Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.
Antigens, CD34/metabolism ; Bone Neoplasms/diagnosis/secondary ; Diagnosis, Differential ; Hemangioendothelioma, Epithelioid/*diagnosis/pathology/ultrasonography ; Humans ; Liver Cirrhosis, Alcoholic/complications/diagnosis ; Liver Neoplasms/*diagnosis/pathology/ultrasonography ; Male ; Pancreatic Neoplasms/diagnosis/secondary ; Rupture, Spontaneous ; Tomography, X-Ray Computed

We report on a case of a 57-year-old male who underwent a curative resection for hepatocellular carcinoma (HCC) with histological confirmation of a spontaneously necrotized tumor. Initial serum AFP level was 4,778 ng/mL. A 3.7 cm hyperechoic mass in segment 6 of the liver was observed on ultrasonography and dynamic contrast-enhanced liver MRI showed a 3.7x3.1 cm sized HCC. He was scheduled to undergo curative surgical resection under the clinical diagnosis of an early stage HCC (Barcelona Clinic Liver Cancer stage A). Without treatment, the serum AFP level declined rapidly to 50 ng/mL over five weeks. He underwent curative wedge resection of segment 6 of the liver. Histology revealed complete necrosis of the mass rimmed by inflamed fibrous capsule on a background of HBV-related cirrhosis with infiltration of lymphoplasma cells. Exact pathophysiology underlying this event is unknown. Among the proposed mechanisms of spontaneous neoplastic remission of HCC, circulatory disturbance and activation of host immune response offer the most scientific explanation for the complete histologic necrosis of HCC in the resected mass seen in our patient.
Carcinoma, Hepatocellular/*diagnosis/diagnostic imaging/pathology ; Hepatitis B/complications/diagnosis ; Humans ; Liver/diagnostic imaging/pathology ; Liver Cirrhosis/etiology ; Liver Neoplasms/*diagnosis/diagnostic imaging/pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Necrosis ; Radiography ; Remission, Spontaneous ; Ultrasonography ; alpha-Fetoproteins/analysis

Breast cancer is a rare disease in men. We report a case of 53-year-old obese male, with known cryptogenic cirrhosis and hepatocellular carcinoma, presenting a tender mass on left breast. He was diagnosed with invasive intraductal carcinoma, which was consistent with a sporadic lesion. On the basis of previous literatures, obesity can be regarded as a cause for breast cancer even in men. However, there has been inconsistent data about link between liver cirrhosis and male breast cancer, which can be due to heterogenity in the etiology of cirrhosis. Through this case, it can be postulated that the risk for male breast cancer may vary according to the etiology of cirrhosis.
Breast Neoplasms, Male/*etiology/secondary/ultrasonography ; Carcinoma, Hepatocellular/diagnosis/pathology ; Humans ; Immunohistochemistry ; Liver Cirrhosis/complications/*diagnosis/pathology ; Liver Neoplasms/diagnosis/pathology ; Male ; Middle Aged ; Receptors, Estrogen/metabolism ; Tomography, X-Ray Computed

Adult ; Aged ; Biopsy, Needle ; Female ; Hepatitis B, Chronic ; diagnosis ; pathology ; Humans ; Liver ; diagnostic imaging ; pathology ; Liver Cirrhosis ; diagnosis ; pathology ; Male ; Middle Aged ; Severity of Illness Index ; Ultrasonography, Doppler ; Young Adult

OBJECTIVEThe purpose of this study was to investigate the clinical and genetic characteristics of autosomal recessive polycystic kidney disease.

METHODTargeted sequencing was used on a children who was accurately diagnosed as autosomal recessive polycystic kidney disease in Peking Union Medical College Hospital to analyze the major clinical manifestations of the disease. An analysis of the PKHD1 genes was made on the patient, and then verified by polymerase chain reaction (PCR). And the related literature was reviewed also.

RESULTThe patient was a boy, 2 years and 3 months old, and had abdominal distention for about one year. The abdominal ultrasound suggested diffuse liver lesions, mild intrahepatic bile duct dilatation, structure disturbance of both kidneys, appearance of multiple strong echo. The child was clinically highly suspected of polycystic kidney disease. Targeted sequencing showed two mutations in exon 32 and exon 50 of PKHD1 gene, respectively, c.4274T > G, leading to p.Leu1425Arg, c.7973T > A, leading to p.Leu2658Ter. Verified by PCR, the father has one mutation of c.4274T > G.

CONCLUSIONThe clinical manifestations of autosomal recessive polycystic kidney disease are multiple renal cyst, cyst of liver and liver fibrosis, intrahepatic bile duct dilatation. Two mutations (c.4274T > G, c.7973T > A) in PKHD1 gene may be pathogenic.

