1/17. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/17. hepatoblastoma with spinal metastases.spinal cord compression secondary to metastases is an infrequent complication of childhood cancer. We describe an infant with hepatoblastoma in whom cord compression developed because of extensive epidural metastases during treatment. This is a hitherto undescribed metastatic site for hepatoblastoma.- - - - - - - - - - ranking = 0.063883189103907keywords = cancer (Clic here for more details about this article) |
3/17. 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 = 1keywords = neoplasm (Clic here for more details about this article) |
4/17. 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 = 2.0036586767172keywords = germ, cancer (Clic here for more details about this article) |
5/17. A case of myoepithelial carcinoma displaying biallelic inactivation of the tumour suppressor gene APC in a patient with familial adenomatous polyposis.Familial adenomatous polyposis (FAP) is an autosomal dominant disorder caused by mutation of the APC gene. It is characterised by the appearance of hundreds to thousands of colorectal adenomas in adolescence and the subsequent development of colorectal cancer. Various extracolonic malignancies are associated with FAP, including desmoids and neoplasms of the stomach, duodenum, pancreas, liver, and brain. We present a family affected by FAP with an exon 14 APC mutation displaying two rare extracolonic lesions, a hepatoblastoma and a myoepithelial carcinoma. The hepatoblastoma was found in a male patient aged 2 years. The second lesion, a myoepithelial carcinoma of the right cheek, was found in a female patient aged 14 years. Inactivation of the normal APC allele was demonstrated in this lesion by loss of heterozygosity analysis, thus implicating APC in the initiation or progression of this neoplasm. This is the first reported case of this lesion in a family affected by FAP.- - - - - - - - - - ranking = 2.0638831891039keywords = neoplasm, cancer (Clic here for more details about this article) |
6/17. Cytogenetic findings in two new cases of hepatoblastoma.We describe two cases of hepatoblastoma occurring in an 18-month-old boy and a 3-month-old girl. cytogenetic analysis of the primary tumors revealed gain of 2q and 20 in both cases. In case 1, t(7;8;11) was seen as a secondary abnormality. Other chromosomal aberrations seen in case 2 were unbalanced t(1;1) and t(2;11), resulting in partial gains of 1q and 2q. These results support previous reports that gains of 2q and 20 and rearrangement of chromosome 1 are strongly associated with hepatoblastoma and may be essential for establishing this neoplasm. The 11q and 7q abnormalities may represent a pathway of genetic evolution associated with hepatoblastoma progression.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/17. Acute lymphoblastic leukemia and hepatoblastoma in a family.Acute lymphoblastic leukemia is the most common and hepatoblastoma is a rare malignancy diagnosed in children. Their report on an Iranian boy with acute lymphoblastic leukemia diagnosed at the age of 2 years; 20 months later his 10-month-old sister was referred to their hospital with hepatoblastoma. The occurrence of such two types of cancer is rare in a family.- - - - - - - - - - ranking = 0.063883189103907keywords = cancer (Clic here for more details about this article) |
8/17. hepatoblastoma associated with beckwith-wiedemann syndrome and hemihypertrophy.Both beckwith-wiedemann syndrome (BWS) and hemihypertrophy (HH) have been recognized to be overgrowth syndromes associated with an increased risk of cancer. We report an infant with hepatoblastoma associated with both BWS and HH in whom high serum alpha-fetoprotein (AFP) levels persisted even after complete tumor resection with no tumor recurrence. This phenomenon might be partly due to the nature of the proliferative disease. It is important to recognize that in some infants with BWS prolonged high serum AFP levels mimic the existence of a tumor, and that treatment should be based not only on AFP measurement, but also on repeated radiologic imaging.- - - - - - - - - - ranking = 0.063883189103907keywords = cancer (Clic here for more details about this article) |
9/17. hepatoblastoma in a 15-month-old male: cytomorphology, electron microscopy, and differential diagnosis.The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small. uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/17. Mixed hepatoblastoma in an adult.We report a case in an elderly adult of a highly malignant liver tumor with blastoid features that resembled hepatoblastoma. A liver tumor with a diameter of 23 cm was removed in a 78-year-old woman. The tumor showed highly differentiated epithelial hepatocellular and poorly differentiated epithelial and mesenchymal components. The blastoid nature and pluripotent differentiation potential were supported by immunohistologic analysis and suggest an origin of a poorly differentiated pluripotent hepatic cell with the potential to mature. We believe that this case of a mixed hepatoblastoma in an adult should be added to the growing number of presumed hepatic precursor cell neoplasms in adults.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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