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Hepatoblastoma (34 unread)

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Relay For Life changing format - Chillicothe Gazette

Posted: January 20th, 2012, 3:34am MST

Relay For Life changing format
Chillicothe Gazette
Caroline is a 1 1/2 year survivor of hepatoblastoma. For more information about local Relay For Life events, visit www.RelayForLife.org/RossCounty or call Alisha Thomas at (740) 708-0573. / Heather Cory/Gazette CHILLICOTHE -- The 2012 Ross County Relay ...

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Kim Kardashian Humbled at Children's Hospital - Gather Celebs News Channel

Posted: January 18th, 2012, 9:05am MST

Kim Kardashian Humbled at Children's Hospital
Gather Celebs News Channel
Kim Kardashian threw on a pair of ripped jeans and headed to the Children's Hospital in Los Angeles where she met a little girl plagued by hepatoblastoma. The reality star spend some time with Violet Rose and seemed to be completely humbled by the ...

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Newark 22-month-old undergoing cancer treatment - The Newark Advocate

Posted: January 13th, 2012, 2:58am MST

Newark 22-month-old undergoing cancer treatment
The Newark Advocate
Alison recently was diagnosed with hepatoblastoma, a type of liver cancer. / Photos by Zach Gray/ The Advocate Brittney Armstrong wishes she could forget Christmas -- or really the whole month of December. At the beginning of that month, her youngest ...

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Spaghetti dinner to support young cancer patient - WNYT

Posted: November 15th, 2011, 9:51am MST

Spaghetti dinner to support young cancer patient
WNYT
The dinner will go from 6 to 8:30 pm and will support three-year-old Lilliana who has been diagnosed with Hepatoblastoma Cancer. There will be a DJ, 50/50 raffle, raffle baskets, gift certificates and gifts donated by local businesses. ...

PubMed - Hepatoblastoma

Paediatric malignant liver tumours.

Fetched: January 25th, 2012, 10:01pm MST

Paediatric malignant liver tumours.

Bull Cancer. 2012 Jan 19;

Authors: Brugières L, Branchereau S, Laithier V

Abstract
Tumours and pseudotumours of the liver are a heterogeneous group of neoplasm including 60% of malignant tumours. Malignant liver tumours account for less than 2% of the lesions in children and vary considerably in incidence throughout the paediatric age range, with hepatoblastoma, rhabdoid tumour of the liver, hemangioendothelioma, biliary tract rhabdomysosarcoma and mesenchymal hamartoma in the first two years of life and hepatocellular carcinoma, focal nodular hyperplasia, and undifferentiated sarcoma in older children and adolescents. Treatment of malignant epithelial tumours is based on the surgical resection of the tumour associated with pre- and postoperative chemotherapy including cisplatinum. Modalities of the treatment are adapted to risk factors. Survival rates at three years are over 80% for localised hepatoblastoma whereas they are less than 30% in hepatocellular carcinomas. The role of targeted therapies still has to be defined.

PMID: 22266074 [PubMed - as supplied by publisher]
Hepatoblastoma: recent developments in research and treatment.

Fetched: January 20th, 2012, 10:00pm MST

Hepatoblastoma: recent developments in research and treatment.

Semin Pediatr Surg. 2012 Feb;21(1):21-30

Authors: von Schweinitz D

Abstract
Hepatoblastoma is the most common liver tumor of early childhood. According to recent studies its incidence seems to be increasing in North America and Europe. Since new histological variants have been described recently the formerly clear-cut distinction of hepatoblastoma and hepatocellular carcinoma may not be valid anymore and a new histological classification will be inaugurated by an international working group. Recent research identified prognostically relevant gene signatures as well as potential molecular targets for therapy of hepatoblastoma. The multicentric study groups in the USA, Europe and Japan recommend cisplatin based chemotherapy for neoadjuvant and adjuvant treatment. However, their risk stratification systems and general treatment strategies differ substantially. Therefore the four groups agreed to pool their patients' data for an analysis of prognostic criteria which can be used for defining common risk groups. While 90% of standard risk and 65% of high risk hepatoblastomas can be cured, the still dismal outcome of multifocal disseminated and metastasising tumors warrants the investigation of new cytotoxic drugs and substances against specific molecular targets.

PMID: 22248967 [PubMed - in process]
Prognostic stratification for children with hepatoblastoma: The SIOPEL experience.

Fetched: January 20th, 2012, 10:00pm MST

Prognostic stratification for children with hepatoblastoma: The SIOPEL experience.

Eur J Cancer. 2012 Jan 13;

Authors: Maibach R, Roebuck D, Brugieres L, Capra M, Brock P, Dall'igna P, Otte JB, De Camargo B, Zsiros J, Zimmermann A, Aronson D, Childs M, Scopinaro M, Morland B, Plaschkes J, Czauderna P, Perilongo G

Abstract
PURPOSE: To identify factors relevant to long-term outcome in newly diagnosed hepatoblastoma, and define subgroups for clinical research on tailoring treatment to the individual patient. PATIENTS AND METHODS: Between 1995 and 2006 the SIOPEL group conducted two clinical trials which established risk-adapted therapy for hepatoblastoma patients. Patients were stratified into high-risk (AFP<100ng/mL and/or PRETEXT IV and/or vascular invasion and/or extra-hepatic intra-abdominal disease (V+/P+/E+) and/or metastases) and standard-risk (all others). The hierarchy of these factors plus multifocality, PRETEXT III, AFP>1,200,000ng/mL, patient age, platelet count and histology were further explored. The outcome measure was event-free survival (EFS). RESULTS: In 541 patients, reduced EFS correlated significantly with AFP<100ng/ml (hazard ratio [HR] 4.09, 95% confidence interval 2.16-7.75), AFP⩾1.2×10(6)ng/mL (2.48, 1.47-4.17), metastatic disease (3.02, 2.05-4.44), PRETEXT IV (2.15, 1.19-3.87), multifocality (1.59, 1.01-2.50), age>5years (2.76, 1.68-4.53); borderline with small cell undifferentiated (SCU) histology (2.29, 95% confidence interval 0.91-5.77); but not with PRETEXT III, age 30-60months, platelet count or V+/P+/E+. By using the significant factors and SCU to stratify the population, we have identified three distinct prognostic groups: PRETEXT I/II/III, and no other factors, have 3year EFS of 90%, PRETEXT IV and/or multifocal tumour and/or age>5years and/or AFP>1.2×10(6) have 3year EFS of 71% and SCU and/or AFP<100ng/mL and/or metastatic have a 3year EFS of 49%. CONCLUSION: Prognostic stratification for clinical research on newly diagnosed hepatoblastoma should take into consideration PRETEXT, metastatic disease, AFP, multifocality, age and SCU histology.

