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https://www.readbyqxmd.com/read/27913912/genetic-counselor-practices-involving-pediatric-patients-with-fap-an-investigation-of-their-self-reported-strategies-for-genetic-testing-and-hepatoblastoma-screening
#1
Caitlin E Lawson, Thomas M Attard, Hongying Dai, Seth Septer
Familial adenomatous polyposis (FAP) is a cancer predisposition syndrome that causes early-onset polyposis and is associated with an increased risk for hepatoblastoma. There is currently a lack of consensus on when to order APC (adenomatous polyposis coli) gene testing or implement surveillance for hepatoblastoma. An online questionnaire was completed by 62 genetic counselors to capture their current practices regarding these questions. Extracolonic findings associated with FAP that were most likely to prompt APC testing in an otherwise asymptomatic 10 year-old child with a negative family history were multiple desmoid tumors, congenital hypertrophy of the retinal pigment epithelium (CHRPE), jaw osteomas, and hepatoblastoma...
December 3, 2016: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/27910913/loss-of-egfr-asap1-signaling-in-metastatic-and-unresectable-hepatoblastoma
#2
Sarangarajan Ranganathan, Mylarappa Ningappa, Chethan Ashokkumar, Brandon W Higgs, Jun Min, Qing Sun, Lori Schmitt, Shankar Subramaniam, Hakon Hakonarson, Rakesh Sindhi
Hepatoblastoma (HBL), the most common childhood liver cancer is cured with surgical resection after chemotherapy or with liver transplantation if local invasion and multifocality preclude resection. However, variable survival rates of 60-80% and debilitating chemotherapy sequelae argue for more informed treatment selection, which is not possible by grading the Wnt-β-catenin over activity present in most HBL tumors. A hypothesis-generating whole transcriptome analysis shows that HBL tumors removed at transplantation are enriched most for cancer signaling pathways which depend predominantly on epidermal growth factor (EGF) signaling, and to a lesser extent, on aberrant Wnt-β-catenin signaling...
December 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27905197/adult-hepatoblastoma-a-review-of-47-cases
#3
Xiao-Feng Duan, Qiang Zhao
BACKGROUND: Adult hepatoblastoma is a very rare and aggressive neoplasm. The aim of this study was to review the cases of adult hepatoblastoma patients. METHODS: A search of all case reports about adult hepatoblastoma from 1958 to 2016 by medical database was performed. Data about demographic information, clinical symptoms and signs, laboratory test and imaging examination, treatment and clinical outcomes were extracted and analysed. RESULTS: A total of 43 English articles including 47 cases of adult hepatoblastoma patients were collected...
November 30, 2016: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/27895778/regulatory-network-analysis-of-genes-and-micrornas-in-human-hepatoblastoma
#4
Jimin He, Xiaoxin Guo, Linlin Sun, Ning Wang, Jiwei Bao
Hepatoblastoma (HB) is a common type of primary tumor in children. Previous studies have examined the expression of genes, including transcription factors (TFs), target genes, host genes and microRNAs (miRNAs or miRs) associated with HB. However, the regulatory pathways of miRNAs and genes remain unclear. In the present study, a novel perspective is proposed, which focuses on HB and the associated regulatory pathways, to construct three networks at various levels, including a differentially expressed network, an associated network and a global network...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27895749/a-single-center-retrospective-study-of-pediatric-hepatoblastoma
#5
Yi Zhang, Weiling Zhang, Suoqin Tang, Liping Chen, You Yi, Pinwei Zhang, Aiping Liu, Tian Zhi, Dongsheng Huang
Hepatoblastoma is a malignant liver tumor generally diagnosed in infants and children <3 years old. The current retrospective study aimed to investigate the associations of tumor stage, pathological type, metastasis and chemotherapy with clinical outcomes. In the current study, a total of 102 patients with hepatoblastoma were enrolled between September 2006 and June 2014. Clinical records and follow-up information for each of patient were obtained to conduct a Kaplan-Meier survival analysis and log-rank test...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27884763/acox2-deficiency-an-inborn-error-of-bile-acid-synthesis-identified-in-an-adolescent-with-persistent-hypertransaminasemia
#6
Maria J Monte, Marta Alonso-Peña, Oscar Briz, Elisa Herraez, Carmen Berasain, Josepmaria Argemi, Jesus Prieto, Jose J G Marin
BACKGROUND: Acyl-CoA oxidase (ACOX2) is involved in the shortening of C27 cholesterol derivatives to generate C24 bile acids. Inborn errors affecting the rest of peroxisomal enzymes involved in bile acid biosynthesis have been described. These conditions are usually characterized by serious hepatic and/or neurologic manifestations. AIMS: We have investigated bile acid profile and enzymes involved in their biosynthesis in the first reported case of ACOX2 deficiency identified in an adolescent boy with persistent unexplained hypertransaminasemia and his family...
