Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary liver carcinoma (PLC) may express a certain number of markers. Here we communicate results of an analysis of five such markers (alpha-1-antitrypsin--AAT--, carcino-embryonic antigen --CEA--, alpha-fetoprotein --AFP--, and superficial --HBsAg-- and core --HBcAg-- antigens of hepatitis B virus) by means of PAP techniques in 130 cases of PLC, comparing the neoplastic tissue and the non-tumorous liver. Three variants of PLC are distinguished: hepatocarcinoma (HC) (108 cases); cholangiocarcinoma (CC) (19 cases); and three cases of hepatocholangiocarcinoma (HCC). AAT was positive in 29 HC, 2 HCC, and negative in all 19 CC. CEA appeared positive in 16 HC, 16 CC and only one HCC. AFP was positive in two HC, and negative in all CC and HCC. HBsAg displayed positivity in 15 HC and one HCC, being negative in all 19 CC. HBcAg was positive in 4 HC, and negative in all CC and HCC. HBsAg was also positive in two neoplastic emboli associated with HC. On the non-tumorous liver tissue the immunohistochemical results showed positivity for AAT and CEA, but not for AFP. Therefore the present results confirm that in the geographical area from which these tumors proceed, PLC is closely correlated with HBsAg positivity and with cirrhosis.
 ...
PMID:Immunohistochemical characterization of 130 cases of primary hepatic carcinomas. 244 80

One hundred sixty-four consecutive cases of primary liver carcinoma were evaluated for tumor type, (i.e., hepatocellular carcinoma [HCC], cholangiocarcinoma [CC], and combined hepatocholangiocarcinoma [CHCC]), and for signs of alpha-1-antitrypsin deficiency (AATD) in the surrounding liver tissue. Hepatocellular globular alpha-1-antitrypsin deposits, as detected by a monoclonal antibody to the mutant PiZ alpha-1-antitrypsin (AAT), were seen in 13 cases (7.9%). With regard to tumor type, 4 of 111 HCC cases (3.5%), but 4 of 37 CC cases (10.5%), and even 5 of 16 CHCC cases (30%) were positive for this antitrypsin variant. In all but 1 of 13 cases of alpha-1-antitrypsin deficiency, the carcinoma developed in noncirrhotic liver tissue of elderly people (mean age, 62.9 years). In three patients, a heterozygous state of ATT (PiMZ) could be revealed using isoelectric focusing or direct genetic analysis. We conclude from our findings that CHCC and CC especially might be associated with PiZ alpha-1-antitrypsin deficiency. Primary liver carcinoma might develop even in a heterozygote state of PiZ alpha-1-antitrypsin deficiency without concurrent liver disease. Furthermore, liver cirrhosis is not a precondition for these tumors.
 ...
PMID:Liver carcinoma in PiZ alpha-1-antitrypsin deficiency. 963 Jan 82

Hepatocellular carcinoma (HCC) is a well-known complication of genetic hemochromatosis (GH). However, the frequency of primary liver carcinoma (PLC) with biliary differentiation, such as cholangiocarcinoma (CC) and combined hepatocholangiocarcinoma (CHCC), in GH remains unclear We analyzed the histologic type of 20 PLCs occurring in the background of GH; all patients were homozygotic for the C282Y mutation. Ten were depleted of iron by successive phlebotomies, while the remaining 10 were untreated. Histologically, 13 cases were classified as HCC, 3 as CC, and 4 as CHCC. Immunohistochemical detection of Hep Par 1, cytokeratin 19 (CK19), and MUC1 supported this classification; PLC with biliary differentiation was immunoreactive for MUC1 in 86% (6/7) of cases and for CK19 in 100% (7/7) of cases. The nontumoral liver exhibited no cirrhosis or extensive fibrosis in 6 cases. Von Meyenburg complexes were present in 11 cases and intraparenchymal bile duct adenomas in 3. These data suggest that PLCs in patients with GH present a wide histologic spectrum, with tumors showing frequent biliary differentiation; may arise on a nonfibrotic or a cirrhotic liver; and often are associated with Von Meyenburg complexes and to a lesser extent with bile duct adenomas.
 ...
PMID:Primary liver carcinoma in genetic hemochromatosis reveals a broad histologic spectrum. 1171 Jun 92