PMID: 22244829 [PubMed - as supplied by publisher]
[Cryopreserved iliac vein for reconstruction of middle hepatic vein in living donor right liver transplantation].

Fetched: January 12th, 2012, 10:01pm MST

[Cryopreserved iliac vein for reconstruction of middle hepatic vein in living donor right liver transplantation].

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2011 Nov;25(11):1393-6

Authors: Guan Z, Ren X, Xu G, Zang Y, Chen X, Shen Z

Abstract
OBJECTIVE: To summarize the experience of living donor liver transplantation using cryopreserved iliac vein for middle hepatic vein reconstruction.
METHODS: Between July 2006 and June 2009, right liver transplantation without middle hepatic vein was performed in 37 cases of 85 patients undergoing living donor liver transplantation; of 37 cases, 30 received middle hepatic vein reconstruction using cryopreserved iliac vein. There were 27 males and 3 females, aged from 10 to 57 years (median, 44 years). Thirty cases included 11 hepatocellular carcinoma, 10 hepatic cirrhosis, 2 Wilson's disease, 1 cholangiocarcinoma, 1 hepatoblastoma, 1 congenital hepatic fibrosis, 1 chronic severe hepatitis, and 1 congenital biliary atresia. Iliac veins harvested from donors were put into 0-4 degrees C mixed antibiotics saline and transported to the operating room. The iliac veins were trimmed, placed into sterile bags (containing RMPI 1640 + 20% DMSO + 10% calf protein solution) and frozen at -70 degrees C. In living donor liver transplantation process, the veins were melt and used for middle hepatic vein reconstruction. After operation, the patency of veins was monitored by regular Doppler ultrasound examination or enhanced CT for 3 months.
RESULTS: In 30 patients, 30 iliac veins were used. The average cryopreserve time was 14 days (range, 3-44 days). Anastomosis were all successful; after cryopreservation, the blood vessels texture and elasticity were fit for surgery. No easily tearing or severe suture bleeding was observed. In 30 patients, 6 had segment V veins reconstruction; 3 had segment VIII; and 21 had both segments V and VIII. The patency rate of reconstructed vessels was 93% at 1 week, 90% at 2 weeks, 90% at 1 month, and 67% at 3 months. No serious complication was observed in donors. The prognosis was good with no small-for-size syndrome.
CONCLUSION: Cryopreserved iliac vein is an ideal material for the right hepatic living donor liver transplantation in the reconstruction of middle hepatic vein.

PMID: 22229202 [PubMed - in process]
A better way forward: targeting hedgehog signaling in liver cancer.

Fetched: January 2nd, 2012, 10:00pm MST

A better way forward: targeting hedgehog signaling in liver cancer.

Front Biosci (Schol Ed). 2012;4:277-86

Authors: Kappler R, von Schweinitz D

Abstract
Accumulated experimental evidence indicates that Hedgehog (Hh) signaling regulates cell proliferation and specification in a variety of organs during embryonic development. However, abnormal activation of this pathway in postnatal tissues has been linked to a large number of human cancers. With respect to the liver, it is known that Hh signaling not only influences bipotential precursor cells capable of pancreas and liver development, but is also implicated in the pathogenesis of liver tumors such as hepatoblastoma, hepatocellular and cholangiocellular carcinoma, if aberrantly activated. Blockade of Hh signaling by several specific inhibitors has been proven to successfully inhibit tumor growth of various Hh-associated cancers in vitro and in preclinical mouse models, and recent clinical data suggest that the implementation of novel anticancer therapeutics based on Hh interference into commonly accepted regimens are within reach. Thus, it is highly probable that Hh targeted therapies could be used for the treatment of Hh-dependent liver cancers in the future.

PMID: 22202060 [PubMed - in process]
Proteasome inhibition overcomes TRAIL resistance in human hepatoblastoma cells.

Fetched: January 2nd, 2012, 10:00pm MST

Proteasome inhibition overcomes TRAIL resistance in human hepatoblastoma cells.

Front Biosci (Elite Ed). 2012;4:2194-202

Authors: Armeanu-Ebinger S, Fuchs J, Wenz J, Seitz G, Ruck P, Warmann SW

Abstract
Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is responsible for cell death in many cancer cells while being non-toxic for most normal cells. In this study, we investigated the role of TRAIL in human hepatoblastoma (HB) cells and analyzed different approaches to reverse TRAIL resistance in these tumors. Death receptors DR4 and DR5 expression was found on all analyzed primary HB samples and on the cell lines HuH6 and HepT1 by immunofluorescence staining. Recombinant TRAIL alone did not induce in vitro cytotoxicity. Decoy receptor blocking by antibodies led to moderate effects in HepT1 but not in HUH6 cells, whereas FLIP knock-down using siRNA rendered HUH6 cells but not HepT1 cells sensible to TRAIL. Bcl-2 inhibition with ABT-737 enhanced TRAIL-mediated apoptosis in all HB cells. Strongest cytotoxic TRAIL effects were seen in HB cell lines with synchronous proteasome inhibition using bortezomib. FLIP and Bcl-2 contributed to the TRAIL resistance in HB. Overcoming TRAIL resistance in HB by proteasome inhibitors has been identified a possible additive to improve treatment results in HB patients with drug resistant tumors.

PMID: 22202031 [PubMed - in process]
Treatment of hepatoblastoma: the North American cooperative group experience.

