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https://www.readbyqxmd.com/read/28921939/extreme-hepatic-resections-for-the-treatment-of-advanced-hepatoblastoma-are-planned-close-margins-an-acceptable-approach
#1
Adriana Fonseca, Abha Gupta, Furqan Shaikh, Raveena Ramphal, Vicky Ng, Ian McGilvray, J Ted Gerstle
BACKGROUND: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. OBJECTIVE: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. METHODS: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015...
September 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28921839/hepatoblastoma-in-children-aged-less-than-six-months-at-diagnosis-a-report-from-the-siopel-group
#2
Patrizia Dall'Igna, Laurence Brugieres, Anne Sanlaville Christin, Rudolf Maibach, Michela Casanova, Rita Alaggio, Jean de Ville de Goyet, Jozsef Zsiros, Bruce Morland, Piotr Czauderna, Margaret Childs, Daniel C Aronson, Sophie Branchereau, Penelope Brock, Giorgio Perilongo
BACKGROUND: The purpose of this study was to evaluate clinical characteristics, treatment, and survival of children, who were diagnosed with hepatoblastoma (HB) in their first 6 months of age, enrolled in the SIOPEL 2 and 3 protocols. METHODS: Seventy-nine patients, treated between 1994 and 2006, were analyzed after stratification into three age groups: <1 month, between 1 and 3 months, and between 3 and 6 months. All received preoperative chemotherapy. RESULTS: Clinical characteristics were similar in both trials: 4 patients had pulmonary metastases at diagnosis, 4 had α-fetoprotein <100 ng/ml, 68 were operated by partial hepatectomy, and 7 received liver transplant...
September 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28915934/increased-yap-activation-is-associated-with-hepatic-cyst-epithelial-cell-proliferation-in-arpkd-chf
#3
Lu Jiang, Lina Sun, Genea Edwards, Michael Manley, Darren Wallace, Seth Septer, Chirag Manohar, Michele T Pritchard, Udayan Apte
Autosomal recessive polycystic kidney disease/congenital hepatic fibrosis (ARPKD/CHF) is a rare but fatal genetic disease characterized by progressive cyst development in the kidneys and liver. Liver cysts arise from aberrantly proliferative cholangiocytes accompanied by pericystic fibrosis and inflammation. Yes associated protein (YAP), the downstream effector of the Hippo signaling pathway, is implicated in human hepatic malignancies such as hepatocellular carcinoma, cholangiocarcinoma, and hepatoblastoma, but its role in hepatic cystogenesis in CHF/ARPKD is unknown...
September 15, 2017: Gene Expression
https://www.readbyqxmd.com/read/28892430/characterization-of-pulmonary-metastases-in-children-with-hepatoblastoma-treated-on-children-s-oncology-group-protocol-ahep0731-the-treatment-of-children-with-all-stages-of-hepatoblastoma-a-report-from-the-children-s-oncology-group
#4
Allison F O'Neill, Alexander J Towbin, Mark D Krailo, Caihong Xia, Yun Gao, M Beth McCarville, Rebecka L Meyers, Eugene D McGahren, Greg M Tiao, Stephen P Dunn, Max R Langham, Christopher B Weldon, Milton J Finegold, Sarangarajan Ranganathan, Wayne L Furman, Marcio Malogolowkin, Carlos Rodriguez-Galindo, Howard M Katzenstein
Purpose To determine whether the pattern of lung nodules in children with metastatic hepatoblastoma (HB) correlates with outcome. Methods Thirty-two patients with metastatic HB were enrolled on Children's Oncology Group Protocol AHEP0731 and treated with vincristine and irinotecan (VI). Responders to VI received two additional cycles of VI intermixed with six cycles of cisplatin/fluorouracil/vincristine/doxorubicin (C5VD), and nonresponders received six cycles of C5VD alone. Patients were imaged after every two cycles and at the conclusion of therapy...
September 11, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28888119/multiple-endpoints-gene-alteration-based-mega-assay-a-toxicogenomics-approach-for-water-quality-assessment-of-wastewater-effluents
#5
Toshikazu Fukushima, Hiroe Hara-Yamamura, Koji Nakashima, Lea Chua Tan, Satoshi Okabe
Wastewater effluents contain a significant number of toxic contaminants, which, even at low concentrations, display a wide variety of toxic actions. In this study, we developed a multiple-endpoints gene alteration-based (MEGA) assay, a real-time PCR-based transcriptomic analysis, to assess the water quality of wastewater effluents for human health risk assessment and management. Twenty-one genes from the human hepatoblastoma cell line (HepG2), covering the basic health-relevant stress responses such as response to xenobiotics, genotoxicity, and cytotoxicity, were selected and incorporated into the MEGA assay...