November 21, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27884679/risk-stratified-staging-in-paediatric-hepatoblastoma-a-unified-analysis-from-the-children-s-hepatic-tumors-international-collaboration
#7
Rebecka L Meyers, Rudolf Maibach, Eiso Hiyama, Beate Häberle, Mark Krailo, Arun Rangaswami, Daniel C Aronson, Marcio H Malogolowkin, Giorgio Perilongo, Dietrich von Schweinitz, Marc Ansari, Dolores Lopez-Terrada, Yukichi Tanaka, Rita Alaggio, Ivo Leuschner, Tomoro Hishiki, Irene Schmid, Kenichiro Watanabe, Kenichi Yoshimura, Yurong Feng, Eugenia Rinaldi, Davide Saraceno, Marisa Derosa, Piotr Czauderna
BACKGROUND: Comparative assessment of treatment results in paediatric hepatoblastoma trials has been hampered by small patient numbers and the use of multiple disparate staging systems by the four major trial groups. To address this challenge, we formed a global coalition, the Children's Hepatic tumors International Collaboration (CHIC), with the aim of creating a common approach to staging and risk stratification in this rare cancer. METHODS: The CHIC steering committee-consisting of leadership from the four major cooperative trial groups (the International Childhood Liver Tumours Strategy Group, Children's Oncology Group, the German Society for Paediatric Oncology and Haematology, and the Japanese Study Group for Paediatric Liver Tumours)-created a shared international database that includes comprehensive data from 1605 children treated in eight multicentre hepatoblastoma trials over 25 years...
November 21, 2016: Lancet Oncology
https://www.readbyqxmd.com/read/27884678/a-common-global-risk-stratification-system-for-hepatoblastoma
#8
Furqan Shaikh
No abstract text is available yet for this article.
November 21, 2016: Lancet Oncology
https://www.readbyqxmd.com/read/27826578/early-multifocal-recurrence-of-hepatoblastoma-in-the-residual-liver-after-r0-liver-resection-with-alpps-procedure-a-case-report
#9
Abid Q Qazi, Aamir Ali Syed, Ahsun W Khan, Faisal Hanif
Associated liver partition and portal vein ligation for staged hepatectomy (ALPPS) has been successfully employed in adults to avoid potential liver insufficiency in patient with small future liver volume (FLV) when subjected to conventional right trisectionectomy. We are reporting an infant with hepatoblastoma, who underwent a technically successful ALPPS procedure with R0 resection. However he developed very early recurrence within the first month after the procedure. We present this report with a view to share our unexpected finding of early multifocal recurrence of hepatoblastoma in the residual liver after ALPPS procedure in a very young patient...
October 2016: Annals of Translational Medicine
https://www.readbyqxmd.com/read/27793881/therapeutic-innovations-for-targeting-hepatoblastoma
#10
REVIEW
Agnès Garnier, Matthias Ilmer, Roland Kappler, Michael Berger
Hepatoblastoma is the most common pediatric liver tumor. Despite recent advances in treatment with surgery and chemotherapy, the prognosis in advanced stages remains poor. The neurokinin-1 receptor (NK1R) has recently been described to be pivotal in the development of cancer. Furthermore, overwhelming evidence now exists showing that pharmacological manipulation of NK1R can cause a robust anticancer effect. Consequently, NK1R antagonists, such as the clinical drug aprepitant, are under current investigation as future innovative anticancer agents...
November 2016: Anticancer Research
https://www.readbyqxmd.com/read/27790374/loss-of-function-mutations-and-global-rearrangements-in-gpc3-in-patients-with-simpson-golabi-behmel-syndrome
#11
Keiko Shimojima, Yumiko Ondo, Eriko Nishi, Seiji Mizuno, Miharu Ito, Aya Ioi, Mariko Shimizu, Maho Sato, Masami Inoue, Nobuhiko Okamoto, Toshiyuki Yamamoto
Simpson-Golabi-Behmel syndrome is a congenital malformation syndrome associated with mutations in GPC3, which is located in the Xq26 region. Three new loss-of-function mutations and a global X-chromosome rearrangement involving GPC3 were identified. A female sibling of the patient, who presented with a cleft palate and hepatoblastoma, carries the same chromosomal rearrangement and a paradoxical pattern of X-chromosome inactivation. These findings support variable GPC3 alterations, with a possible mechanism in female patients...