Fetched: January 2nd, 2012, 10:00pm MST

Treatment of hepatoblastoma: the North American cooperative group experience.

Front Biosci (Elite Ed). 2012;4:1717-23

Authors: Malogolowkin MH, Katzenstein HM, Krailo M, Meyers RL

Abstract
In North America, children with malignant liver tumors have been treated in a cooperative group study since early 1970s. This manuscript describes the results of these studies.

PMID: 22201987 [PubMed - in process]
Liver transplantation in the management of unresectable hepatoblastoma in children.

Fetched: January 2nd, 2012, 10:00pm MST

Liver transplantation in the management of unresectable hepatoblastoma in children.

Front Biosci (Elite Ed). 2012;4:1293-302

Authors: Meyers RL, Tiao GM, Dunn SP, Langham MR

Abstract
Complete surgical resection is essential to long-term survival in children with hepatoblastoma. We present the guidelines from the Children's Oncology Group (COG), liver tumor study group of the Societe Internationale Oncologie Pediatrique (SIOPEL), and German Pediatric Oncology Group (GPOH) for early referral of children with potentially unresectable hepatoblastoma to a specialty center with expertise in extreme resection and liver transplantation. Patients who will become candidates for liver transplantation should receive chemotherapy following the same protocols as for children undergoing a partial hepatectomy. The Pediatric Liver Unresectable Tumor Observatory (PLUTO) is an international prospective database established to collect data and make future recommendations on controversial issues regarding the use of transplant in hepatoblastoma including: 1) What is the optimal treatment of multifocal tumors. 2) What is the role of extreme resection vs. liver transplant in patients with major venous involvement. 3) What is the role of transplant in patients who present with lung metastasis. 3) Should patients with tumor relapse be offered a rescue transplant. 4) What is the role of pre- and post- transplant chemotherapy.

PMID: 22201955 [PubMed - in process]
Cytogenetics of hepatoblastoma.

Fetched: January 2nd, 2012, 10:00pm MST

Cytogenetics of hepatoblastoma.

Front Biosci (Elite Ed). 2012;4:1287-92

Authors: Tomlinson GE

Abstract
The cytogenetics of hepatoblastoma demonstrate recurring events which include whole chromosome trisomies, most commonly trisomy of chromosome 2, 8, or 10. In addition, unbalanced translocations involving a breakpoint on the proximal short arm of chromosome 1 are observed which result in a duplication of the long arm of chromosome 1q. The most commonly involved reciprocal chromosomal arm is 4q, although the reciprocal chromosome is highly variable and always results in a loss of chromosomal material. The full significance of these chromosomal changes has yet to be confirmed in large studies, however a suggestion of an association of duplication of regions of 2q with a poor prognosis. A rare sub-type of hepatoblastoma, known as the small cell undifferentiated variant, is associated with deletion or translocation of 22q, the locus of the rhabdoid tumor gene, SMARCB1.

PMID: 22201954 [PubMed - in process]
Treatment of hepatoblastoma in the German cooperative pediatric liver tumor studies.

Fetched: January 2nd, 2012, 10:00pm MST

Treatment of hepatoblastoma in the German cooperative pediatric liver tumor studies.

Front Biosci (Elite Ed). 2012;4:493-8

Authors: Haeberle B, Schweinitz D

Abstract
Treatment with neoadjuvant and adjuvant chemotherapy together with tumor resection changed treatment strategies in hepatoblastoma and led to prospective cooperative studies. The treatment strategies and results of three German liver tumor studies HB89, HB94 and HB99 are reviewed. Here we provide an overview of the treatment of this tumor in the years 1989 to 2008 in Germany. The treatment protocols, aim of studies and results are outlined. The overall-survival (OS), response to chemotherapy and toxicity are followed over this period of different treatment. The overall-survival improved over the last years with 75percent in HB89, 77percent in HB94 and 89percent in HB99. Patients with potentially resectable tumors have a good prognosis although the treatment was reduced over the last years. Patients with non resectable tumors or lung metastases have also a better but still bad prognosis. The intensified treatment for these patients in Germany in the last years showed comparable results to international studies but no advantage.

PMID: 22201890 [PubMed - in process]
Molecular imaging and photodynamic therapy in hepatoblastoma.

Fetched: January 2nd, 2012, 10:00pm MST

Molecular imaging and photodynamic therapy in hepatoblastoma.

Front Biosci (Elite Ed). 2012;4:487-92

Authors: Seitz G, Fuchs J, Schaefer JF, Warmann SW

Abstract
Molecular imaging is a novel field in cancer research combining various in vivo imaging modalities with molecular biology. Different techniques such as magnetic resonance tomography (MRI), positron emission tomography (PET), optical imaging methods (bioluminescence, fluorescence), or combination of these are used in basic research as well as in patients in different tumor entities. In hepatoblastoma (HB), there are only few reports on molecular imaging methods in a preclinical (optical imaging) and clinical setting (PET, PET-CT). Unimprovable treatment outcomes of patients in advanced tumor stages require novel treatment approaches. Photodynamic diagnosis (PDD) and photodynamic therapy (PDT) are novel diagnostic and therapeutic tools. Photodynamic diagnosis allows in vitro and in vivo detection of tumor cells using their fluorescending behaviour. PDT is a novel anticancer treatment approach leading to tumor cell destruction via apoptosis. In hepatoblastoma, there are only few reports on in vitro and in vivo studies using this treatment modality. First results seem to be promising and further studies will be required to further evaluate these techniques and to transfer them into clinical settings. This paper reviews different modalities of molecular imaging, photodynamic diagnosis and photodynamic therapy in childhood hepatoblastoma.

PMID: 22201889 [PubMed - in process]
Activation of Wnt and Myc signaling in hepatoblastoma.

Fetched: January 2nd, 2012, 10:00pm MST

Activation of Wnt and Myc signaling in hepatoblastoma.