August 21, 2017: Chemosphere
https://www.readbyqxmd.com/read/28883002/genetic-dissociation-of-glycolysis-and-the-tca-cycle-affects-neither-normal-nor-neoplastic-proliferation
#6
Laura E Jackson, Sucheta Kulkarni, Huabo Wang, Jie Lu, James M Dolezal, Sivakama S Bharathi, Sarangarajan Ranganathan, Mulchand S Patel, Rahul Deshpande, Frances Alencastro, Stacy G Wendell, Eric S Goetzman, Andrew W Duncan, Edward V Prochownik
Rapidly proliferating cells increase glycolysis at the expense of oxidative phosphorylation (oxphos) to generate sufficient levels of glycolytic intermediates for use as anabolic substrates. The pyruvate dehydrogenase complex (PDC) is a critical mitochondrial enzyme that catalyzes pyruvate's conversion to acetyl coenzyme A (AcCoA), thereby connecting these two pathways in response to complex energetic, enzymatic and metabolic cues. Here we utilized a mouse model of hepatocyte-specific PDC inactivation to determine the need for this metabolic link during normal hepatocyte regeneration and malignant transformation...
September 7, 2017: Cancer Research
https://www.readbyqxmd.com/read/28851352/prognostic-roles-of-pathology-markers-immunoexpression-and-clinical-parameters-in-hepatoblastoma
#7
Jia-Feng Wu, Hsiu-Hao Chang, Meng-Yao Lu, Shiann-Tarng Jou, Kai-Chi Chang, Yen-Hsuan Ni, Mei-Hwei Chang
BACKGROUND: Hepatoblastoma, a leading primary hepatic malignant tumor in children, is originated from primitive hepatic stem cells. We aimed to elucidate the relationships between the histological distribution of β-catenin and hepatic stem cell markers with the clinical outcomes of hepatoblastoma. METHODS: Immunohistochemistry was applied to detect β-catenin and hepatic stem cell markers expression in 31 hepatoblastoma tumors. We analyzed the relationship between the stem cell markers and the clinical course of hepatoblastoma...
August 29, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28844238/mechanisms-of-chromosomal-aberrations-induced-by-sesamin-metabolites-in-chinese-hamster-lung-cells
#8
Yoshiko Ono, Namino Tomimori, Hisako Hori, Yoshinori Kitagawa, Hiroshi Shibata
Sesamin is a major lignan in sesame seeds and oil. We previously demonstrated that sesamin induces chromosomal aberrations (CA) in Chinese hamster lung (CHL/IU) cells in the presence of a metabolic activation system (S9 mix), although no genotoxicity was detected in vivo. To clarify the mechanism of CA induction by sesamin, we identified its principal active metabolite. A mono-catechol derivative, [2-(3,4-methylenedioxyphenyl)-6-(3,4-dihydroxyphenyl)-3,7-dioxabi-cyclo[3.3.0]octane (SC-1)], was previously identified in culture medium when sesamin was incubated with S9 mix...
October 2017: Mutation Research
https://www.readbyqxmd.com/read/28837514/fatal-central-nervous-system-post-transplant-lymphoproliferative-disease-in-a-patient-who-underwent-liver-transplantation-for-hepatoblastoma
#9
Kenneth Ng, Abbas Rana, Prakash Masand, Kalyani Patel, Andras Heczey, John Goss, Ryan Himes
No abstract text is available yet for this article.