Child, Preschool ; DNA Mutational Analysis ; Exons ; genetics ; Humans ; Kidney ; diagnostic imaging ; pathology ; Liver ; diagnostic imaging ; pathology ; Liver Cirrhosis ; pathology ; Male ; Mutation ; Polycystic Kidney, Autosomal Recessive ; diagnosis ; genetics ; pathology ; Polymerase Chain Reaction ; Receptors, Cell Surface ; genetics ; Sequence Homology, Amino Acid ; Ultrasonography

BACKGROUND/AIMS: The red-blood-cell distribution width (RDW) is a newly recognized risk marker in patients with cardiovascular disease, but its role in nonalcoholic fatty liver disease (NAFLD) has not been well defined. The aim of the present study was to determine the association between RDW values and the level of fibrosis in NAFLD according to BARD and FIB-4 scores. METHODS: This study included 24,547 subjects who had been diagnosed with NAFLD based on abdominal ultrasonography and questionnaires about alcohol consumption. The degree of liver fibrosis was determined according to BARD and FIB-4 scores. The association between RDW values and the degree of fibrosis in NAFLD was analyzed retrospectively. RESULTS: After adjusting for age, hemoglobin level, mean corpuscular volume, history of hypertension, history of diabetes, and high-sensitivity C-reactive protein, the RDW values were 12.61+/-0.41% (mean+/-SD), 12.70+/-0.70%, 12.77+/-0.62%, 12.87+/-0.82%, and 13.25+/-0.90% for those with BARD scores of 0, 1, 2, 3, and 4, respectively, and 12.71+/-0.72%, 12.79+/-0.66%, and 13.23+/-1.52% for those with FIB-4 scores of <1.30, 1.31-2.66, and > or =2.67, respectively (P<0.05). The prevalence of advanced fibrosis (BARD score of 24 and FIB-4 score of > or =1.3) increased with the RDW [BARD score: 51.1% in quartile 1 (Q1) vs. 63.6% in Q4; FIB-4 score: 6.9% in Q1 vs. 10.5% in Q4; P<0.001]. After adjustments, the odds ratio of having advanced fibrosis for those in Q4 compared to Q1 were 1.76 (95%CI=1.55-2.00, P<0.001) relative to BARD score and 1.69 (95%CI=1.52-1.98, P<0.001) relative to FIB-4 score. CONCLUSIONS: Elevated RDW is independently associated with advanced fibrosis in NAFLD.
Adult ; Alcohol Drinking ; C-Reactive Protein/analysis ; Diabetes Mellitus/pathology ; Erythrocyte Indices ; Fatty Liver/complications/*diagnosis/ultrasonography ; Female ; Humans ; Hypertension/pathology ; Liver Cirrhosis/*diagnosis/epidemiology/etiology ; Male ; Middle Aged ; Odds Ratio ; Prevalence ; Questionnaires ; Severity of Illness Index

BACKGROUND/AIMS: Transient elastography (Fibroscan, Echosens, France) is a new, simple, and non-invasive method to assess the degree of hepatic fibrosis by measuring liver stiffness. Recent reports have shown that liver stiffness measurement using Fibroscan allowed accurate prediction of hepatic fibrosis in patient with chronic hepatitis C. The aim of this study was to evaluate accuracy of Fibroscan for the detection of hepatic fibrosis in Korea with various etiologies of chronic liver disease by comparison with fibrosis assessed by histologic examination. METHODS: Fifty-four patients with chronic liver diseases, which were histologically confirmed within recent 6 months were enrolled. Etiologies were HBV, HCV infection, autoimmune hepatitis, and non alcoholic steatohepatitis. Hepatic fibrosis was graded on the basis of standard guideline proposed by the Korean Study Group for the Pathology of Digestive Diseases. RESULTS: Fibroscan values were significantly higher in F3 (16.96 kPa) and F4 (19.86 kPa) than others (p=0.003). Liver stiffness measurement was significantly correlated to the fibrosis stage (r=0.614, p<0.0001). CONCLUSIONS: Liver stiffness measurement by Fibroscan is a promising method for the assessment of hepatic fibrosis in chronic liver disease because it accompanies no complication.
Adult ; Aged ; Biological Markers/blood ; Chronic Disease ; Elasticity ; Elasticity Imaging Techniques/*methods ; Humans ; Liver Cirrhosis/diagnosis/etiology/*ultrasonography ; Liver Diseases/complications/pathology/*ultrasonography ; Male ; Middle Aged ; Predictive Value of Tests ; ROC Curve ; Regression Analysis ; Sensitivity and Specificity

OBJECTIVETo assess the clinical utility of liver fibrosis indexes determination in patients with nonalcoholic fatty liver disease.

METHODSLiver fibrosis indexes of 79 patients with fatty liver, diagnosed by B ultrasonic echography, 37 patients with posthepatitic cirrhosis patients and 32 healthy subjects were determined.

RESULTSThe level of procollagen III(PC III), hyaluronic acid (HA), collagen IV(C IV), laminin (LN) in the fatty liver cases were significantly higher than those of healthy subjects (P<0.05 - 0.01), but lower than those in the liver cirrhosis cases (P<0.01). The difference of PC III, C IV, LN between the severe and early fatty liver cases was significant (P<0.05 - 0.01). Positive rate of these indexes was higher in severe cases.

CONCLUSIONThere is a trend of liver fibrosis in severe fatty liver patients. Combined determination of liver fibrosis indexes may help the diagnosis of liver fibrosis in the fatty liver patients.