Front Biosci (Elite Ed). 2012;4:480-6

Authors: Cairo S, Armengol C, Buendia MA

Abstract
Hepatoblastoma (HB) is the most common type of pediatric liver cancer. This tumor is thought to derive from hepatic progenitor cells that are arrested at various stages of liver development, as illustrated by a variety of histologic subtypes. Recent genomic studies have led to better understand the molecular pathogenesis of HB, to point out the crucial roles of the Wnt Myc signaling pathways in malignant transformation of liver progenitor cells. Molecular classification of HB based on genome-wide studies, as well as identification of reliable diagnostic prognostic markers, open the way to the development of new personalized targeted therapies for the management of aggressive lethal childhood tumors.

PMID: 22201888 [PubMed - in process]
Hepatoblastoma throughout SIOPEL trials - clinical lessons learnt.

Fetched: January 2nd, 2012, 10:00pm MST

Hepatoblastoma throughout SIOPEL trials - clinical lessons learnt.

Front Biosci (Elite Ed). 2012;4:470-9

Authors: Czauderna P

Abstract
International Childhood Liver Tumors Strategy Group (SIOPEL) introduced the concept of preoperative chemotherapy in hepatoblastoma, most common malignant liver tumor in children. This required introduction of the preoperative tumor staging system called PRETEXT. SIOPEL 1 study proved the value of preoperative chemotherapy consisting of cisplatin and doxorubicin (PLADO) in hepatoblastoma leading to 5-year overall survival of 75percent and event-free-survival of 66percent. Both presence of metastases and PRETEXT were significant prognostic factors which led to development of two risk categories: standard (SR) and high risk (HR) hepatoblastomas. In SIOPEL 2 study two different strategies were developed for SR and HR tumors with corresponding 3-year overall and progression-free survival of 91percent and 89percent, and 53percent and 48percent respectively. In the next SIOPEL 3 SR arm study monotherapy regimen based on CDDP alone was non-inferior to PLADO for SR hepatoblastoma and less toxic. Cisplatin-based chemotherapy in combination with delayed definitive surgery / liver transplantation improved the survival of children with hepatoblastoma. However certain patients, especially those with metastatic disease and low alphafetoprotein still have inferior prognosis.

PMID: 22201887 [PubMed - in process]
Extended hepatic resection in advanced hepatoblastoma.

Fetched: January 2nd, 2012, 10:00pm MST

Extended hepatic resection in advanced hepatoblastoma.

Front Biosci (Elite Ed). 2012;4:462-9

Authors: Fuchs J, Seitz G, Handgretinger R, Warmann SW

Abstract
Complete surgical resection of the primary tumor is one of the most important prognostic factors for hepatoblastoma (HB). This goal can mean a relevant challenge in some cases of advanced HB, especially since an anatomical resection should be realised. Over recent years several surgical techniques for advanced liver resections in children have been developed and refined. In this article the authors summarize their own experience with advanced liver resections for HB and give an overview over indications and specifications for the different approaches.

PMID: 22201886 [PubMed - in process]
Experience of fluoroscopy-aided thoracoscopic resection of pulmonary nodule localised with Lipiodol in a child.

Fetched: January 2nd, 2012, 10:00pm MST

Experience of fluoroscopy-aided thoracoscopic resection of pulmonary nodule localised with Lipiodol in a child.

J Med Imaging Radiat Oncol. 2011 Aug;55(4):401-3

Authors: Yamagami T, Miura H, Yoshimatsu R, Tanaka O, Ono S, Iehara T, Hosoi H, Nishimura T

Abstract
We report the case of a 12-year-old boy with a huge liver tumour 20 cm in diameter with multiple lung metastases. Six months after systemic chemotherapy was initiated, all tumours had disappeared with the exception of the liver tumour and a tiny lung tumour 2.5 mm in diameter. Fluoroscopy-assisted thoracoscopic resection of the pulmonary nodule was performed to evaluate whether viable tumour tissue remained in the lung lesion. Before moving the patient to the operating room, the nodule was marked by Lipiodol under CT fluoroscopic guidance with the patient under local anaesthesia. This procedure allowed correct visualisation of the area that should be resected.

PMID: 21843175 [PubMed - indexed for MEDLINE]
Aqueous extract of Tribulus terrestris Linn induces cell growth arrest and apoptosis by down-regulating NF-κB signaling in liver cancer cells.

Fetched: January 2nd, 2012, 10:00pm MST

Aqueous extract of Tribulus terrestris Linn induces cell growth arrest and apoptosis by down-regulating NF-κB signaling in liver cancer cells.

J Ethnopharmacol. 2011 Jun 14;136(1):197-203

Authors: Kim HJ, Kim JC, Min JS, Kim MJ, Kim JA, Kor MH, Yoo HS, Ahn JK

Abstract
ETHNOPHARMACOLOGICAL RELEVANCE: A medicinal herb Tribulus terrestris Linn has been used to treat various diseases including hepatocellular carcinoma. The aim of the present study was to investigate the anticancer activity of Tribulus terrestris Linn (TT) in liver cancer cells.
MATERIALS AND METHODS: The antitumor activity of aqueous TT extract was analyzed by testing the cytotoxicity and the effect on clonogenecity in HepG2 cells. Apoptosis and cell cycle arrest induced by TT were dissected by flow cytometry and its inhibitory effect on NF-κB activity was determined by analyzing the expression levels of NF-κB/IκB subunit proteins. The suppression of NF-κB-regulated gene expression by TT was assessed by RT-PCR.
RESULTS: TT extract repressed clonogenecity and proliferation, induced apoptosis, and enhanced accumulation in the G0/G1 phase of liver cancer cells. It also turned out that TT extract inhibited NF-κB-dependent reporter gene expression and NF-κB subunit p50 expression, while it enhanced the cellular level of IκBα by inhibiting the phosphorylation and degradation of IκBα. In addition, IKK activity was inhibited in a dose-dependent manner. Furthermore, TT extract suppressed the transcription of genes associated with cell cycle regulation, anti-apoptosis, and invasion.
CONCLUSION: These data showed that TT extract blocks proliferation and induces apoptosis in human liver cancer cells through the inhibition of NF-κB signaling. Aqueous TT extract can be used as an anticancer drug for hepatocellular carcinoma patients.

