1/53. recurrence of hepatocellular carcinoma as a mixed hepatoblastoma after liver transplantation.BACKGROUND: hepatoblastoma is an exceptional cause of primary malignant liver tumour in the adult. PATIENT: The case is reported of an adult patient transplanted for alcoholic cirrhosis complicated by multifocal hepatocellular carcinoma in whom a recurrence in the form of a mixed hepatoblastoma invading the whole transplanted liver developed three months after liver transplantation. methods: Complete clinical, histopathological, and immunohistochemical data were reviewed. RESULTS: The recurrent tumour invaded the whole liver. The major component was a mixed hepatoblastoma, with an epithelial component expressing cytokeratin and a mesenchymal component expressing vimentin. The tumour also contained a minor hepatocarcinomatous component expressing alpha fetoprotein. The rapid growth of the tumour prevented any attempt at treatment. Although direct evidence is lacking, the most likely hypothesis to explain the observations is a marked phenotypic change in the initial malignant population at recurrence. CONCLUSION: This case supports a possible filiation between hepatocellular carcinoma and hepatoblastoma in adults.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/53. A case of well-differentiated, fetal-type hepatoblastoma with very low serum alpha-fetoprotein.serum alpha-fetoprotein (AFP) is elevated in 96.6% of infants and children with hepatoblastoma and hepatocarcinoma. serum AFP was of normal level in only one case of immature-type hepatoblastoma, and very low in only one case of fetal-type hepatoblastoma among 43 cases reviewed in 1986. Recently, a case of hepatoblastoma with very low serum AFP was encountered in the authors' surgical department. In October 1998, a 55-month-old girl was admitted. Her birth weight was 717 g. A large space-occupying lesion (SOL) located in the left lobe of the liver was seen by magnetic resonance imaging (MRI), and her serum AFP level was 322 ng/mL with a subfractionation pattern of the "benign liver disease" type based on reactivity with lens culnaris hemagglutinin and concanavalin a. The patient underwent successful left lobectomy, and histological examination showed that the tumor, weighing 268 g, was hepatoblastoma of the well-differentiated, fetal type. immunohistochemistry findings for AFP were negative. A hepatic mass in infants and children with low serum AFP level indicates the presence of either well-differentiated or immature hepatoblastoma, or fibrolamellar hepatocellular carcinoma. Subfractionation of AFP may contribute to the differential diagnosis of these subtypes.- - - - - - - - - - ranking = 0.25keywords = liver (Clic here for more details about this article) |
3/53. renin-producing hepatoblastoma.renin-producing tumors of extrarenal origin are rare in children. An 8-year-old boy with hepatoblastoma and hypertension associated with a high plasma renin level is reported. After chemotherapy, the plasma renin level normalized and the hypertension spontaneously resolved. The patient underwent surgery, and a right trisegmentectomy of the liver and a partial resection of the second and third segments were performed. The tumor was as shown the source of renin by immunohistochemical study and reverse transcriptase-polymerase chain reaction.- - - - - - - - - - ranking = 0.125keywords = liver (Clic here for more details about this article) |
4/53. A new technique for resecting 'unresectable' liver tumors.BACKGROUND/PURPOSE: Liver tumors that surround the three major hepatic veins traditionally have been considered unresectable. This report describes an extended atypical left hepatectomy technique for tumors around the major hepatic veins. methods: Three children with tumors surrounding the 3 hepatic veins underwent intraoperative evaluation for extended atypical left hepatectomy. The left hepatic artery, left branch of the portal vein, and the 3 hepatic veins are occluded with vascular clamps. perfusion of the remaining liver is through the right hepatic artery and portal vein into the retrohepatic vena cava via the retro hepatic veins. If the liver remains soft and does not become mottled, division of the 3 hepatic veins and resection of the tumor are carried out. RESULTS: Extended atypical left hepatectomy was successful in 2 children. bile leak occurred in 1 instance and healed spontaneously. Both patients had transiently elevated serum bilirubin and transaminase levels and an elevated prothrombin time for 2 weeks. Both survived after treatment with chemotherapy. In the third child the liver became tense and mottled, and the procedure was abandoned. CONCLUSIONS: Successful extended atypical left hepatectomy depends on the ability of the retro hepatic veins to adequately drain blood into the vena cava after interruption (clamping) of the main hepatic veins. If the liver becomes mottled and tense the procedure must be abandoned and the patient should be considered for hepatic transplantation.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
