This study evaluates current trends in incidence, clinical outcomes and factors predictive of survival in children with hepatoblastoma (HB).
The Surveillance, Epidemiology and End Results (SEER) database was queried for the period 1973-2009 for all patients aged <20 years with HB.
A total of 606 patients were identified. The age-adjusted incidence was 0.13 patients per 100 000 in 2009. An annual percentage change of 2.18% (95% confidence interval (CI) 1.10-3.27; P < 0.05) was seen over the study period. Overall survival rates at 5, 10 and 20 years were 63%, 61% and 59%, respectively. Ten-year survival rates significantly improved in patients with resectable disease who underwent operative treatment in comparison with those with non-resectable HB (86% versus 39%; P < 0.0001). Multivariate analysis showed surgical treatment (hazard ratio (HR) = 0.23, 95% CI 0.17-0.31; P < 0.0001), Hispanic ethnicity (HR = 0.61, 95% CI 0.43-0.89; P = 0.01), local disease at presentation (HR = 0.43, 95% CI 0.29-0.63; P < 0.0001) and age < 5 years (HR = 0.63, 95% CI 0.41-0.95; P < 0.03) to be independent prognostic factors of survival.
The incidence of paediatric HB has increased over time. Hepatoblastoma is almost exclusively seen in children aged < 5 years. When HB presents after the age of 5 years, the prognosis is most unfavourable. Tumour extirpation markedly improves survival in paediatric patients with local disease.
"Hepatoblastoma, the most common pediatric hepatic malignancy, accounts for about 50% of malignant liver tumors in children . More than half of the children presenting with hepatoblastoma have advanced or metastatic disease at the time of diagnosis , and survival remains poor for those with unresectable or recurrent disease . Malignant rhabdoid kidney tumors (MRKT) are another type of aggressive pediatric solid tumor. "
[Show abstract][Hide abstract] ABSTRACT: Recently, investigators showed that mice with syngeneic murine gliomas that were treated with a neuroattenuated oncolytic herpes simplex virus-1 (oHSV), M002, had a significant increase in survival. M002 has deletions in both copies of the γ134.5 gene, enabling replication in tumor cells but precluding infection of normal cells. Previous studies have shown antitumor effects of other oHSV against a number of adult tumors including hepatocellular carcinoma and renal cell carcinoma. The purpose of the current study was to investigate the oncolytic potential of M002 against difficult to treat pediatric liver and kidney tumors. We showed that the oHSV, M002, infected, replicated, and decreased cell survival in hepatoblastoma, malignant rhabdoid kidney tumor, and renal sarcoma cell lines. In addition, we showed that in murine xenografts, treatment with M002 significantly increased survival and decreased tumor growth. Finally, these studies showed that the primary entry protein for oHSV, CD111 (nectin-1) was present in human hepatoblastoma and malignant rhabdoid kidney tumor specimens. We concluded that M002 effectively targeted these rare aggressive tumor types and that M002 may have potential for use in children with unresponsive or relapsed pediatric solid tumors.
PLoS ONE 01/2014; 9(1):e86843. DOI:10.1371/journal.pone.0086843 · 3.23 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Hepatoblastoma is the most common hepatic tumor in paediatric population with an increasing incidence. It occurs almost in patients younger than 3 years without history of liver disease. Even if the aetiology is unknown, it can be associated with a congenital abnormality or an APC mutation. Hepatoblastoma appears as a well-circumscribed liver mass with, in most cases, an abnormal elevation of alfa-foetoprotein level (AFP). The imaging characteristics of the tumor reflect its gross pathologic appearance and histological composition. A biopsy is mandatory to confirm the diagnosis and study biological markers of the tissue. The radiological characteristics and the histology have permitted to build a new classification and a stratification of the treatment. Cisplatinum associated with surgical resection is the mainstay of treatment. Tumors considered unresectable may be treated with liver donor transplantation. The intensification of the chemotherapy and the radical surgery have increased the complete resection rate and the overall survival (80%) even in the high risk stage.
[Show abstract][Hide abstract] ABSTRACT: Hepatoblastoma (HB) is the most common primary liver cancer in childhood. The fetal and mixed embryonal/fetal epithelial subtypes of HB differ not only in grade of differentiation but probably also in prognosis. We aimed to determine microRNA (miRNA) expression patterns of the main subtypes of epithelial HBs to reveal differences and relate them to survival. We studied 20 cases of epithelial HB, subtyped as pure fetal (n = 12) or embryonal/fetal (n = 8). Tissues were sampled according to subtype to arrive at 15 purely fetal and eight purely embryonal samples (n = 8) and 15 samples of non-tumorous surrounding liver (SL). Relative expression of miR-17-5p, miR-18a, miR-21, miR-34a, miR-96, miR-122, miR-181a, miR-195, miR-210, miR-214, miR-221, miR-222, miR-223, and miR-224 was determined by TaqMan MicroRNA Assays applying miR-140 as reference. A higher level of miR-18a (p < 0.01) was found in embryonal samples than in fetal samples. Lower miR-17-5p, miR-195, miR-210, miR-214, and higher miR-221 levels were detected in fetal samples (p < 0.02) in comparison with SL samples, whereas a lower miR-122 level was observed in embryonal samples (p < 0.003). Histological subtype did not correlate with survival; however, high miR-21, low miR-222, and low miR-224 levels proved to be independently prognostic for HB with significantly increased overall survival (p < 0.03). The fetal and embryonal components of epithelial HB, as well as SL, revealed different miRNA expression patterns. Furthermore, miR-21, miR-222, and miR-224 levels predict overall survival of HB patients regardless of epithelial subtype.
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin 02/2014; 464(4). DOI:10.1007/s00428-014-1549-y · 2.65 Impact Factor
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