Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describi...Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describing hepatic tumors in GSD patients; however, most of these reports were of hepatocellular adenomas, whereas there are only few reports describing focal nodular hyperplasia (FNH) or hepatocellular carcinoma (HCC). We report a case with GSD-Ia who had undergone a partial resection of the liver for FNH at 18 years of age and in whom moderately differentiated HCC had developed. Preoperative imaging studies, including ultrasonography, dynamic computer tomography (CT) and magnetic resonance imaging, revealed benign and malignant features. In particular, fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT revealed the atypical findings that FDG accumulated at high levels in the non-tumorous hepatic parenchyma and low levels in the tumor. Right hemihepatectomy was performed. During the perioperative period, high-dose glucose and sodium bicarbonate were administered to control metabolic acidosis. He had multiple recurrences of HCC at 10 mo after surgery and was followed-up with transcatheter arterial chemoembolization. The tumor was already highly advanced when it was found by chance; therefore, a careful follow-up should be mandatory for GSD-I patients as they are at a high risk for HCC, similar to hepatitis patients.展开更多
This report describes a hepatocellular carcinoma (HCC) with concomitant focal nodular hyperplasia (FNH) in a 56 years old Chinese man. There were two well circumscribed tumours measuring 3×2.5×2 cm and 2...This report describes a hepatocellular carcinoma (HCC) with concomitant focal nodular hyperplasia (FNH) in a 56 years old Chinese man. There were two well circumscribed tumours measuring 3×2.5×2 cm and 2×1.5×1.5 cm. The larger mass was grey and soft with a small area of bleeding and necrosis and an intact capsule. The smaller mass was yellow and had no capsule. Clonal analysis was carried out to clarify the relation between the HCC and the adjacent FNH. The clonal analysis was based on the methylation pattern of the polymorphic X chromosome linked androgen receptor gene (HUMARA). In FNH, after Hpa Ⅱ digestion, the allelic bands showed two well defined peaks. The intensity of the two peaks in the DNA from cirrhotic tissue did not differ significantly, consistent with a random pattern of X chromosome inactivation. However, in HCC, after Hpa Ⅱ digestion, the allelic bands differed significantly in intensity. Therefore, there was a typical polyclonal pattern of inactivation in FNH but the HCC was interpreted as being monoclonal.展开更多
We report a case of hepatocellular carcinoma (HCC) occurring in a patient with Crohn’s disease (CD) without chronic hepatitis or liver cirrhosis, and review the clinicopathological features of HCC in CD patients. A 3...We report a case of hepatocellular carcinoma (HCC) occurring in a patient with Crohn’s disease (CD) without chronic hepatitis or liver cirrhosis, and review the clinicopathological features of HCC in CD patients. A 37-year-old Japanese man with an 8-year history of CD and a medication history of azathioprine underwent resection of a liver tumor. The histopathology of the liver tumor was pseudoglandular type HCC. In the nonneoplastic liver, focal hepatocyte glycogenosis (FHG) was observed, however, there was no evidence of liver cirrhosis or primary sclerosing cholangitis. Only nine cases of HCC in CD patients have been reported previously in the English-language literature. Eight of 10cases (including the present case) had received azathioprine treatment, and four of these cases also showed FHG, which is considered a preneoplastic liver lesion, within the non-neoplastic liver. Although the precise mechanism of the development of HCC in CD patients is controversial, these results suggest that azathioprine therapy and FHG in the non-neoplastic liver contribute to the development of HCC. These findings also indicate that it is important to survey CD patients treated with prolonged azathioprine therapy for potential liver tumors.展开更多
