plus HCC among younger patients are reported at rates of 0 to 10% [4,5,7-10,12,14], which is much lower than the range for older patients. Rates of Child-Pugh A are 69.1 to 92.3% among younger patients [4-6,8-12], which is higher than the range in older patients. It has been reported that histological hepatitis or cirrhosis of non-cancerous liver is significantly less common in younger hepatectomy patients than in older hepatectomy patients among cases with HCC [3,4,12]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Background The aim of this study was to analyze the clinicopathological characteristics and the prognostic factors for survival and recurrence of young patients who had undergone hepatectomy for hepatocellular carcinoma. Methods Between 1990 and 2010, 31 patients aged 40 years or younger (younger patient group) among 811 consecutive patients with hepatocellular carcinoma who had undergone primary hepatectomy were analyzed with regard to patient factors, including liver function, tumor factors and operative factors. The clinicopathological characteristics of the younger patients were compared with those of patients over the age of 40 (older patient group). Then the prognostic factors of the younger patients were analyzed. Continuous variables were expressed as the means ± standard deviation and compared using the χ2 test for categorical variables. Overall survival and recurrence-free survival rates were determined by the Kaplan-Meier method and analyzed by the log-rank test. The Cox proportional hazards model was used for multivariate analysis. Results In the younger patients, the rates of HBs-antigen-positivity, high alpha-fetoprotein, portal invasion, intrahepatic metastasis, large tumors, low indocyanin green retention rate at 15 minutes, and anatomical resection were significantly higher than the same measures in the older patients. The five-year overall survival rate of the young patients was 49.6%. The prognostic factors of survival were HCV-antibody-positivity and low albumin status. Prognostic factors of recurrence were multiple tumors and the presence of portal invasion. Conclusions In younger patients, survival appeared to be primarily affected by liver function, while recurrence was affected by tumor factors. Young patients with hepatocellular carcinoma should be aggressively treated with hepatectomy due to their good pre-surgical liver function.
    Full-text · Article · Mar 2013 · World Journal of Surgical Oncology
  • Source
    • "The neoplastic cells show expression of the expected hepatocellular cytokeratin 8 [84] and 18 [84], but also are strongly positive for cytokeratin 7 [35, 68, 83–86] and occasionally (between 5 and 25%) for cytokeratin 19 [35, 68, 83, 84]. These latter cytokeratins are normally expressed in biliary epithelium. "
    [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.
    Full-text · Article · Sep 2012
  • Source
    • "Several authors have reported cholangiocarcinomas in patients aged less than 39 years (table 3) [9, 15, 16, 17]. Yeh et al. indicated that age is a risk factor for cholangiocarcinoma and that only one patient in the younger cholangiocarcinoma group survived for 24.2 months, whereas the remaining patients died within 12 months [9]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: A 29-year-old man with advanced hilar cholangiocarcinoma was successfully treated with an extended right lobectomy. The carbohydrate antigen 19-9 (CA19-9) level was elevated to 939 IU/l, and the pathological findings revealed moderately differentiated tubular adenocarcinoma which involved almost the entire thickness of the hepatic duct and the adjacent liver tissue (T3) and which was associated with lymph node metastasis (N1). It was a stage IIB (T3N1M0) tubular adenocarcinoma according to UICC pathological staging. Immunohistochemical examination revealed that Ki-67, cyclin D1, and MMP-7 were positive, and 14-3-3sigma and p27 were negative. The pathological and immunohistochemical findings indicated high malignant potential indicating poor prognosis. We administrated the postoperative adjunct gemcitabine combined with S-1 chemotherapy. The patient is alive without recurrence and doing well two years after surgery. We also review other reports of cholangiocarcinoma patients aged less than 30 years.
    Full-text · Article · May 2010 · Case Reports in Gastroenterology
Show more