5/53. Antenatal diagnosis of congenital hepatoblastoma in utero.A fetus with a huge hepatic tumor was detected by sonography at 36 weeks of gestation. The mass appeared as a single, solid and polylobular tumor located in the right lobe of the liver. Foci of hemorrhage, necrosis and some tiny calcifications were seen. The adjacent right kidney appeared normal but was displaced. The right adrenal gland was not visualized. Three-dimensional power Doppler sonography further depicted the corresponding vascular anatomy of the tumor, including its vascularization pattern and blood supply. The tumor was situated to the right of the umbilical vein and portal sinus, possibly deriving its blood supply from the portal circulation. The fundamental findings suggested the diagnosis of hepatoblastoma by exclusion of other possibilities. The baby was delivered by cesarean section at 36 weeks' gestation, due to signs of fetal distress. Unfortunately, hypotension, tachycardia, and tachypnea developed shortly after birth. Surgical intervention was performed, but intractable bleeding occurred intra-operatively. The infant died at 6 days of age. autopsy confirmed the diagnosis of hepatoblastoma. We believe this is the first reported case of the antenatal diagnosis of congenital hepatoblastoma.- - - - - - - - - - ranking = 0.25keywords = liver (Clic here for more details about this article) |
6/53. liver transplantation for biliary atresia associated with malignant hepatic tumors.The authors report 3 cases of liver transplantations in children between 4 and 10 years of age, complicated with malignant hepatic tumors after biliary atresia. The preoperative abdominal computed tomography (CT) scans of all 3 cases showed hepatic masses. The serum alpha-fetoprotein levels were elevated highly in 2 cases. After living-related liver transplantation (LRLT), the pathologic findings of the masses in the resected livers showed hepatocellular carcinoma in 2 cases and hepatoblastoma in the other. All cases were associated with biliary cirrhosis. The stage of the liver tumor in the 3 cases using the TNM system was IVA (T4, N0, M0), II (T2, N0, M0) and IVA (T4, N0, M0). Chemotherapy was used in all cases after liver transplantation, and all patients survived with no recurrence. The results suggest that even though malignant liver tumors rarely are complicated with biliary atresia in childhood, one should be alert to the occurrence of hepatic malignancy and perform routine screening of alpha-fetoprotein levels, abdominal CT scans, and magnetic resonance imagings.- - - - - - - - - - ranking = 0.75keywords = liver (Clic here for more details about this article) |
7/53. Successfully resected hepatoblastoma in a young adult with chronic hepatitis b: report of a case.hepatoblastoma usually occurs in children, but a few cases have also been reported in adults. We report the unusual case of hepatoblastoma in an 18-year-old adult with chronic hepatitis b. He visited a local hospital with right upper abdominal pain. Abdominal ultrasound showed a large mass in the right lobe of his liver. He was referred to our hospital and admitted for further examination. At admission, liver function tests gave slightly elevated results (aspartate aminotransferase (AST) 103 IU/l, alanine aminotransferase (ALT) 63 IU/l). A test for hepatitis virus revealed that he was a hepatitis b surface antigen (HBsAg) carrier and had experienced seroconversion. His alpha-fetoprotein (AFP) was elevated to 1 548 000 IU/ml. Abdominal ultrasound showed a 109 x 96 x 80-mm mass with mosaic pattern in the right lobe of the liver and right portal vein thrombus. Abdominal computed tomography (CT) demonstrated a large low-density mass occupying the right lobe, with some high-density parts that showed calcification. From these results, we diagnosed hepatoblastoma in a young adult. A right lobectomy was performed. Pathological examination showed a highly differentiated hepatoblastoma. Adjuvant chemotherapy was performed with cisplatin and pirarubicin. The patient has been well and free of recurrence for 12 months, and his AFP level remains almost normal.- - - - - - - - - - ranking = 0.375keywords = liver (Clic here for more details about this article) |
