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https://www.readbyqxmd.com/read/28820908/ribosomopathy-like-properties-of-murine-and-human-cancers
#1
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
#2
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...
August 12, 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
#3
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...
August 10, 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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28674120/surveillance-recommendations-for-children-with-overgrowth-syndromes-and-predisposition-to-wilms-tumors-and-hepatoblastoma
#11
REVIEW
Jennifer M Kalish, Leslie Doros, Lee J Helman, Raoul C Hennekam, Roland P Kuiper, Saskia M Maas, Eamonn R Maher, Kim E Nichols, Sharon E Plon, Christopher C Porter, Surya Rednam, Kris Ann P Schultz, Lisa J States, Gail E Tomlinson, Kristin Zelley, Todd E Druley
A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. Here, we outline these rare syndromes with at least a 1% risk to develop these tumors and recommend uniform tumor screening recommendations for North America. Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. For HB, we recommend screening with full abdominal ultrasound and alpha-fetoprotein serum measurements every 3 months from birth (or the time of diagnosis) through the fourth birthday...
July 1, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28674119/cancer-screening-recommendations-and-clinical-management-of-inherited-gastrointestinal-cancer-syndromes-in-childhood
#12
REVIEW
Maria Isabel Achatz, Christopher C Porter, Laurence Brugières, Harriet Druker, Thierry Frebourg, William D Foulkes, Christian P Kratz, Roland P Kuiper, Jordan R Hansford, Hector Salvador Hernandez, Katherine L Nathanson, Wendy K Kohlmann, Leslie Doros, Kenan Onel, Kami Wolfe Schneider, Sarah R Scollon, Uri Tabori, Gail E Tomlinson, D Gareth R Evans, Sharon E Plon
Hereditary gastrointestinal cancer predisposition syndromes have been well characterized, but management strategies and surveillance remain a major challenge, especially in childhood. In October 2016, the American Association for Cancer Research organized the AACR Childhood Cancer Predisposition Workshop in which international experts in care of children with a hereditary risk of cancer met to define surveillance strategies and management of children with cancer predisposition syndromes. In this article, we review the current literature in polyposis syndromes that can be diagnosed in childhood and may be associated with an increased incidence of gastrointestinal neoplasms and other cancer types...
July 1, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28660626/hepatocellular-malignant-neoplasm-nos-a-clinicopathologic-study-of-11-cases-from-a-single-institution
#13
Shengmei Zhou, Rajkumar Venkatramani, Shveta Gupta, Kasper Wang, Larry Wang, Leo Mascarenhas
AIMS: The primary aim of this study is to characterize hepatocellular malignant neoplasm, NOS (HEMNOS), a new provisional entity describing a subset of paediatric hepatocellular tumours, which have histological features of neither typical hepatoblastoma (HB) nor hepatocellular carcinoma (HCC). METHODS AND RESULTS: The clinicopathological features of 11 patients with HEMNOS were analyzed retrospectively. The median age and serum alpha-fetoprotein level at diagnosis was 7 years and 182,000 ng/mL respectively...
June 28, 2017: Histopathology
https://www.readbyqxmd.com/read/28651228/ante-situm-liver-resection-with-inferior-vena-cava-replacement-under-hypothermic-cardiopolmunary-bypass-for-hepatoblastoma-report-of-a-case-and-review-of-the-literature
#14
Roberta Angelico, Annalisa Passariello, Michele Pilato, Tommaso Cozzolino, Marcello Piazza, Roberto Miraglia, Paolo D'Angelo, Mariella Capasso, Maria Cristina Saffioti, Daniele Alberti, Marco Spada
INTRODUCTION: Hepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear. PRESENTATION OF CASE: A 11-months-old boy was referred for a right hepatic lobe mass(90×78mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28638817/a-challenging-case-of-hepatoblastoma-concomitant-with-autosomal-recessive-polycystic-kidney-disease-and-caroli-syndrome-review-of-the-literature
#15
Nevil Kadakia, Steven J Lobritto, Nadia Ovchinsky, Helen E Remotti, Darrell J Yamashiro, Jean C Emond, Mercedes Martinez
We report a rare case of an 18-month-old female with autosomal recessive polycystic kidney disease, Caroli syndrome, and pure fetal type hepatoblastoma. The liver tumor was surgically resected with no chemotherapy given. Now 9 years post resection she demonstrates no local or distant recurrence and stable renal function.