PMID: 21549825 [PubMed - indexed for MEDLINE]
Tumor necrosis predicts survival following neo-adjuvant chemotherapy for hepatoblastoma.

Fetched: December 24th, 2011, 10:01pm MST

Tumor necrosis predicts survival following neo-adjuvant chemotherapy for hepatoblastoma.

Pediatr Blood Cancer. 2011 Dec 20;

Authors: Venkatramani R, Wang L, Malvar J, Dias D, Sposto R, Malogolowkin MH, Mascarenhas L

Abstract
BACKGROUND: Tumor response to chemotherapy has been shown to predict outcome in children with acute lymphoblastic leukemia, osteosarcoma, and Ewing Sarcoma. We evaluated whether tumor necrosis following neo-adjuvant chemotherapy is prognostic for survival in hepatoblastoma (HB). PROCEDURE: Primary tumors from children with newly diagnosed stage III and IV HB who underwent surgical resection following neo-adjuvant chemotherapy were evaluated histologically for the extent of tumor necrosis (total diameter of necrotic and fibrotic tissue divided by total diameter of tumor). Clinical features, laboratory values, pathological features, treatment delivered, and vital status were recorded. Univariate and multivariate Cox regression analyses were performed to evaluate prognostic factors. RESULTS: Thirty-two patients were evaluable. After a median of four cycles of neo-adjuvant chemotherapy gross total surgical resection was achieved in 29 patients and complete resection documented by histology in 22 patients. Three-year event free survival (EFS) and overall survival (OS) of the evaluable patients were 70.3 ± 8.3% and 76.8 ± 7.6%, respectively. Extent of tumor necrosis, platelet count at diagnosis, decline in serum alpha fetoprotein, and surgical margin status (positive vs. negative) were statistically significant predictors for both EFS and OS by univariate analysis. Multivariate analyses revealed that extent of tumor necrosis and surgical margin status predicted improved EFS (P < 0.001) and OS (P < 0.0001). CONCLUSIONS: Extent of tumor necrosis following neo-adjuvant chemotherapy is an independent prognostic factor in patients with newly diagnosed HB. Histological response may potentially be used in strategies to modify post-surgical therapy to improve survival in HB. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc.

PMID: 22190448 [PubMed - as supplied by publisher]
Mutations of PTCH1, MLL2, and MLL3 are not frequent events in hepatoblastoma.

Fetched: December 22nd, 2011, 10:01pm MST

Mutations of PTCH1, MLL2, and MLL3 are not frequent events in hepatoblastoma.

Pediatr Blood Cancer. 2011 Dec 19;

Authors: Chavan RS, Patel KU, Roy A, Thompson PA, Chintagumpala M, Goss JA, Nuchtern JG, Finegold MJ, Parsons DW, López-Terrada DH

PMID: 22183980 [PubMed - as supplied by publisher]
DLK1, a serum marker for hepatoblastoma in young infants.

Fetched: December 21st, 2011, 10:01pm MST

DLK1, a serum marker for hepatoblastoma in young infants.

Pediatr Blood Cancer. 2011 Dec 16;

Authors: Falix FA, Aronson DC, Lamers WH, Hiralall JK, Seppen J

Abstract
Hepatoblastoma is a malignant pediatric liver tumor. The currently used diagnostic serum marker for hepatoblastoma, α-fetoprotein (AFP), is not always reliable in infants with hepatoblastoma, due to the physiologically elevated levels of AFP in this age group. In this report, we show that Delta-like 1 homolog (DLK1), a protein highly expressed during fetal development, but almost completely absent after birth, and an established liver-stem cell marker, is a new candidate serum marker of hepatoblastoma, especially in young infants. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc.

PMID: 22180200 [PubMed - as supplied by publisher]
Treatment effects of the multikinase inhibitor sorafenib on hepatoblastoma cell lines and xenografts in NMRI-Foxn1(nu) mice.

Fetched: December 21st, 2011, 10:01pm MST

Treatment effects of the multikinase inhibitor sorafenib on hepatoblastoma cell lines and xenografts in NMRI-Foxn1(nu) mice.

Liver Int. 2011 Dec 18;

Authors: Eicher C, Dewerth A, Kirchner B, Warmann SW, Fuchs J, Armeanu-Ebinger S

Abstract
BACKGROUND: Multidrug resistance is a major reason for poor treatment results in advanced hepatoblastoma (HB). Several alternative treatment options are currently under investigation to improve the prognosis of affected patients AIMS: This study aimed to analyse the impact of sorafenib on the viability of HB cells and xenotransplanted HB tumours. METHODS: Cell viability and apoptosis were evaluated in two HB cell lines (HUH6 and HepT1) after treatment with sorafenib using MTT and Caspase 3 activation assay. Extracellular signal-regulated kinase (ERK) phosphorylation was investigated using Western blot. In addition, sorafenib (30 mg/kg) was administered orally to NMRI mice bearing subcutaneous HUH6 derived tumours. Tumour progression and viability were monitored by tumour volume and α-fetoprotein (AFP) levels, and apoptosis was assessed using TUNEL assay. Tumour angiogenesis and mean vascular density (MVD) was determined using CD31 staining, ERK phosphorylation was detected using indirect immunofluorescence. RESULTS: Treatment with sorafenib led to decreased ERK phosphorylation, reduced cell viability and induction of apoptosis in HepT1 and HUH6 cells. In HB xenografts, sorafenib significantly reduced tumour growth compared with control (P < 0.05). AFP levels were lower in the sorafenib group (P = 0.07). Relative apoptotic areas detected using TUNEL assay were increased (P = 0.003). CD31 staining revealed inhibition of angiogenesis, and mean vascular density was lower in the sorafenib group (P = 0.02). ERK phosphorylation was reduced in tumours tissues after sorafenib treatment. CONCLUSION: Treatment with sorafenib led to a potent inhibition of cell viability, tumour progression and angiogenesis. Sorafenib might therefore also be a promising treatment option for high risk or recurrent HB.