Hepatocellular carcinoma (HCC) is the fifth most common cancer in the world and the third most common cause of cancer mortality. Despite the high numbers of patients diagnosed worldwide, HCC continues to pose challeng...Hepatocellular carcinoma (HCC) is the fifth most common cancer in the world and the third most common cause of cancer mortality. Despite the high numbers of patients diagnosed worldwide, HCC continues to pose challenging clinical problems. Good-quality ultra sound with careful evaluation of the entire liver can be a screening examination for HCC in patients at risk. The aim of the study was to determine the diagnostic accuracy of the Doppler sonography for differentiation of hepatocellular carcinoma (HCC) from other focal liver lesions by taking histopathology as a gold standard. It was a cross-sectional survey, conducted in the Department of Diagnostic Radiology, King Edward Medical University/Mayo Hospital, Lahore in one year from 13-12-2010 to 12-12-2011. A total of 70 patients were included in this study with focal lesions on ultrasound are included while pregnant patients are excluded. After evaluating by Doppler sonography and doing core biopsy, the patients were sent to the Department of Pathology, Mayo Hospital for histopathological examination. Histopathology was assessed for confirmation of positive and negative cases. Mean age of the patients was 60.9 ± 4.7 years. Out of 70 patients, 47 patients (67.1%) were male while remaining 23 patients (32.9%) were female. On Doppler sonography 37 cases were positive for hepatocellular carcinoma and 33 cases were negative while 36 cases were positive and 34 negative on histopathology. On Doppler sonography, sensitivity (94.4%), specificity (91.1%), diagnostic accuracy (92.8%), positive predictive value (91.8%) and negative predictive value was 93.9%. We concluded that Doppler sonography is a useful method for differen- tiation of Hepatocellular carcinoma from other focal liver lesions, detection and characterization of hepatocellular carcinoma.展开更多
AIM:To investigate the diagnostic performance of acoustic radiation force impulse(ARFI) elastography for characterizing focal liver mass by quantifying their stiffness.METHODS:This prospective study included 62 patien...AIM:To investigate the diagnostic performance of acoustic radiation force impulse(ARFI) elastography for characterizing focal liver mass by quantifying their stiffness.METHODS:This prospective study included 62 patients with a focal liver mass that was well visualized on conventional ultrasonography performed in our institution from February 2011 to November 2011.Among them,12 patients were excluded for ARFI measurement failure due to a lesion that was smaller than the region of the interest and at an inaccessible location(deeper than 8 cm)(n = 7) or poor compliance to hold their breath as required(n = 5).Finally,50 patients with valid ARFI measurements were enrolled.If a patient had multiple liver masses,only one mass of interest was chosen.The masses were diagnosed by histological examination or clinical diagnostic criteria.During ultrasonographic evaluation,stiffness,expressed as velocity,was checked 10 times per focal liver mass and the surrounding liver parenchyma.RESULTS:After further excluding three masses that were non-diagnostic on biopsy,a total of 47 focal mass lesions were tested,including 39(83.0%) malignant masses [24 hepatocellular carcinomas(HCC),seven cholangiocellular carcinomas(CCC),and eight liver metastases] and eight(17.0%) benign masses(five hemangiomas and three focal nodular hyperplasias,FNH).Thirty-seven(74.0%) masses were confirmed by histological examination.The mean velocity was 2.48 m/s in HCCs,1.65 m/s in CCCs,2.35 m/s in metastases,1.83 m/s in hemangiomas,and 0.97 m/s in FNHs.Although considerable overlap was still noted between malignant and benign masses,significant differences in ARFI values were observed between malignant and benign masses(mean 2.31 m/s vs 1.51 m/s,P = 0.047),as well as between HCCs and benign masses(mean 2.48 m/s vs 1.51 m/s,P = 0.006).The areas under the receiver operating characteristics curves(AUROC) for discriminating the malignant masses from benign masses was 0.724(95%CI,0.566-0.883,P = 0.048),and the AUROC for discriminating HCCs from benign masses was 0.813(95%CI,0.649-0.976,P = 0.008).To maximize the sum of sensitivity and specificity,an ARFI value of 1.82 m/s was selected as the cutoff value to differentiate malignant from benign liver masses.Furthermore,the cutoff value for distinguishing HCCs from benign masses was also determined to be 1.82 m/s.The diagnostic performance of the sum of the ARFI values for focal liver masses and the surrounding liver parenchyma to differentiate liver masses improved(AUROC = 0.853;95%CI,0.745-0.960;P = 0.002 in malignant liver masses vs benign ones and AUROC = 0.948;95%CI,0.896-0.992,P < 0.001 in HCCs vs benign masses).CONCLUSION:ARFI elastography provides additional information for the differential diagnosis of liver masses.However,our results should be interpreted in clinical context,because considerable overlap in ARFI values existed among liver masses.展开更多
