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Children under 5 years of age are usually affected.

The tumor is rarely present at birth and an upper abdominal mass or an enlarging abdomen is the most common clinical presentation.

The right lobe of the liver is involved in 60-65% of cases.

The neoplasm is usually a single well-circumscribed mass confined to one lobe of the liver.

Hepatic cirrhosis is not associated with the tumor.

Histological sub-types include fetal, embryonal, macrotrabecular and small-cell undifferentiated (anaplastic), as well as epithelial/ mesenchymal (mixed).

Prognostically, complete resection  (stage 1) seems to offer the best chance of survival.

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The emerging family of hepatoblastoma tumours: from ontogenesis to oncogenesis.Eur J Cancer. 2005 Jul;41(11):1503-14.

The identification of distinct types and subtypes of hepatoblastoma has led to a successful classification of these lesions. In recent years, and particularly within large tumour trials, the spectrum of paediatric epithelial liver tumours has increased. This, together with the need for defining clinically relevant risk groups, will require a new approach to defining and classifying these cancers. Furthermore, an impressive amount of molecular biological information on liver ontogenesis and growth regulation of hepatic tumours has recently accumulated, which will allow the development of a comprehensive classification system with particular emphasis on prognostics. In this review, novel findings relating to these issues are discussed.

Hepatoblastoma: assessment of criteria for histologic classification.Med Pediatr Oncol. 2002 Nov;39(5):478-83.

BACKGROUND: Comparison of outcomes in different clinicopathologic studies of hepatoblastoma requires reproducible histologic classification. This review examines the diagnostic criteria employed by different pathologists for the classification of subtypes of hepatoblastoma and identifies specific problem areas. PROCEDURE: A selected review of published literature is provided. RESULTS: Published studies demonstrate that uniform criteria have not been applied in the classification of hepatoblastoma. These discrepancies hinder attempts to compare outcome data from different studies. Sampling error and potential treatment effects further complicate analysis of the published literature on the relationship between morphologic classification and outcome. CONCLUSIONS: Standardized criteria are essential to allow reproducible histologic classification of hepatoblastoma. There is significant variation in diagnostic criteria used to define the major subtypes of hepatoblastoma in published studies. Additional potential problems are identified in sampling methods and treatment effects.

Clinicopathological and immunohistochemical study of hepatoblastoma.Hua Xi Yi Ke Da Xue Xue Bao. 1998 Sep;29(3):298-301.

To investigate the relationship between immunoreaction of histologic subtype and prognosis, this paper analysed the clinicopathological data from 20 cases of hepatoblastoma. Immunohistochemical staining was performed in 18 cases. The results showed that cytopolasmic postivities of epithlial tumor cells were observed by CK, AFP, S-100 protein and vimentin in 14, 10, 9 and 4 cases respectively. Positive staining for CEA was seen in the nuclei of epithelial tumor cells in 11 cases. Nuclear P53 protein staining was found in 9 cases. Nuclear and cytoplasmic postivities of P16 protein were observed in 7 cases. S-100 protein, vimentin, CK and P16 protein were detected in mesenchymal component in 1 case. This study suggested that immunoreactions of hepatoblastomas were different in histologic subtypes. The expression may correlate with the neoplastic differentiation and prognosis.

Neuroendocrine differentiation in hepatoblastoma. An immunohistochemical investigation.Am J Surg Pathol. 1990;14(9):847-55.

Hepatoblastoma exhibits a wide range of epithelial and mesenchymal lines of differentiation. Neuroendocrine differentiation in this tumor has not previously been reported. We investigated seven hepatoblastomas of different subtypes (five pure epithelial hepatoblastomas, including one small-cell hepatoblastoma, and two mixed hepatoblastomas) using a broad panel of antibodies against epithelial, mesenchymal, neural, and neuroendocrine markers, alpha-1-antitrypsin (alpha 1-AT), alpha-1-antichymotrypsin (alpha 1-ACT), alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), serotonin, and 14 regulatory peptides. Chromogranin A-immunoreactive neuroendocrine tumor cells, some of which also exhibited immunoreactivity for serotonin and somatostatin, were found in the fetal and embryonal parts of the mixed hepatoblastomas. The osteoid-like material in the mixed hepatoblastomas contained cells with immunoreactivity for chromogranin A, neuron-specific enolase, keratin, and alpha 1-AT, alpha 1-ACT, AFP, and CEA, in addition to S-100 protein and vimentin. Parallels to the neuroendocrine differentiation in hepatoblastomas are found in tumors of the gastrointestinal tract and bronchopulmonary tree. These tumors may also exhibit a neuroendocrine component; that is, multidirectional differentiation may occur, as in hepatoblastoma. The immunoreactivity of some of the cells of the osteoid-like material for keratin, alpha 1-AT, alpha 1-ACT, AFP, CEA, and chromogranin A suggests that these cells--and probably the surrounding material--are of epithelial origin.