PMID: 22176637 [PubMed - as supplied by publisher]
Wnt signaling and telomerase activation of hepatoblastoma: correlation with chemosensitivity and surgical resectability.

Fetched: December 20th, 2011, 10:01pm MST

Wnt signaling and telomerase activation of hepatoblastoma: correlation with chemosensitivity and surgical resectability.

J Pediatr Surg. 2011 Dec;46(12):2221-7

Authors: Ueda Y, Hiyama E, Kamimatsuse A, Kamei N, Ogura K, Sueda T

Abstract
PURPOSE: Recently, it became apparent that telomerase directly modulated Wnt signaling as a cofactor in a β-catenin transcriptional complex. In this study, we investigated Wnt/β-catenin signaling and telomerase activation in hepatoblastoma (HBL).
METHODS: Tumors derived from 56 HBL cases treated with the Japanese Study Group for Pediatric Liver Tumors (JPLT) Protocol-2 were analyzed for oncogenic mutations (missense mutations and interstitial deletions in the third exon) of the CTNNB1 gene-encoding β-catenin and for the expression levels of telomerase reverse transcriptase (TERT).
RESULTS: Oncogenic mutations of CTNNB1 were detected in 42 cases (75%). The expression levels of TERT were significantly higher in 14 cases without mutation (P < .05) and in 8 cases with metastasis (P < .01). Interestingly, Wnt/β-catenin target genes were significantly activated in the tumors without mutations (P = .013). In cases with mutations, preoperative chemotherapy was more effective (P = .008), and complete resection rate was higher (P = .034). Consequently, 2 patients with mutations and 4 patients without mutations died of disease (P = .013). High expression of TERT was detected in all tumors of these dead patients.
CONCLUSIONS: Wnt/β-catenin signaling in the HBLs without CTNNB1 mutations was activated by high expression of TERT. The clinical courses in HBLs without CTNNB1 mutations seemed to be unfavorable because of chemoresistance and low rates of resectability.

PMID: 22152854 [PubMed - in process]
Evaluation of hydro-alcoholic extract of Eclipta alba for its anticancer potential: an in vitro study.

Fetched: December 20th, 2011, 10:01pm MST

Evaluation of hydro-alcoholic extract of Eclipta alba for its anticancer potential: an in vitro study.

J Ethnopharmacol. 2011 Jun 22;136(2):363-7

Authors: Chaudhary H, Dhuna V, Singh J, Kamboj SS, Seshadri S

Abstract
ETHNOPHARMACOLOGICAL RELEVANCE: Eclipta alba is traditionally used as hepatoprotective agent. The study was designed to explore its antiproliferative activity on liver and other related cancer.
AIM OF THE STUDY: The present study was designed to assess and establish the role of Eclipta alba as anti-cancer agent using HepG2, C6 glioma and A498 cell lines as model system.
MATERIALS AND METHODS: Antiproliferative and cytotoxic effects of the Eclipta alba hydroalcoholic extract (EAE) was determined using MTT assay. The expression level of NF-kB was analysed by western blotting and RT PCR. Gelatin zymography was done for gelatinase matrix metalloproteinases (MMP-2 and 9) analysis.
RESULTS: EAE inhibited the cell proliferation in dose dependent manner in HepG2, A498 and C6 glioma cell lines with an IC50 of 22±2.9, 25±3.6 and 50±8.7 μg/ml, respectively. The expression of MMP (2 and 9) was down-regulated with EAE treatment. DNA damage was observed following 72h of extract treatment, leading to apoptosis. Additionally, the expression level of NF-kB was evaluated with western blotting and RT-PCR and was found to be down-regulated/inactivated.
CONCLUSIONS: The data establish the existence of anti-proliferative, DNA damaging and anti-metastasis properties in EAE which is yet unexplored and hold high therapeutic impact.

PMID: 21575697 [PubMed - indexed for MEDLINE]
Potential biomarkers for hepatoblastoma: Results from the SIOPEL-3 study.

Fetched: December 8th, 2011, 10:00pm MST

Potential biomarkers for hepatoblastoma: Results from the SIOPEL-3 study.

Eur J Cancer. 2011 Nov 30;

Authors: Purcell R, Childs M, Maibach R, Miles C, Turner C, Zimmermann A, Czauderna P, Sullivan M

Abstract
Hepatoblastoma (HB) is a rare malignant liver tumour found in infants. Many heterogenous histological tumour subtypes exist. Although survival rates have improved dramatically in recent years with the use of platinum-based chemotherapy, there still exists a subset of HB that does not respond to treatment. There are currently no tumour biomarkers in use and in this study we aim to evaluate potential biomarkers to aid identification of relapse cases that would otherwise be overlooked by current prognostication. This may identify patients that would benefit from more aggressive therapy and could improve overall survival rates. We used immunohistochemistry to analyse the expression of β-catenin, E-cadherin, Cyclin D1, Ki-67 and alpha-fetoprotein (AFP) protein in tumours from 91 patients prospectively enroled into the SIOPEL 3 clinical trial. The relationship between these biomarkers and clinicopathologic features and patient survival were statistically analysed. We identified one biomarker, Cyclin D1, which has a correlation with mixed epithelial/mesenchymal HB approaching significance (P=0.07). Survival analysis using these markers has revealed two potential prognostic indicators; Cyclin D1 and Ki-67 (P=0.01, 0.01).

PMID: 22137595 [PubMed - as supplied by publisher]
Hepatic tumours in childhood: an experience at the Children Hospital and Institute of Child Health, Lahore.

Fetched: December 3rd, 2011, 10:00pm MST

Hepatic tumours in childhood: an experience at the Children Hospital and Institute of Child Health, Lahore.