Background and Aim: Hepatic focal lesions differ in their tissue composition and in the degree of stiffness, so our aim was to evaluate the role of Ultrasonic Transient Elastography (Fibroscan) in the measurement of h...Background and Aim: Hepatic focal lesions differ in their tissue composition and in the degree of stiffness, so our aim was to evaluate the role of Ultrasonic Transient Elastography (Fibroscan) in the measurement of hepatic focal lesions stiffness in order to differentiate hepatocellular carcinoma (HCC) focal lesions from other non HCC focal lesions. Methods: The study was conducted on 34 patients with hepatic focal lesion(s) in the right lobe located near the liver surface and more than or equal4 cmin diameter, detected by imaging studies and diagnosed by CT and histopathology. Stiffness over the focal lesions was measured by the fibroscan. Results: The median value of stiffness was 72.5 kPa over HCC focal lesions, 17.2 kPa over lymphoma focal lesions, 6.5 kPa over metastatic focal lesions and 10.5 kPa over the sarcoidosis focal lesion. Conclusions: Hepatocellular carcinoma focal lesions are much stiffer than lymphoma, metastasis or sarcoidosis focal lesions. Fibroscan may be a useful non-invasive method in the prediction of hepatocellular carcinoma in the future.展开更多
The extensive use of imaging techniques in differential diagnosis of abdominal conditions and screening of hepatocellular carcinoma in patients with chronic hepatic diseases,has led to an important increase in identif...The extensive use of imaging techniques in differential diagnosis of abdominal conditions and screening of hepatocellular carcinoma in patients with chronic hepatic diseases,has led to an important increase in identification of focal liver lesions.The development of contrastenhanced ultrasound(CEUS) opens a new window in the diagnosis and follow-up of these lesions.This technique offers obvious advantages over the computed tomography and magnetic resonance,without a decrease in its sensitivity and specificity.The new second generation contrast agents,due to their intravascular distribution,allow a continuous evaluation of the enhancement pattern,which is crucial in characterization of liver lesions.The dual blood supply in the liver shows three different phases,namely arterial,portal and late phases.The enhancement during portal and late phases can give important information about the lesion's behavior.Each liver lesion has a different enhancement pattern that makes possible an accurate approach to their diagnosis.The role of emerging techniques as a contrastenhanced three-dimensional US is also discussed.In this article,the advantages,indications and technique employed during CEUS and the different enhancement patterns of most benign and malignant focal liver lesions are discussed.展开更多
文摘Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describing hepatic tumors in GSD patients; however, most of these reports were of hepatocellular adenomas, whereas there are only few reports describing focal nodular hyperplasia (FNH) or hepatocellular carcinoma (HCC). We report a case with GSD-Ia who had undergone a partial resection of the liver for FNH at 18 years of age and in whom moderately differentiated HCC had developed. Preoperative imaging studies, including ultrasonography, dynamic computer tomography (CT) and magnetic resonance imaging, revealed benign and malignant features. In particular, fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT revealed the atypical findings that FDG accumulated at high levels in the non-tumorous hepatic parenchyma and low levels in the tumor. Right hemihepatectomy was performed. During the perioperative period, high-dose glucose and sodium bicarbonate were administered to control metabolic acidosis. He had multiple recurrences of HCC at 10 mo after surgery and was followed-up with transcatheter arterial chemoembolization. The tumor was already highly advanced when it was found by chance; therefore, a careful follow-up should be mandatory for GSD-I patients as they are at a high risk for HCC, similar to hepatitis patients.