August 23, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28834194/pediatric-liver-transplantation-for-hepatocellular-cancer-and-rare-liver-malignancies-us-multi-and-single-center-experience-1981-2015
#10
Rohan Vinayak, Ruy J Cruz, Sarangarajan Ranganathan, Ravi Mohanka, George Mazariegos, Kyle Soltys, Geoff Bond, Sameh Tadros, Abhinav Humar, J Wallis Marsh, Robert R Selby, Jorge Reyes, Kimberly Haberman, Rakesh Sindhi
Pediatric liver transplantation (LTx) is increasingly performed for rare unresectable liver malignancies other than hepatoblastoma (HBL). PURPOSE: We performed a retrospective review of outcomes after LTx for malignancy in the multi-center US Scientific Registry for Transplant Recipients (SRTR, n=677, 1987-2015). We then reviewed the Children's Hospital of Pittsburgh (CHP, n=74, 1981-2014) experience focusing on LTx for unresectable hepatocellular cancer (HCC), non-HBL embryonal tumors (EMB) and metastatic liver tumors (METS)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28820908/ribosomopathy-like-properties-of-murine-and-human-cancers
#11
Sucheta Kulkarni, James M Dolezal, Huabo Wang, Laura Jackson, Jie Lu, Brian P Frodey, Atinuke Dosunmu-Ogunbi, Youjun Li, Marc Fromherz, Audry Kang, Lucas Santana-Santos, Panayiotis V Benos, Edward V Prochownik
Ribosomopathies comprise a heterogeneous group of hematologic and developmental disorders, often characterized by bone marrow failure, skeletal and other developmental abnormalities and cancer predisposition. They are associated with mutations and/or haplo-insufficiencies of ribosomal proteins (RPs) and inefficient ribosomal RNA (rRNA) processing. The resulting ribosomal stress induces the canonical p19ARF/Mdm2/p53 tumor suppressor pathway leading to proliferative arrest and/or apoptosis. It has been proposed that this pathway is then inactivated during subsequent neoplastic evolution...
2017: PloS One
https://www.readbyqxmd.com/read/28806258/recurrent-scapular-metastasis-from-hepatoblastoma-shown-on-fdg-pet-ct-and-f-dopa-pet-ct
#12
Bing Zhang, Qiao He, Xinchong Shi, Xiaoyan Wang, Xiangsong Zhang
We report the case of a 4-year-old girl with a biochemical relapse (plasma α-fetoprotein of 57,987.6 μg/L) after hepatoblastoma and extrahepatic metastases removal and adjuvant chemotherapy. Abdominal ultrasound, CT, and MRI failed to determine the site of recurrence. F-FDG PET/CT showed increased activity in the region of left scapula and adjacent soft tissue, which was incorrectly interpreted as the postoperative repair or inflammatory change. F-DOPA PET/CT showed increased activity and noticeable progressed lesion in the same place...
October 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28802572/effect-of-cadmium-on-uptake-of-iron-zinc-and-copper-and-mrna-expression-of-metallothioneins-in-hepg2-cells-in-vitro
#13
Sabine Sampels, Hana Kocour Kroupova, Pavla Linhartova
The intake of cadmium contaminated fish was mimicked by incubating human hepatoblastoma cells (Cell line HepG2) with a combination of different levels of cadmium (0-5μM) plus the n-3 fatty acids docosahexaenoic acid and eicosapentaenoic acid, which are typical for fish. Uptake of cadmium, iron, copper and zinc was measured by ICP-MS. In addition mRNA expression of two metallothioneins (mt1 g and mt1 m) was evaluated by real-time PCR. The obtained data shows that the presence of cadmium increases the uptake of iron and zinc into the HepG2 cells while the uptake of copper remains unaffected...
October 2017: Toxicology in Vitro: An International Journal Published in Association with BIBRA
https://www.readbyqxmd.com/read/28767567/a-4-and-a-half-years-old-boy-with-mesenchymal-hamartomas-in-the-left-lateral-lobe-of-the-liver-a-case-report-and-literature-review
#14
REVIEW
Wei Liao, Binhao Zhang, Wei Zhang, Lin Chen, Wanguang Zhang, Bixiang Zhang, Xiaoping Chen
RATIONALE: Mesenchymal hamartomas of the liver is one type of rare liver tumor. PATIENT CONCERNS: Mesenchymal hamartomas of the liver (MHL) is rarely reported in the left lobe of the liver in children who are more than 2 years old. It is difficult to distinguish it from liver lesions such as hepatoblastoma in children, and hepatocellular carcinoma and focal nodular hyperplasia in adults. In addition, it is hard to correctly diagnose it without pathological examination...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28755188/liver-cancer-in-tyrosinemia-type-1
#15
Willem G van Ginkel, Jan P Pennings, Francjan J van Spronsen
Hereditary Tyrosinemia type I (HT1) is clinically mainly characterised by severe liver disease. Most patients present in their first months of life with liver failure, but others can present later with issues of compensated cirrhosis, renal tubulopathy or acute intermittent porphyria. If patients survive the acute phase with liver failure or if they present later with compensated cirrhosis, they often develop hepatocellular carcinoma early but also later in life. The course of the disease changed after the introduction of 2-(2 nitro-4-3 trifluoro-methylbenzoyl)-1, 3-cyclohexanedione (NTBC), which blocks the tyrosine degradation pathway at an earlier step...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28728220/-diagnostic-utility-of-tyrosine-hydroxylase-in-peripheral-neuroblastic-tumors
#16
N Zhang, N Liu, L J He
Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as primitive neuroectodermal tumor (PNET), rhabdomyosarcoma, lymphoma, nephroblastoma, as well as other more common tumors (medulloblastoma, hepatoblastoma, pleuropulmonary blastoma, renal clear cell sarcoma, Langerhans cell histiocytosis, lipoblatoma etc)...
