| 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. ( info) |
2/112. Thrombotic microangiopathy associated with reactivation of human herpesvirus-6 following high-dose chemotherapy with autologous bone marrow transplantation in young children. Thrombotic microangiopathy (TMA) is a serious complication of BMT. Several factors are important in the etiology of TMA, such as cyclosporin A, GVHD, irradiation, intensive conditioning chemotherapy and infection, which cause damage to vascular endothelial cells leading to activation of these cells. We describe two young children with TMA following high-dose chemotherapy with autologous BMT. Development of TMA was accompanied by reactivation of HHV-6, which was identified by both an increase in the copy number of HHV-6 dna in the peripheral blood and a significant increase in antibody titers to HHV-6. Thus, it was suggested that reactivation of HHV-6 together with high-dose chemotherapy played an important role in the pathogenesis of TMA in these patients. Since HHV-6 is known to infect vascular endothelial cells, and CMV which is virologically closely related to HHV-6, has been reported to be a pathogen that causes TMA, infection with HHV-6 of vascular endothelial cells may induce TMA via damage and activation of these cells. ( info) |
3/112. Karyotypic analyses of hepatoblastoma. Report of two cases and review of the literature suggesting chromosomal loci responsible for the pathogenesis of this disease. Two cases of fetal hepatoblastoma with unique karyotypic changes are described. One was a 17-month-old boy with multiple unbalanced chromosomal translocations, resulting in four types of derivative chromosomes involving chromosomal loci at 1q21, 1q32, 2q23, 6q27, 7p22, and 21p12, partial tetrasomy of 1q, partial trisomy of 2q, and partial monosomy of 21p. The clonal karyotype of this tumor was 46,XY,der(2)t(1;2)(q32;q37), der(6)t(1;6)(q12;q27), der(7)t(2;7)(q23;p22), der(21)t(2;21) (q23;p12). In the other case, a 4-year-old girl, karyotypic analyses revealed trisomy 2 and 8, and the clonal karyotype of this case was 48,XX, 2, 8. review of these cases together with previous reports suggested the significance of chromosomal changes including numerical abnormalities of 1q, 2(or 2q), 20, and 8 (or 8q), and breakage of 1q and 2q in the development of hepatoblastoma. The results presented herein underscore the significance of numerical abnormalities of chromosomal regions 1q and 2q and of chromosome 8 in the development of hepatoblastoma, in addition to abnormalities of 6q27, 7p22, and 21p12-13 as other chromosomal loci that may be responsible for the pathogenesis of this embryonal type of tumor. ( info) |
4/112. 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. ( info) |
5/112. Successful transumbilical embolization of congenitally ruptured hepatoblastoma. To avoid a high-risk operation on a moribund neonate with a ruptured hepatic tumor, transumbilical embolization of the bleeding tumor was attempted in a 2-day-old neonate. A 3F microferret catheter was advanced through the right umbilical artery. After identifying the left hepatic artery feeding the bleeding tumor, Gelfoam (Upjohn, Kalamazoo, MI) particles were injected. Bleeding was successfully controlled. The infant was able to tolerate enteral feeding when stable. Seventeen days after embolization, elective left hepatic lobectomy was performed. Histological examination showed a fetal epithelial type hepatoblastoma. The baby is 13 months old now and is receiving chemotherapy. ( info) |
| 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. ( info) |
7/112. Cytogenetic characterization of childhood hepatoblastoma. We describe the cytogenetic abnormalities in two cases of childhood hepatoblastoma. The first case was of fetal histology with squamous metaplasia, and cytogenetic study showed an add(5)(q31). Although an association between hepatoblastoma and familial adenomatous polyposis is recognized, the breakpoint in this case is distal to 5q21 and most probably does not involve the APC gene at that location. The second case was of macrotrabecular histology, and cytogenetic study showed an unbalanced translocation in the form of der(4)t(1;4)(q12;q34) in a hyperdiploid clone. Including our case, der(4)t(1;4)(q12;q34) has been recognized in four cases of hepatoblastoma, and it may be the first recurrent translocation in this tumor. Understanding the molecular mechanism and clinical significance of this translocation awaits analysis of more cases. ( info) |
8/112. Sialoblastoma and hepatoblastoma in a neonate. We report a case of salivary gland neoplasm and associated hepatoblastoma. The sialoblastoma was diagnosed by prenatal sonography; however, the hepatoblastoma was imaged post-operatively. Prior knowledge could have prevented a subsequent operation, including the additional risks of repeat anesthesia. We suggest that if a sialoblastoma is in the differential diagnosis, then additional imaging may be indicated because of the possibility of associated lesions. ( info) |
| We present a case of an infant with severe macroglossia, hypoglycaemia and inguinal hernia associated with hepatoblastoma (beckwith-wiedemann syndrome) in which a laryngeal mask airway (LMA) was useful to secure the airway during central line insertion. carbon dioxide monitoring through LMA proved effective to assess airway patency during positioning for central vein puncture. In this syndrome, where a potentially difficult airway may be encountered, LMA allowed adequate ventilation, avoiding the risk and inconvenience of tracheal intubation. ( info) |
10/112. 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. ( info) |