Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019204 (hepatocellular carcinoma)
 71,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Peripheral/Intrahepatic Cholangiocarcinoma (IHC), a malignant epithelial tumor originating from the intrahepatic bile ducts, is the second most common primary liver cancer after hepatocellular carcinoma. Unlike hepatocellular carcinoma, however, IHC is infrequently associated with chronic underlying liver disease. A recent study from the United States has demonstrated a 9% annual percentage increase and an overall 10-fold increase in mortality related to IHC since 1973. Despite this observation, however, IHC remains a rare disease in Western countries. Of the approximately 4000 patients seen at Memorial Sloan-Kettering Cancer Center with hepatic lesions since from 1995-2001, 7% had a diagnosis of cholangiocarcinoma and only 1% had IHC. As a result, progress in elucidating the pathogenesis and clinical behavior of these tumors has been slow. However, the increasing number of reports in recent years has increased awareness of IHC and provided some insight into its biology. At present, complete resection is the only therapy that offers the possibility of long-term survival. Single agent or combination chemotherapy and radiation therapy have not been shown to have a significant impact, either as primary treatment or as an adjuvant to resection. This report reviews the relevant literature pertaining to IHC with emphasis on clinical presentation, radiologic evaluation, treatment and outcome.
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PMID:Intrahepatic cholangiocarcinoma. Current management. 1460 59

Focal eosinophilic infiltration is a rare disease entity that in patients with malignancies may mimic malignant hepatic nodules. We describe two cases in which focal eosinophilic infiltration of the liver, as well as primary liver cancer, occurred. In these patients, imaging findings similar to those observed in hepatocellular carcinoma (HCC) were noted: Enhancement at dynamic magnetic resonance (MR) imaging substantially increased perfusion at computed tomography during hepatic arteriography (CTHA) and a perfusion defect at computed tomography during arterial portography (CTAP).
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PMID:Focal eosinophilic infiltration of the liver mimicking hepatocellular carcinoma: case reports. 1499 46

Primary hepatic mucosa-associated lymphoid tissue (MALT) lymphoma is an extremely rare disease. A 65-year-old female patient with chronic hepatitis B presented with multiple solid masses in segment (S) 4, S5, and S6 of the liver. The nodule in S5 was diagnosed preoperatively as hepatocellular carcinoma by computed tomography, magnetic resonance imaging, and angiography. The nodule in S4 was initially interpreted as lymphoid follicles by needle biopsy. Segmentectomy of S5 and partial resection of S6 were performed. Microscopic examination of the S5 nodule revealed moderately differentiated hepatocellular carcinoma. The nodule from S6 showed nodular proliferation of atypical intermediate to medium-sized lymphoid cells in the portal area and lymph epithelial lesions of bile ducts. The atypical lymphoid cells were positive for LCA, L-26 and bcl-2 and negative for UCHL-1. These features were consistent with the diagnosis of MALT lymphoma. This is the first case report of synchronous hepatic MALT lymphoma and hepatocellular carcinoma associated with chronic hepatitis B.
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PMID:Hepatic mucosa-associated lymphoid tissue lymphoma and hepatocellular carcinoma in a patient with hepatitis B virus infection. 1536 14

Primary liver lymphoma is a very rare disease and is frequently overlooked as a possible diagnosis. We report the case of an asymptomatic middle-aged man with chronic hepatitis C who developed primary liver lymphoma (PLL). A large solitary tumor in the left lobe of the liver was incidentally detected on routine ultrasound examination. Imaging studies showed mixed iso- and hypoechogenicity with hypoechoic rim, hypodense in the pre-contrast phase and thick wall enhancement in the post-contrast phase on computed tomographic study, hypointensity on T1WI, and hyperintensity of the central portion and slightly higher intensity in the peripheral wall on T2WI. These pictures were different from focal nodular hyperplasia, hepatocellular carcinoma, cholangiocarcinoma or metastases. Atypical hepatectomy was performed and the pathology of the hepatic tumor revealed non-Hodgkin's lymphoma. Systemic staging revealed no evidence of nodal or bone marrow involvement, so PLL was diagnosed. There was no tumor recurrence more than 4 years after operation and chemotherapy. PLL should be included in the differential diagnosis of solitary hepatic tumor in patients who are hepatitis C virus-positive, and who have atypical imaging and no known malignancy or elevated tumor marker levels.
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PMID:Primary liver lymphoma in a patient with chronic hepatitis C. 1652 Aug 42

Diamond Blackfan anemia (DBA) is a rare disease characterized by aplasia or hypoplasia of erythroid lineage. Normochromic, usually macrocytic, but occasionally normocytic anemia and reticulocytopenia are characteristic findings of DBA. DBA is associated with an increased risk of malignancy. Most of the reported malignancies are acute myeloid leukemia. Solid tumors including hepatocellular carcinoma and osteosarcoma have also been identified. We could find 29 reported cases with DBA and malignancy. Two of them were diagnosed as Hodgkin lymphoma at 15 and 23 years, respectively. Here we report a 7-year-old boy with DBA who developed Hodgkin disease.
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PMID:Hodgkin lymphoma in a child with Diamond Blackfan anemia. 1667 21