Hepatoblastoma: an immunohistochemical and ultrastructural study.Hum Pathol. 1987 Oct;18(10):1025-35.

The ultrastructural and immunohistochemical features of 19 hepatoblastomas were examined to evaluate the phenotypic expressivity of this solid embryonic neoplasm of childhood. Electron microscopy confirmed the embryonal and fetal characteristics of the neoplastic hepatocytes, but in addition, cells with features intermediate between these two cell types were identified. Dense bundles of collagen corresponding to the osteoid-like material by light microscopy surrounded nests of cells; the cells within this matrix stained for epithelial membrane antigen and vimentin and focally for cytokeratin, and they showed ultrastructural features of epithelial cells. The two cases of small cell hepatoblastoma reacted positively for vimentin and cytokeratin; the remaining 17 cases were immunoreactive for cytokeratin and alpha-fetoprotein, and some also for alpha 1-antitrypsin, ferritin, and vimentin. A histogenetic scheme based on our findings is proposed to explain the divergent morphologic features of this neoplasm.

Hepatoblastoma. Attempt at characterization of histologic subtypes.
Am J Surg Pathol. 1982 Oct;6(7):599-612.

This is a clinicopathologic study summarizing the experience with hepatoblastomas at Children's Memorial Hospital of Chicago between 1954 and 1981. Of 21 patients studied, 13 (61.9%) died. Three major histologic epithelial patterns were identified: fetal, embryonal, and macrotrabecular. The first two may represent different stages of cytodifferentiation of hepatoblastoma cells. The macrotrabecular type had features similar to hepatocellular carcinoma of adults, from which distinction was difficult; this type pursued an aggressive clinical course. The fetal type exhibited advanced differentiation, and two cases in this category survived after surgery only; local extrahepatic dissemination was present in other cases of the fetal type. Mixed epithelial and mesenchymal tumors constituted only 23% of this series, and none contained rhabdomyoblastic elements. Although modern chemotherapy may alter the course of this disease, the small size of this series precluded definite statements in this regard. Only patients in whom the tumor was completely excised as primary treatment became long-term survivors.

Protocol for the examination of specimens from pediatric patients with hepatoblastoma.Arch Pathol Lab Med. 2007 Apr;131(4):520-9.

Predictive value of staging systems in hepatoblastoma.J Clin Oncol. 2007 Feb 20;25(6):737author reply 737-8.

Cytologic diagnosis of small cell anaplastic hepatoblastoma: a case report.Acta Cytol. 2006 Mar-Apr;50(2):205-7.

Claudin-1 and claudin-2 differentiate fetal and embryonal components in human hepatoblastoma.Hum Pathol. 2006 May;37(5):555-61.

Predictive value of the pretreatment extent of disease system in hepatoblastoma: results from the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL-1 study. J Clin Oncol. 2005 Feb 20;23(6):1245-52.

Fine needle aspiration cytology of hepatoblastoma. Recognition of subtypes on cytomorphology.Acta Cytol. 2005 Jul-Aug;49(4):355-64.

Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases.J Pediatr Surg. 2004 Sep;39(9):1321-7.

Hepatoblastoma in a 15-month-old male: cytomorphology, electron microscopy, and differential diagnosis.Ultrastruct Pathol. 2003 Sep-Oct;27(5):369-73.

Hepatoblastoma: cytomorphologic characteristics in serious cavity fluids.Cancer. 2002 Oct 25;96(5):267-74.

Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group.Cancer. 2002 Feb 15;94(4):1111-20

Hepatic tumors in children.Clin Liver Dis. 2001 Feb;5(1):259-81.

Pediatric liver neoplasms: a radiologic-pathologic correlation.Eur Radiol. 1999;9(7):1339-47.

Immunohistochemical evaluation of hepatoblastomas with use of the hepatocyte-specific marker, hepatocyte paraffin 1, and the polyclonal anti-carcinoembryonic antigen.Mod Pathol. 1998 Oct;11(10):934-8.

An approach to handling pediatric liver tumors.Am J Clin Pathol. 1998 Apr;109(4 Suppl 1):S67-72.

Hepatoblastoma. Report of a case with cytologic, histologic and ultrastructural findings. Acta Cytol. 1994 May-Jun;38(3):455-8.

 
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