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28631020/utility-of-pas-and-%C3%AE-catenin-staining-in-histological-categorisation-and-prediction-of-prognosis-of-hepatoblastomas
#16
Goutam Bera, Ram Narayan Das, Paromita Roy, Ranajoy Ghosh, Nelofar Islam, Prafulla Kumar Mishra, Uttara Chatterjee
AIMS: The aim of this study was to assess the usefulness of PAS, β-catenin and Ki-67 in categorising hepatoblastomas (HBs) and their significance in predicting prognosis. In addition, we have also documented the various chemotherapy induced histologic changes in HBs. METHOD: In this retrospective observational study of 29 cases of hepatoblastomas, 27 cases were considered for statistical analysis, excluding two cases of diagnostic core biopsies. All clinicopathological parameters and follow up data were collected...
June 19, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28624605/transplacental-exposure-to-environmental-carcinogens-association-with-childhood-cancer-risks-and-the-role-of-modulating-factors
#17
REVIEW
A Fucic, V Guszak, A Mantovani
Biological responses to carcinogens from environmental exposure during adulthood are modulated over years or decades. Conversely, during transplacental exposure, the effects on the human foetus change within weeks, intertwining with developmental mechanisms: even short periods of transplacental exposure may be imprinted in the organism for a lifetime. The pathways leading to childhood and juvenile cancers, such as leukaemias, neuroblastoma/brain tumours, hepatoblastoma, and Willm's tumor involve prenatally-induced genomic, epigenomic and/or non-genomic effects caused by xenobiotics...
June 14, 2017: Reproductive Toxicology
https://www.readbyqxmd.com/read/28620649/hepatoblastoma-transplant-versus-resection-experience-in-a-latin-american-transplant-center
#18
Luis A Caicedo, Angie Sabogal, Oscar Serrano, Jorge I Villegas, Verónica Botero, María T Agudelo, Viviana Lotero, Diana Dávalos, Eliana Manzi, Ana M Aristizabal, Catalina Gomez, Gabriel J Echeverri
BACKGROUND: Hepatoblastoma is the most common primary malignant liver tumor in children and is usually diagnosed during the first 3 years of life. Overall survival has increased 50% due to chemotherapeutic schemes, expertise surgery centers, and liver transplantation. METHODS: A retrospective collection of data was performed from pediatric patients with diagnosis of hepatoblastoma. Variables included demographic, diagnostic tools and histological classification; chemotherapy and surgical treatment; and outcomes and patient survival...
June 2017: Transplantation Direct
https://www.readbyqxmd.com/read/28617762/racial-disparities-and-sociodemographic-differences-in-incidence-and-survival-among-pediatric-patients-in-the-united-states-with-primary-liver-cancer-a-surveillance-epidemiology-and-end-results-seer-population-study
#19
Felix Nautsch, Johannes M Ludwig, Minzhi Xing, Kevin M Johnson, Hyun S Kim
BACKGROUND: Primary liver cancer, including Hepatoblastoma (HB) and hepatocellular carcinoma (HCC), in pediatric populations is often fatal. The outcomes are poor despite universal health care access in pediatric patients. AIM: We investigated the sociodemographic factors affecting outcomes in pediatric patients with primary liver cancer. MATERIALS AND METHODS: This is a large population database study of Surveillance, Epidemiology, and End Results cancer registry data from 1973 to 2011...
June 14, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28602756/antrodia-cinnamomea-sensitizes-radio-chemo-therapy-of-cancer-stem-like-cells-by-modulating-microrna-expression
#20
Yu-Kai Su, Ping-Hsiao Shih, Wei-Hwa Lee, Oluwaseun Adebayo Bamodu, Alexander T H Wu, Chun-Chih Huang, Yew-Min Tzeng, Michael Hsiao, Chi-Tai Yeh, Chien-Min Lin
ETHNOPHARMACOLOGICAL RELEVANCE: The discovery of many tissue-specific cancer stem cells (CSCs) continues to attract scientific attention. These CSCs are considered to be associated with chemo- and radio-resistance, and consequently, failure of conventional anticancer therapies. The recent demonstration of several microRNAs as enhancers of tumorigenicity via modulation of epithelial-mesenchymal transition and cancer stemness, makes them putative novel therapeutic target in oncology. Antrodia cinnamomea is a Chinese traditional medicine with several biological functions including anti-inflammation, antioxidant, and cancer prevention...
June 8, 2017: Journal of Ethnopharmacology
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