2016: Human Genome Variation
https://www.readbyqxmd.com/read/27788486/gata4-promotes-hepatoblastoma-cell-proliferation-by-altering-expression-of-mir125b-and-dkk3
#12
Yihua Pei, Qin Yao, Sibo Yuan, Bozhen Xie, Yan Liu, Chunsheng Ye, Huiqin Zhuo
GATA4 is a zinc finger DNA-binding protein that plays an important role in mammalian liver development. However, the effects of GATA4 in hepatoblastoma (HB), a common liver cancer in pediatric patients, remain largely unknown. In this study, we demonstrate that GATA4 promotes growth and survival in the Huh6 human hepatoblastoma cell line. GATA4 expression was high in Huh6 cells, and its knockdown decreased expression of Dickkopf-related protein 3 (DKK3), a gene that may contribute to premature or undifferentiated phenotypes in HB...
October 24, 2016: Oncotarget
https://www.readbyqxmd.com/read/27783314/space-occupying-lesions-in-the-liver
#13
REVIEW
Manigandan Subramanyam Thyagarajan, Khalid Sharif
Space occupying liver lesions usually present with abdominal pain or abnormal physical findings, such as a palpable abdominal mass or distention. Liver lesions identified in children include benign and malignant neoplasms, inflammatory masses, cysts and metastatic lesions. Two-thirds of liver lesions in children are malignant. Hepatoblastoma accounts for two-thirds of malignant liver tumors in children. Benign lesions of the liver in children include vascular lesions, hamartomas, adenomas, and focal nodular hyperplasia...
October 25, 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27781375/multidisciplinary-management-of-hepatoblastoma-in-children-experience-from-a-developing-country
#14
Naresh Shanmugam, Julius Xavier Scott, Vimal Kumar, Mukul Vij, Priya Ramachandran, Gomathy Narasimhan, Mettu Srinivas Reddy, Venugopal Kota, Deenadayalan Munirathnam, Chayarani Kelgeri, Karthick Sundaram, Mohamed Rela
BACKGROUND: Advances in chemotherapy, liver resection techniques, and pediatric liver transplantation have vastly improved survival in children with hepatoblastoma (HB). These are best managed by a multidisciplinary team (MDT) in a setting where all treatment options are available. Until recently, this was difficult to achieve in India. METHODS: All children (<16 years) with HB treated in a pediatric liver surgery and transplantation unit between January 2011 and July 2016 were reviewed...
October 26, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27775819/genomic-analysis-of-hepatoblastoma-identifies-distinct-molecular-and-prognostic-subgroups
#15
Pavel Sumazin, Yidong Chen, Lisa R Treviño, Stephen F Sarabia, Oliver A Hampton, Kayuri Patel, Toni-Ann Mistretta, Barry Zorman, Patrick Thompson, Andras Heczey, Sarah Comerford, David A Wheeler, Murali Chintagumpala, Rebecka Meyers, Dinesh Rakheja, Milton J Finegold, Gail Tomlinson, D Williams Parsons, Dolores López-Terrada
Despite being the most common liver cancer in children, hepatoblastoma (HB) is a rare neoplasm. Consequently, few pre-treatment tumors have been molecularly profiled and there are no validated prognostic or therapeutic biomarkers for HB patients. We report on the first large-scale effort to profile pre-treatment HBs at diagnosis. Our analysis of 88 clinically-annotated HBs revealed three risk-stratifying molecular subtypes that are characterized by differential activation of hepatic progenitor cell markers and metabolic pathways: high-risk tumors were characterized by up-regulated NFE2L2 activity, high LIN28B, HMGA2, SALL4 and AFP expression, and high coordinated expression of oncofetal proteins and stem cell markers; while low-risk tumors had low LIN28B and let-7 expression, and high HNF1A activity...
October 24, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27775667/potential-anticancer-effects-of-polyphenols-from-chestnut-shell-extracts-modulation-of-cell-growth-and-cytokinomic-and-metabolomic-profiles
#16
Angela Sorice, Francesco Siano, Francesca Capone, Eliana Guerriero, Gianluca Picariello, Alfredo Budillon, Gennaro Ciliberto, Marina Paolucci, Susan Costantini, Maria Grazia Volpe
In this study, a hydroalcoholic chestnut shell extract was characterized and tested on six different human cell lines. Gallic, ellagic, and syringic acids were the most abundant non-condensed compounds in the chestnut extract, as determined by high performance liquid chromatography (HPLC). Tannins were mainly represented by condensed monomeric units of epigallocatechin and catechin/epicatechin. After 48 h of treatment, only the human hepatoblastoma HepG2 cells reached an inhibition corresponding to IC50 with an increase of apoptosis and mitochondrial depolarization...
