Liver Tumors in Children

Abstract

After completing this course, the reader should be able to:1. Describe the current epidemiologic trends in hepatoblastoma.2. Identify the genetic syndromes that are seen in a subset of liver tumors.3. Assess the need for complete tumor resection in the treatment of liver tumors in children as well as the increasingly important option of liver transplantation for those patients with unresectable tumors.4. Discuss the impact of the hepatitis vaccine in reducing the incidence of hepatocellular carcinoma.5. Explain the prognostic impact of different histologic subtypes of hepatoblastoma.6. Promote the need for future clinical trials in testing new agents for hepatocellular carcinoma in children.7. Employ the different staging systems used in liver tumors, including the traditional North American postsurgical staging system and the European presurgical staging system using imaging.This article is available for continuing medical education credit at CME.TheOncologist.com. CME CME ABSTRACT

Genome-wide analysis of DNA methylation in hepatoblastoma tissues

“…Although genome-wide copy number alterations in HB have been studied, little is known about genome-wide methylation changes (4). The present study is the first to explore whole-genome DNA methylation status in HB.…”

“…This disease is most commonly diagnosed during a child's first 3 years of life (3); only 5% of new HB cases are diagnosed in children older than 4 years (4). The disease occurs significantly more frequently in boys than in girls, for reasons that remain to be determined (4).…”

Adult hepatoblastoma

“…The AHB histology has been compared with the pediatric hepatoblastoma. The epithelial IHB has been broadly subdivided into four histological patterns: the fetal pattern (1/3 of cases), the mixed fetal and embryonic pattern (20 %), the macrotrabecular pattern (3 %) and the small cell undifferentiated type (2-3 %) (2,7,8,10).…”

“…With these treatment regimens and curative resections (R0) (hepatectomy or liver transplant), 75 % five-year survival rate and 66 % free of recurrence survival have been described. In children with good prognostic factors, Cisplatin monotherapy neoadjuvant regimen has been shown as effective as poli-chemotherapy, although with less toxicity (2,7). In spite of the scarce experience in adults, it seems reasonable to apply the staging and therapeutic regimens previously described in children, due to the tumor pattern similarities as well as to exploit new molecular targets.…”

Portal vein thrombosis in a patient with a malignant rhabdoid tumor of the liver (Case report)

“…В отли-чие от взрослых, у которых тромбоз воротной вены выявляется у 8,4 % пациентов с гепатоцеллюлярным раком [10][11][12][13], данные по распространенности тром-бозов воротной вены у детей со ЗНО печени представ-лены единичными работами [14][15][16]. Это может быть обусловлено низкой распространенностью ЗНО пече-ни в педиатрической практике -1 % среди всех солид-ных ЗНО [16].…”