文摘This report describes a hepatocellular carcinoma (HCC) with concomitant focal nodular hyperplasia (FNH) in a 56 years old Chinese man. There were two well circumscribed tumours measuring 3×2.5×2 cm and 2×1.5×1.5 cm. The larger mass was grey and soft with a small area of bleeding and necrosis and an intact capsule. The smaller mass was yellow and had no capsule. Clonal analysis was carried out to clarify the relation between the HCC and the adjacent FNH. The clonal analysis was based on the methylation pattern of the polymorphic X chromosome linked androgen receptor gene (HUMARA). In FNH, after Hpa Ⅱ digestion, the allelic bands showed two well defined peaks. The intensity of the two peaks in the DNA from cirrhotic tissue did not differ significantly, consistent with a random pattern of X chromosome inactivation. However, in HCC, after Hpa Ⅱ digestion, the allelic bands differed significantly in intensity. Therefore, there was a typical polyclonal pattern of inactivation in FNH but the HCC was interpreted as being monoclonal.
文摘We report a case of hepatocellular carcinoma (HCC) occurring in a patient with Crohn’s disease (CD) without chronic hepatitis or liver cirrhosis, and review the clinicopathological features of HCC in CD patients. A 37-year-old Japanese man with an 8-year history of CD and a medication history of azathioprine underwent resection of a liver tumor. The histopathology of the liver tumor was pseudoglandular type HCC. In the nonneoplastic liver, focal hepatocyte glycogenosis (FHG) was observed, however, there was no evidence of liver cirrhosis or primary sclerosing cholangitis. Only nine cases of HCC in CD patients have been reported previously in the English-language literature. Eight of 10cases (including the present case) had received azathioprine treatment, and four of these cases also showed FHG, which is considered a preneoplastic liver lesion, within the non-neoplastic liver. Although the precise mechanism of the development of HCC in CD patients is controversial, these results suggest that azathioprine therapy and FHG in the non-neoplastic liver contribute to the development of HCC. These findings also indicate that it is important to survey CD patients treated with prolonged azathioprine therapy for potential liver tumors.
文摘Hepatocellular carcinoma (HCC) is the fifth most common cancer in the world and the third most common cause of cancer mortality. Despite the high numbers of patients diagnosed worldwide, HCC continues to pose challenging clinical problems. Good-quality ultra sound with careful evaluation of the entire liver can be a screening examination for HCC in patients at risk. The aim of the study was to determine the diagnostic accuracy of the Doppler sonography for differentiation of hepatocellular carcinoma (HCC) from other focal liver lesions by taking histopathology as a gold standard. It was a cross-sectional survey, conducted in the Department of Diagnostic Radiology, King Edward Medical University/Mayo Hospital, Lahore in one year from 13-12-2010 to 12-12-2011. A total of 70 patients were included in this study with focal lesions on ultrasound are included while pregnant patients are excluded. After evaluating by Doppler sonography and doing core biopsy, the patients were sent to the Department of Pathology, Mayo Hospital for histopathological examination. Histopathology was assessed for confirmation of positive and negative cases. Mean age of the patients was 60.9 ± 4.7 years. Out of 70 patients, 47 patients (67.1%) were male while remaining 23 patients (32.9%) were female. On Doppler sonography 37 cases were positive for hepatocellular carcinoma and 33 cases were negative while 36 cases were positive and 34 negative on histopathology. On Doppler sonography, sensitivity (94.4%), specificity (91.1%), diagnostic accuracy (92.8%), positive predictive value (91.8%) and negative predictive value was 93.9%. We concluded that Doppler sonography is a useful method for differen- tiation of Hepatocellular carcinoma from other focal liver lesions, detection and characterization of hepatocellular carcinoma.