8/53. Teratoid hepatoblastoma: multidirectional differentiation of stem cell of the liver.hepatoblastoma is the most common malignant hepatic neoplasm of childhood, showing a wide spectrum of epithelial and mesenchymal components. Teratoid hepatoblastoma, which reveals multiple lines of tissue differentiation such as mucinous epithelium, melanin pigment, endocrine differentiation, glial and mesenchymal components, has rarely been observed. We report a case of teratoid hepatoblastoma in a 22-month-old girl. She had been diagnosed with hepatoblastoma through percutaneous needle biopsy of the liver and treated with 10 chemotherapy cycles of epirubicin, VP-16 and cisplatin and with hepatic artery embolization. After 10 months, an extended left lobectomy was performed. Grossly, a multinodular, partly well-demarcated, solid mass (7 x 5 cm) with dense fibrosis and focal cystic change occupied almost the entire specimen. There was extensive necrosis due to preoperative treatment. Microscopically, the tumor showed multiple lines of differentiation, which was composed of embryonal, fetal hepatocytes and mesenchymal elements with numerous foci of osteoid. There were also other components showing endodermal, neural, melanocytic and endocrine differentiation. These teratoid components were considered relatively resistant to preoperative chemotherapy, in contrast to extensive necrosis of both embryonal and fetal hepatocytes. These teratoid features of hepatoblastoma are considered to be a multidirectional differentiation of the small epithelial cells or stem cells of the tumor.- - - - - - - - - - ranking = 0.625keywords = liver (Clic here for more details about this article) |
9/53. An 8-cM interstitial deletion on 4q21-q22 in dna from an infant with hepatoblastoma overlaps with a commonly deleted region in adult liver cancers.We performed molecular analysis of a germline interstitial deletion of chromosome 4 [del(4)(q21.22q23)], which had been observed in a male infant manifesting early-onset hepatoblastoma (HBL). The chromosomal anomaly in this child was associated with a unique congenital syndrome including HBL, atrial septal defect, ventricular septal defect, patent ductus arteriosus, mental retardation, and seizures. However, the patient did not exhibit a megalencephaly typical of 4q21-22 deletions. His HBL was associated with an increasing serum alpha-fetoprotein level and rapid growth. To define the chromosomal deletion at the molecular level in this child, we analyzed his lymphoblasts with fluorescence in situ hybridization, using as probes a panel of BAC/PAC genomic clones containing STS markers covering the 4q12-27 region. The analysis revealed that the affected chromosome had an 8-cM deletion within 4q21-q22, flanked by markers D4S2964 and D4S2966. This microdeletion overlaps with the commonly deleted region at 4q21-q22 that was recently defined in adult hepatocellular carcinomas.- - - - - - - - - - ranking = 0.5keywords = liver (Clic here for more details about this article) |
10/53. Treatment of alpha-fetoprotein secreting hepatoblastoma by response of serum alpha-fetoprotein levels: a new concept.hepatoblastoma, the commonest primary malignant liver tumor in infants and children, is usually associated with elevated serum alpha-fetoprotein (AFP) levels. The authors sought to determine if AFP levels can be used to modify treatment, thereby avoiding the wait for formal imaging studies and prolonged suboptimal treatment and limiting the use of effective but toxic chemotherapy. From April 1984 to December 1997, 8 children were diagnosed with AFP-secreting hepatoblastoma. serum AFP levels were measured weekly. If AFP levels failed to improve, or increased on at least 2 successive examinations, the chemotherapy protocol was changed. When an excellent response was achieved, less toxic chemotherapy was substituted. Six patients (75%) were disease-free for at least 2 years, some with high-risk or metastatic disease. Two patients died. Six of the 7 nonmetastatic patients (86%) remain disease-free (only one had a resectable tumor). Chemotherapy changes resulted in reduced AFP levels in 7 patients. This study supports the use of AFP monitoring to modify treatment in hepatoblastoma responding to therapy with less toxic drugs and the use of nonstandard therapy when suboptimal responses are obtained.- - - - - - - - - - ranking = 0.125keywords = liver (Clic here for more details about this article) |
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