July 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28727971/congenital-extrahepatic-portosystemic-shunt-abernethy-malformation-type-ib-with-associated-hepatocellular-carcinoma-case-report-and-literature-review
#17
Mark Benedict, Manuel Rodriguez-Davalos, Sukru Emre, Zenta Walther, Raffaella Morotti
Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra- and intrahepatic shunts. Two shunts have been defined: Type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28714114/split-liver-transplantation-for-retroperitoneal-immature-teratoma-masquerading-as-hepatoblastoma
#18
Dongkyu Oh, Nam-Joon Yi, Sanghee Song, Ok Kyung Kim, Suk Kyun Hong, Kyung Chul Yoon, Sung Woo Ahn, Hyo-Sin Kim, Hyeyoung Kim, Hyun-Young Kim, Hyoung Jin Kang, Myungsu Lee, Kyoung Bun Lee, Kwang-Woong Lee, Kyung Suk Suh
Extragonadal teratoma originating from the retroperitoneum represents less than 5% of all teratomas and accounts for less than 10% of all pediatric retroperitoneal neoplasms. To date, there has been no report of teratoma managed with LT. This study reports an infant aged 3 months with retroperitoneal immature teratoma involving the hepatic hilum, refractory to chemotherapy and treated with LT. The patient was referred to our hospital for management of a growing abdominal mass. Histopathology of a fine needle biopsy of the lesion suggested the possibility of a hepatoblastoma with teratoid features...
July 16, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28692553/simultaneous-presentation-of-wilms-tumor-and-immature-ovarian-teratoma-in-beckwith-wiedemann-syndrome
#19
Jason C White, Jinglan Liu, Akash Nahar
The Beckwith-Wiedemann syndrome is a cancer predisposition syndrome characterized by a predilection to embryonal tumor growth, especially Wilms tumor, adrenocortical carcinomas, and hepatoblastomas. Genetic analysis of patients has revealed a link to the imprinted domain of the 11p15.5 chromosome and methylation status of the H19 locus and Igf-2. These genes have also been studied in other cancers, including ovarian teratomas. Our case is a patient with a simultaneous presentation of a Wilms tumor and immature ovarian teratoma and subsequently diagnosed with Beckwith-Wiedemann syndrome, which has not been previously described...
July 7, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28682067/expanding-the-antiviral-spectrum-of-3-fluoro-2-phosphonomethoxy-propyl-acyclic-nucleoside-phosphonates-diamyl-aspartate-amidate-prodrugs
#20
Min Luo, Elisabetta Groaz, Graciela Andrei, Robert Snoeck, Raj Kalkeri, Roger G Ptak, Tracy Hartman, Robert W Buckheit, Dominique Schols, Steven De Jonghe, Piet Herdewijn
Acyclic nucleosides containing a 3-fluoro-2-(phosphonomethoxy)propyl (FPMP) side chain are known to be moderately potent antihuman immunodeficiency virus (HIV) agents, while being completely devoid of antiviral activity against a wide range of DNA viruses. The derivatization of the phosphonic acid functionality of FPMPs with a diamyl aspartate phenoxyamidate group led to a novel generation of compounds that not only demonstrate drastically improved antiretroviral potency but also are characterized by an expanded spectrum of activity that also covers hepatitis B and herpes viruses...
July 27, 2017: Journal of Medicinal Chemistry
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