J Pak Med Assoc. 2011 Nov;61(11):1079-82

Authors: Zaman S, Hanif G, Hussain M, Basit Z, Khan S, Rathore Z, Naeem A, Nasir M

Abstract
OBJECTIVES: In this report, we share our experience about the common types of childhood hepatic tumours during 10 years (2001-2010) and compare them with other studies.
METHODS: During 10 years (2001-2010), all the hepatic tumours of childhood received at Pathology Department of the Children Hospital and Institute of Child Health, Lahore Pakistan are recorded. This includes both resected specimens and biopsies. All the slides were reviewed and the pathologic diagnosis was confirmed.
RESULTS: We diagnosed 48 liver tumour cases in children (below 18 years of age). Among these tumours, 39 (81.25%) were malignant. Male to female ratio was 2:1. Hepatoblastoma was the most common liver tumour in this age group accounting for 69.23% of all malignant tumours (27 cases). The second most common primary tumour was hepatocellular carcinoma diagnosed in six patients (15.38%). Other malignant tumours were undifferentiated embryonal sarcoma and biliary rhabdomyosarcoma. Benign tumours included mesenchymal hamartoma, infantile haemangioendotheloima, hemangioma and benign cyst. There were also three metastatic tumours during these 10 years. In one case there was tumour necrosis only and as such no definitive diagnosis was rendered.
CONCLUSION: The spectrum of hepatic tumours in children is different from that found in the older age group and most of them are malignant.

PMID: 22125982 [PubMed - in process]
Inflammatory stress exacerbates hepatic cholesterol accumulation via disrupting cellular cholesterol export.

Fetched: November 29th, 2011, 10:00pm MST

Inflammatory stress exacerbates hepatic cholesterol accumulation via disrupting cellular cholesterol export.

J Gastroenterol Hepatol. 2011 Nov 18;

Authors: Chen Y, Chen Y, Zhao L, Chen Y, Mei M, Li Q, Huang A, Varghese Z, Moorhead JF, Z Ruan X

Abstract
Background and Aim: Both inflammation and cholesterol accumulation play important roles in the development of non-alcoholic fatty liver disease. This study was undertaken to investigate whether inflammation aggravated cholesterol accumulation via disrupting hepatic cholesterol export and explored underlying mechanisms. Methods: We used casein injection in C57BL/6J mice, tumor necrosis factor alpha (TNFα) stimulation in human hepatoblastoma cell line (HepG2) cells to induce inflammation. Intracellular cholesterol level was examined by Oil Red O staining and quantitative analysis. Bile acid level was quantified by colorimetric analysis. (3) [H] cholesterol assay by liquid scintillation counter was performed to evaluate the cholesterol efflux. The mRNA and protein expression was examined by real-time PCR and Western blot. Results: Inflammation increased cholesterol accumulation in livers of C57BL/6J mice and in HepG2 cells. High fat diet in mice and low density lipoprotein (LDL) loading in HepG2 cells increased bile acid synthesis and cholesterol efflux , enhanced the mRNA and protein expression of liver X receptor (LXRα) , peroxisome proliferator -activated receptors (PPARα, γ), cholesterol 7α-hydroxylase (CYP7A1) and ATP-binding cassette transporter A1 (ABCA1). However, inflammation reduced bile acid synthesis and cholesterol efflux even in high fat diet fed-mice and HepG2 cells in presence of LDL loading. The enhanced effects of these genes and proteins expression due to high fat diet and LDL loading were inhibited by inflammation both in vivo and in vitro. Conclusions: Inflammation disrupted PPAR-LXR-CYP7A1/ABCA1-mediated bile acid synthesis and cholesterol efflux resulting in exacerbated cholesterol accumulation in livers of C57BL/6J mice and HepG2 cells.

PMID: 22098164 [PubMed - as supplied by publisher]
Deregulation of Hippo kinase signalling in Human hepatic malignancies.

Fetched: November 29th, 2011, 10:00pm MST

Deregulation of Hippo kinase signalling in Human hepatic malignancies.

Liver Int. 2011 Oct 20;

Authors: Li H, Wolfe A, Septer S, Edwards G, Zhong X, Bashar Abdulkarim A, Ranganathan S, Apte U

Abstract
BACKGROUND/AIMS: Hepatocellular carcinoma (HCC), cholangiocarcinoma (CC) and hepatoblastoma (HB) are the main hepatic malignancies with limited treatment options and high mortality. Recent studies have implicated Hippo kinase pathway in cancer development, but detailed analysis of Hippo kinase signalling in human hepatic malignancies, especially CC and HB, is lacking. METHODS: We investigated Hippo kinase signalling in HCC, CC and HB using cells and patient samples. RESULTS: Increased expression of yes-associated protein (Yap), the downstream effector of the Hippo kinase pathway, was observed in HCC cells, and siRNA-mediated knockdown of Yap resulted in decreased survival of HCC cells. The density-dependent activation of Hippo kinase pathway characteristic of normal cells was not observed in HCC cells and CCLP cells, a cholangiocarcinoma cell line. Immunohistochemistry of Yap in HCC, CC and HB tissues indicated extensive nuclear localization of Yap in majority of tissues. Western blot analysis performed using total cell extracts from patient samples and normal livers showed extensive activation of Yap. Marked induction of Glypican-3, CTGF and Survivin, the three Yap target genes was observed in the tumour samples. Further analysis revealed significant decrease in expression and activity of Lats kinase, the main upstream regulator of Yap. However, no change in activation of Mst-2 kinase, the upstream regulator of Lats kinase was observed. CONCLUSIONS: These data show that Yap induction mediated by inactivation of Lats is observed in hepatic malignancies. These studies highlight Hippo kinase pathway as a novel therapeutic target for hepatic malignancies.

PMID: 22098159 [PubMed - as supplied by publisher]
Hepatoblastoma in the nordic countries.

Fetched: November 22nd, 2011, 10:00pm MST

Hepatoblastoma in the nordic countries.