Combined hepatocellular and cholangiocarcinoma is a rare form of primary hepatic carcinoma. We present a case of combined hepatocellular and cholangiocarcinoma coexisting with hepatolithiasis in a 53-year-old woman. The tumor was detected during surgery for the treatment of intrahepatic stones. A left hepatectomy was performed. A histologic examination of the specimen revealed typical features of combined hepatocellular and cholangiocarcinoma. Immunohistochemical analyses showed a biphenotypic antigen expression profile of the tumor cells. Risk factors commonly related to the development of this type of tumor include viral hepatitis and cirrhosis. This report describes an unusual etiologic factor that has not been reported previously.
 ...
PMID:Combined hepatocellular and cholangiocarcinoma associated with hepatolithiasis: report of a case. 2143 1

Mixed hepatocellular cholangiocarcinoma (HCC-CC) and intrahepatic cholangiocarcinoma (I-CC) are increasingly being reported in patients with cirrhosis. The aims of this study were (1) to evaluate the incidence, imaging features, and posttransplant outcomes for patients who underwent transplantation for hepatocellular carcinoma (HCC) and were found to have HCC-CC or I-CC in the explant and (2) to compare the outcomes of these patients with those of controls with HCC who were matched (1:3) by the tumor size and the number of nodules in the explant. In the explant specimens of 10 of 302 patients (3.3%) who underwent liver transplantation (LT) for HCC, mixed HCC-CC or I-CC was identified. There were 4 additional incidental cases of HCC-CC. After a median follow-up period of 32 months, 8 of the 14 patients (57%) suffered from tumor recurrence, and the median disease-free survival time was 8 months. The cumulative risk of tumor recurrence was 40% and 70% at 1 and 5 years, respectively, for these 14 patients. When the 4 incidental cases were excluded, the study group with HCC-CC or I-CC (n = 10) had a significantly lower incidence of well-differentiated tumors (11.1% versus 43.3%, P < 0.02) and a higher rate of recurrence (60% versus 16.7%, P = 0.008) in comparison with the control group of patients with HCC (n = 30). The 1- and 5-year cumulative risks of tumor recurrence were 42% and 65%, respectively, in the study group and 10% and 17%, respectively, in the control group (P < 0.002). The actuarial 1- and 5-year patient survival rates without recurrence were also significantly lower in the study group (79% and 32% in the study group and 90% and 62% in the control group, P < 0.03). Dynamic contrast-enhanced computed tomography or magnetic resonance imaging showed progressive contrast enhancement throughout the arterial and portal venous phases without washout in 8 of the 10 patients. In conclusion, HCC-CC and I-CC are associated with a poor prognosis and a high rate of tumor recurrence after LT, and both tumors exhibit radiographic features that are distinct from those observed with HCC.
 ...
PMID:Mixed hepatocellular cholangiocarcinoma and intrahepatic cholangiocarcinoma in patients undergoing transplantation for hepatocellular carcinoma. 2143 29

Combined hepatocellular carcinoma and cholangiocarcinoma (cHCC-CC) is a rare form of primary liver cancer (PLC). It is difficult to make a correct preoperative diagnosis of cHCC-CC because of the lack of special features of the disease. We here present a case of a 68-year-old man who presented with fluctuant fever, chills, and sweating and was eventually diagnosed as cHCC-CC after surgery. The tumor was 6.0 cm in diameter with distinct borders and no satellite lesions or lymph nodes were observed during macroscopic examination of the resection specimen. The fever resolved in the postoperative period till the 28th day after surgery, when the patient developed extensive abdominal metastases and died shortly after. More attention should be paid to the patient with PLC showing abnormal features such as FUO, normal range of tumor markers, atypical imaging, and less cirrhosis. Hepatic resection is the treatment of choice although with short-term outcomes.
 ...
PMID:Combined hepatocellular-cholangiocarcinoma with fever of unknown origin: a case report and review of literature. 2406 20