October 21, 2016: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/27775293/-thoracoscopic-resection-of-lung-metastases-guided-by-hook-wire
#17
L Rodríguez Caraballo, J Gómez-Chacón Villalban, P Rodríguez Iglesias, L Mangas Álvarez, A Marco Macián
OBJECTIVE: To describe our experience in the use of the guided hook wire placement to undergo thoracoscopic resection of pulmonary metastases in children. MATERIAL AND METHODS: We conducted a retrospective review, between January 2008 and December 2014 of the patients that were diagnosed with pulmonary metastases by image and who underwent a thoracoscopic metastasectomy. Patients in whom a CT- guided hook was done before the surgery were included. RESULTS: Eleven procedures were done in ten patients with a median age of 10 years (range 2-16 years)...
October 10, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27771945/binding-and-uptake-into-human-hepatocellular-carcinoma-cells-of-peptide-functionalized-gold-nanoparticles
#18
Satadru Jha, Federico Ramadori, Santina Quarta, Alessandra Biasiolo, Enrica Fabris, Paola Baldan, Gaetano Guarino, Mariagrazia Ruvoletto, Gianmarco Villano, Cristian Turato, Angelo Gatta, Fabrizio Mancin, Patrizia Pontisso, Paolo Scrimin
One of the most daunting challenges of nanomedicine is the finding of appropriate targeting agents to deliver suitable payloads precisely to cells affected by malignancies. Even more complex is to achieve the ability to ensure the nanosystems enter those cells. Here we use 2 nm (metal core) gold nanoparticles to target human hepatocellular carcinoma (HepG2) cells stably transfected with the SERPINB3 (SB3) protein. The nanoparticles were coated with a 85:15 mixture of thiols featuring, respectively, a phosphoryl choline, to ensure water solubility and biocompatibility, and a 28-mer peptide corresponding to the amino acid sequence 21-47 of the hepatitis B virus-PreS1 protein (PreS1(21-47))...
October 22, 2016: Bioconjugate Chemistry
https://www.readbyqxmd.com/read/27768709/maternal-and-birth-characteristics-and-childhood-embryonal-solid-tumors-a-population-based-report-from-brazil
#19
Neimar de Paula Silva, Rejane de Souza Reis, Rafael Garcia Cunha, Júlio Fernando Pinto Oliveira, Marceli de Oliveira Santos, Maria S Pombo-de-Oliveira, Beatriz de Camargo
BACKGROUND: Several maternal and birth characteristics have been reported to be associated with an increased risk of many childhood cancers. Our goal was to evaluate the risk of childhood embryonal solid tumors in relation to pre- and perinatal characteristics. METHODS: A case-cohort study was performed using two population-based datasets, which were linked through R software. Tumors were classified as central nervous system (CNS) or non-CNS-embryonal (retinoblastoma, neuroblastoma, renal tumors, germ cell tumors, hepatoblastoma and soft tissue sarcoma)...
2016: PloS One
https://www.readbyqxmd.com/read/27738108/coordinated-activities-of-multiple-myc-dependent-and-myc-independent-biosynthetic-pathways-in-hepatoblastoma
#20
Huabo Wang, Jie Lu, Lia R Edmunds, Sucheta Kulkarni, James Dolezal, Junyan Tao, Sarangarajan Ranganathan, Laura Jackson, Marc Fromherz, Donna Beer Stolz, Radha Uppala, Sivakama Bharathi, Satdarshan P Monga, Eric S Goetzman, Edward V Prochownik
Hepatoblastoma (HB) is associated with aberrant activation of the β-catenin and Hippo/YAP signaling pathways. Over-expression of mutant β-catenin and YAP in mice induces HBs that express high levels of c-Myc (Myc). In light of recent observations that Myc in unnecessary for long-term hepatocyte proliferation, we have now examined its role in HB pathogenesis using the above model. While Myc was found to be dispensable for in vivo HB initiation it was necessary to sustain rapid tumor growth. Gene expression profiling identified key molecular differences between myc+/+ (WT) and myc-/- (KO) hepatocytes and HBs that explain these behaviors...
October 13, 2016: Journal of Biological Chemistry
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