Adult ; Alanine Transaminase ; blood ; Collagen Type III ; blood ; Collagen Type IV ; blood ; Fatty Liver ; blood ; diagnostic imaging ; pathology ; Female ; Humans ; Hyaluronic Acid ; blood ; Laminin ; blood ; Liver Cirrhosis ; blood ; diagnosis ; Male ; Middle Aged ; Ultrasonography

OBJECTIVETo investigate the correlation between the stage of hepatic fibrosis and ultrasonographic findings of the liver, spleen and gallbladder and establish a sensitive ultrasonographic semi-quantitative scoring system for screening compensated liver cirrhosis.

METHODSTotalling 248 patients with chronic hepatitis B and hepatitis C virus infection underwent liver biopsy and ultrasonic examination. The images of the liver surface, parenchymal echo, intrahepatic vessels, gallbladder, spleen and diameter of portal vein were analyzed.

RESULTSThe stages of hepatic fibrosis were not correlated to ultrasonographic findings of the liver surface or diameter of portal vein, but hepatic fibrosis of different stages showed significant differences in parenchymal echo, intrahepatic vessels, gallbladder and splenomegaly. In cases with normal liver parenchymal, intrahepatic vessels, gallbladder and spleen, the negative predictive value of the ultrasonographic semi-quantitative scoring system for diagnosing compensated liver cirrhosis amounted to 96.3%. The sensitivity of a score not lower than 5 was 90% for detecting compensated cirrhosis. With a score not lower than 7, the diagnostic accuracy and specificity was 85.9% and 95.2%, respectively, but the sensitivity was lowered to 37.5%.

CONCLUSIONThe ultrasonic images of the liver parenchyma, intrahepatic vessels, gallbladder and spleen in patients with compensated liver cirrhosis vary significantly in patients with hepatic fibrosis of different stages, and this ultrasonographic scoring system allows for a sensitive diagnosis of compensated cirrhosis.

Female ; Fibrosis ; Gallbladder ; diagnostic imaging ; Hepatitis B, Chronic ; complications ; Hepatitis C ; complications ; Humans ; Liver ; diagnostic imaging ; pathology ; virology ; Liver Cirrhosis ; complications ; diagnosis ; Male ; Reproducibility of Results ; Sensitivity and Specificity ; Spleen ; diagnostic imaging ; Splenomegaly ; diagnostic imaging ; Ultrasonography ; methods

BACKGROUND: Hepatolithiasis is rarely associated with a cholangiocarcinoma and the etiologic relationship between them has not been proved. Hepatolithiasis is a risk factor for cholangiocarcinoma. Accurate preoperative diagnosis remains low, and the survival outcome is still dismal, despite improvements in imaging studies. METHODS: Eighteen cases of intrahepatic cholangiocarcinomas among the 522 cases undergoing treatments from Jan. 1984 to May 1998 for hepatolithiasis at the Department of Surgery, Kyung Hee University Hospital were reviewed. RESULTS: The patients had a significantly long duration of symptoms, a high incidence of previous biliary surgery, and a lower rate of preoperative diagnosis. The eighteen cases accounted for 3.4% of the hepatolithiasis cases and 8.5% of the cholangiocarcinomas. There were 17 women and 1 man, and the most prevalent age group was in the 5th and the 6th decades. HBsAg was positive in one case who had chronic hepatitis, 3 patients had clonorchiasis, and 2 patients had liver cirrhosis. CEA and CA19-9 were elevated in 42.9% (3/7) and 27.3% (3/11) of the patients. Various imaging studies, including ultrasonography, CT, choangiography, angiography, and ERCP were performed for diagnosis. The malignant lesions could not be detected preoperatively in 9 patients, and the presence of a coexisting malignant tumor was confirmed by frozen section or by permanent tissue pathology. The operative methods were as follows; a lobectomy in 4 patients, a segmentectomy in 4 patients, and a CBD resection for palliation or explo-laparotomy only in 4 patients because of multiple hepatic metastases. During the operation, the exact location of the stones and the gross pathology of the tumor were recorded in each case. The left side was more prevalent than the right. The mean survival was 15 months for resection group and 9 months for non resection group. CONCLUSIONS: The lower survival rates may be attributed to delayed diagnosis, lower diagnostic accuracy, and relatively fewer curative resections in the patients with stone-containing cholangiocarcinomas. Hepatolithiasis is one of the risk factors of a cholangiocarcinoma. During the diagnostic and operative procedures, we should not overlook a cholangiocarcinoma which can coexist with hepatolithiasis, and we should examine tumor markers, the results of imaging studies and tissue examinations, and the operative findings.
Angiography ; Biomarkers, Tumor ; Cholangiocarcinoma* ; Cholangiopancreatography, Endoscopic Retrograde ; Clonorchiasis ; Delayed Diagnosis ; Diagnosis ; Female ; Frozen Sections ; Hepatitis B Surface Antigens ; Hepatitis, Chronic ; Humans ; Incidence ; Liver Cirrhosis ; Mastectomy, Segmental ; Neoplasm Metastasis ; Pathology ; Risk Factors ; Surgical Procedures, Operative ; Survival Rate ; Ultrasonography