基金Supported by A Grant of the Korea Healthcare Technology R and D Project,Ministry of Health and Welfare,South Korea, A102065
文摘AIM:To investigate the diagnostic performance of acoustic radiation force impulse(ARFI) elastography for characterizing focal liver mass by quantifying their stiffness.METHODS:This prospective study included 62 patients with a focal liver mass that was well visualized on conventional ultrasonography performed in our institution from February 2011 to November 2011.Among them,12 patients were excluded for ARFI measurement failure due to a lesion that was smaller than the region of the interest and at an inaccessible location(deeper than 8 cm)(n = 7) or poor compliance to hold their breath as required(n = 5).Finally,50 patients with valid ARFI measurements were enrolled.If a patient had multiple liver masses,only one mass of interest was chosen.The masses were diagnosed by histological examination or clinical diagnostic criteria.During ultrasonographic evaluation,stiffness,expressed as velocity,was checked 10 times per focal liver mass and the surrounding liver parenchyma.RESULTS:After further excluding three masses that were non-diagnostic on biopsy,a total of 47 focal mass lesions were tested,including 39(83.0%) malignant masses [24 hepatocellular carcinomas(HCC),seven cholangiocellular carcinomas(CCC),and eight liver metastases] and eight(17.0%) benign masses(five hemangiomas and three focal nodular hyperplasias,FNH).Thirty-seven(74.0%) masses were confirmed by histological examination.The mean velocity was 2.48 m/s in HCCs,1.65 m/s in CCCs,2.35 m/s in metastases,1.83 m/s in hemangiomas,and 0.97 m/s in FNHs.Although considerable overlap was still noted between malignant and benign masses,significant differences in ARFI values were observed between malignant and benign masses(mean 2.31 m/s vs 1.51 m/s,P = 0.047),as well as between HCCs and benign masses(mean 2.48 m/s vs 1.51 m/s,P = 0.006).The areas under the receiver operating characteristics curves(AUROC) for discriminating the malignant masses from benign masses was 0.724(95%CI,0.566-0.883,P = 0.048),and the AUROC for discriminating HCCs from benign masses was 0.813(95%CI,0.649-0.976,P = 0.008).To maximize the sum of sensitivity and specificity,an ARFI value of 1.82 m/s was selected as the cutoff value to differentiate malignant from benign liver masses.Furthermore,the cutoff value for distinguishing HCCs from benign masses was also determined to be 1.82 m/s.The diagnostic performance of the sum of the ARFI values for focal liver masses and the surrounding liver parenchyma to differentiate liver masses improved(AUROC = 0.853;95%CI,0.745-0.960;P = 0.002 in malignant liver masses vs benign ones and AUROC = 0.948;95%CI,0.896-0.992,P < 0.001 in HCCs vs benign masses).CONCLUSION:ARFI elastography provides additional information for the differential diagnosis of liver masses.However,our results should be interpreted in clinical context,because considerable overlap in ARFI values existed among liver masses.
文摘Background and Aim: Hepatic focal lesions differ in their tissue composition and in the degree of stiffness, so our aim was to evaluate the role of Ultrasonic Transient Elastography (Fibroscan) in the measurement of hepatic focal lesions stiffness in order to differentiate hepatocellular carcinoma (HCC) focal lesions from other non HCC focal lesions. Methods: The study was conducted on 34 patients with hepatic focal lesion(s) in the right lobe located near the liver surface and more than or equal4 cmin diameter, detected by imaging studies and diagnosed by CT and histopathology. Stiffness over the focal lesions was measured by the fibroscan. Results: The median value of stiffness was 72.5 kPa over HCC focal lesions, 17.2 kPa over lymphoma focal lesions, 6.5 kPa over metastatic focal lesions and 10.5 kPa over the sarcoidosis focal lesion. Conclusions: Hepatocellular carcinoma focal lesions are much stiffer than lymphoma, metastasis or sarcoidosis focal lesions. Fibroscan may be a useful non-invasive method in the prediction of hepatocellular carcinoma in the future.
文摘The extensive use of imaging techniques in differential diagnosis of abdominal conditions and screening of hepatocellular carcinoma in patients with chronic hepatic diseases,has led to an important increase in identification of focal liver lesions.The development of contrastenhanced ultrasound(CEUS) opens a new window in the diagnosis and follow-up of these lesions.This technique offers obvious advantages over the computed tomography and magnetic resonance,without a decrease in its sensitivity and specificity.The new second generation contrast agents,due to their intravascular distribution,allow a continuous evaluation of the enhancement pattern,which is crucial in characterization of liver lesions.The dual blood supply in the liver shows three different phases,namely arterial,portal and late phases.The enhancement during portal and late phases can give important information about the lesion's behavior.Each liver lesion has a different enhancement pattern that makes possible an accurate approach to their diagnosis.The role of emerging techniques as a contrastenhanced three-dimensional US is also discussed.In this article,the advantages,indications and technique employed during CEUS and the different enhancement patterns of most benign and malignant focal liver lesions are discussed.