We document a case of testicular metastasis from hepatocellular carcinoma. The patient suffered from bilateral testicular painful swelling for 6 months. Scrotal ultrasonography showed bilateral testicular tumors and the whole abdominal computed tomography revealed a huge tumor in the left lobe of the liver. Bilateral orchiectomy and postoperative ultrasound-guided liver biopsy were done. Pathological examination revealed metastatic hepatocellular carcinoma. Hepatocellular carcinoma with testicular metastases is a very rare disease.
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PMID:Testicular metastasis from hepatocellular carcinoma. 1688 85

A primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) is very rare. We found a solitary mass 27 mm in size in the left lobe of the liver of a 58-year-old Japanese man with a history of hepatitis-C infection. Based on the results of imaging studies, the tumor was diagnosed as a hepatocellular carcinoma (HCC). The left lobe of the liver was lobectomized and microscopic findings showed that the tumor was a hepatic MALT lymphoma, while immunohistochemistry showed it to be positive for CD20 and CD79a. In a fluorodeoxyglucose-positron emission tomography examination integrated with computed tomography scanning (FDG-PET CT) before surgery, the tumor was revealed to have a high standardized uptake value (SUV) for FDG. The patient received chemotherapy after surgery. To the best of our knowledge, 45 cases had been reported with a mean age for all patients of 61.4 years. The pathogenesis remains unclear, although half of the patients had a past history of chronic inflammatory liver disease. Surgical resection was performed in most cases and some patients received postoperative chemotherapy or radiotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.
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PMID:Primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type: case report and review of the literature. 1880 27

Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to inferior vena cava, resulting from thrombosis or its fibrous sequellae. This rare disease affects mainly young adults. Multiple risk factors have been identified and are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients; their diagnosis can be made by showing the V617F mutation in Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes at bone marrow biopsy. Presentation and manifestations are extremely varied, so that the diagnosis must be considered in any patient with acute or chronic liver disease. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences, the collaterals to hepatic veins or inferior vena cava. The disease is considered to be spontaneously lethal within 3 years of first symptoms. A therapeutic strategy has been proposed where anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension are used first; then angioplasty for short-length venous stenoses; then TIPS; and ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 90%. Medium-term prognosis depends on the severity of liver disease. Long-term outcome might be jeopardized by transformation of underlying conditions and hepatocellular carcinoma.
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PMID:Primary Budd-Chiari syndrome. 1901 88

Since the introduction of hepatitis B immunoglobulin and nucleoside/nucleotide antivirals in the 1990's, outcomes of LT for hepatitis B virus (HBV)-related liver disease, regardless of whether for decompensated cirrhosis, hepatocellular carcinoma satisfying Milan criteria or fulminant hepatic failure (FHF), have been favorable with results comparable if not better to other liver transplant recipients. Unfortunately the same optimism does not hold true for hepatitis C which differs from post- transplant hepatitis B in many ways, most striking of which are the limited options for treatment of recurrent hepatitis C (HCV). As time has passed, the initial enthusiasm for liver transplantation for HCV has waned as the original excellent five year survival rates have now translated into disappointing medium- and long-term survival data. Cirrhosis can also develop in between 10-25% of patients by five years post-transplant which in turn has led to recurrent HCV-related cirrhosis emerging as an important yet controversial indication for retransplantation. A variety of diseases can cause FHF with drug-related hepatotoxicity, particularly from acetaminophen accounting for 50-60% of cases in United Kingdom and the United States while viral hepatitis appears to be declining as a cause. Although FHF is a relatively rare disease affecting approximately 2000 patients per year in the United States, it is associated with high morbidity and mortality without transplantation yet only 25% of patients in the United States undergo liver transplantation. This review article will discuss liver transplantation for HBV and HCV and will conclude with reviewing the etiology, epidemiology and management of FHF.
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PMID:Liver transplant for viral hepatitis and fulminant hepatic failure. 1921 11

Hemangioma is one of the most frequently encountered benign hepatic neoplasm which can develop secondary degeneration. Sclerosed hemangioma is a rare disease histologically characterized by large amount of collagen and elastic fibril between sclerosed small vessels. Its differential diagnosis is very difficult. It should be included in the differential diagnosis of other hepatic lesions such as hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic hepatic tumor. A 77-year old male was admitted with upper abdominal discomfort. Abdominal ultrasonography revealed GB stone, dilated common bile duct with bile duct stone, and a 4.6 cm sized hyperechoic mass at segment 5 and 6 of the liver. Abdominal dynamic computed tomography demonstrated dilated intrahepatic bile ducts and a 5 x 5 cm sized mass which showed minimally delayed enhancement. Abdominal magnetic resonance imaging revealed the mass with low signal intensity in T1 weighted image, high signal intensity and focal low signal in T2 weighted image which showed minimal enhancement. We removed common bile duct stone with endoscopic retrograde cholangiopancreatography then decided to undergo right lower segmentectomy of liver due to possibility of cholangiocarcinoma. Histopathological examination of hepatic mass showed large amount of fibrous tissue with occasional residual vascular channels. We describe one case of sclerosed hemangioma mimicking cholangiocarcinoma.
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PMID:[A case of sclerosed hemangioma mimicking intrahepatic cholangiocarcinoma]. 2002 96


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