Int J Cancer. 2011 Nov 18;

Authors: de Fine Licht S, Schmidt LS, Rod NH, Schmiegelow K, Lähteenmäki PM, Kogner P, Träger C, Stokland T, Schüz J

Abstract
Little is known about the aetiology of hepatoblastoma. Because of the young age at diagnosis, several studies have looked at various birth characteristics. The purpose of this study was to investigate the incidence of hepatoblastoma in the Nordic countries and the association between selected birth characteristics and hepatoblastoma. Data from national cancer registries and birth registries in Denmark, Sweden, Norway and Finland 1985-2006 was used. Overall, 155 children with hepatoblastoma aged 0-14 years were included and individually matched to 5 controls drawn randomly from national population registries. The incidence rate of hepatoblastoma was 1.7 per million person-years with a predominance of boys (1.5:1). Incidence rate was highest before the age of 1 year (8.3 per million person-years). A higher risk of hepatoblastoma was found in children with birth weight <1500g (Odds ratio (OR)=9.5; 95% confidence interval (CI): 2.3, 38.2), born preterm in week 22-32 (OR=4.5; CI: 1.8, 11.5) and Apgar scores <7 after 1 minute (OR=3.1; CI: 1.3, 7.1) and 5 minutes (OR=7.5; CI: 1.8, 32.4). A doubling in risk was found in children who were large for gestational age (OR= 2.3; CI: 1.0, 5.3). No associations were found with birth order, maternal age or maternal smoking. This study indicates that intrauterine and/or neonatal factors are associated with increased risk of hepatoblastoma. These may include low birth weight and asphyxia leading to neonatal intensive care. Alternatively, the factors may be a consequence of hepatoblastoma developing in utero.

PMID: 22095187 [PubMed - as supplied by publisher]
The effects of garlic-derived sulfur compounds on cell proliferation, caspase 3 activity, thiol levels and anaerobic sulfur metabolism in human hepatoblastoma HepG2 cells.

Fetched: November 22nd, 2011, 10:00pm MST

The effects of garlic-derived sulfur compounds on cell proliferation, caspase 3 activity, thiol levels and anaerobic sulfur metabolism in human hepatoblastoma HepG2 cells.

Cell Biochem Funct. 2011 Nov 18;

Authors: Iciek M, Kwiecień I, Chwatko G, Sokołowska-Jeżewicz M, Kowalczyk-Pachel D, Rokita H

Abstract
The aim of the present studies was to determine whether the mechanism of biological action of garlic-derived sulfur compounds in human hepatoma (HepG2) cells can be dependent on the presence of labile sulfane sulfur in their molecules. We investigated the effect of allyl sulfides from garlic: monosulfide, disulfide and trisulfide on cell proliferation and viability, caspase 3 activity and hydrogen peroxide (H(2) O(2) ) production in HepG2 cells. In parallel, we also examined the influence of the previously mentioned compounds on the levels of thiols, glutathione, cysteine and cysteinyl-glycine, and on the level of sulfane sulfur and the activity of its metabolic enzymes: rhodanese, 3-mercaptopyruvate sulfurtransferase and cystathionase. Among the compounds under study, diallyl trisulfide (DATS), a sulfane sulfur-containing compound, showed the highest biological activity in HepG2 cells. This compound increased the H(2) O(2) formation, lowered the thiol level and produced the strongest inhibition of cell proliferation and the greatest induction of caspase 3 activity in HepG2 cells. DATS did not affect the activity of sulfurtransferases and lowered sulfane sulfur level in HepG2 cells. It appears that sulfane sulfur containing DATS can be bioreduced in cancer cells to hydroperthiol that leads to H(2) O(2) generation, thereby influencing transmission of signals regulating cell proliferation and apoptosis. Copyright © 2011 John Wiley & Sons, Ltd.

PMID: 22095390 [PubMed - as supplied by publisher]
Outcome of Hepatoblastoma in the Indian Context.

Fetched: November 16th, 2011, 10:01pm MST

Outcome of Hepatoblastoma in the Indian Context.

Indian Pediatr. 2011 Nov 1;

Authors: Arora RS

Abstract
A comprehensive review and critical appraisal of published and grey literature was undertaken to identify current treatment practices and outcomes of children with hepatoblastoma in India. Eight single-centre studies with 157 patients (range five to 36 patients in each study) were included. Pre-operative chemotherapy (mainly cisplatin and doxorubicin) followed by surgical resection and additional chemotherapy was the usual practice. There was no stratification of treatment by risk group in any of the studies. The median event-free survival ranged from 33-100%. The two main reasons for treatment failure were treatment-related mortality (0-50%) and progression of disease (0-30%).

PMID: 22080621 [PubMed - as supplied by publisher]
Hepatoblastoma: 15-year experience and role of surgical treatment.

Fetched: November 10th, 2011, 10:01pm MST

Hepatoblastoma: 15-year experience and role of surgical treatment.

J Korean Surg Soc. 2011 Aug;81(2):134-40

Authors: Moon SB, Shin HB, Seo JM, Lee SK

Abstract
PURPOSE: Hepatoblastoma is the most common malignant liver tumor in children. The aim of this study was to review our results of hepatoblastoma treatment and to determine the role of surgical treatment in hepatoblastoma.
METHODS: This is a retrospective clinical study. The medical records of patients with hepatoblastoma, treated between October 1994 and October 2009, were reviewed. The patients were classified according to the pretreatment extent of disease (PRETEXT) grouping system. The main outcome variable was survival. Secondary outcome variables were complete, partial and no response to chemotherapy and surgery, when indicated.
RESULTS: Twenty-seven patients were treated during the observation period. Eighteen were males. Five were PRETEXT group I, 8 group II, 13 group III and 1 group IV. Complete excision was achieved in all patients except in one case that underwent liver transplantation (group IV). Median follow-up and survival rate were 2.3 years and 100%, 6.6 years and 75%, 5.8 years and 92%, 7.7 years and 100%, for groups I to IV, respectively. Twenty patients are currently considered to be in complete response status and three patients are receiving postoperative chemotherapy. Four patients died; the causes of death were cytomegalovirus hepatitis, bone marrow suppression during adjuvant chemotherapy, primarynonfunction after the transplantation for recurrent tumor and metachronous rectal cancer, respectively.
CONCLUSION: Favorable long-term outcome could be expected for hepatoblastoma with complete tumor excision and adjuvant chemotherapy.

PMID: 22066113 [PubMed - in process]

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