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Children hepatic tumor

María José Garcés Hernández, Miguel Klünder, Nayely Garibay Nieto, Juan Carlos López Alvarenga, Jenny Vilchis Gil, Samuel Flores Huerta, Rosa Quispe Siccha, Joselin Hernandez
OBJECTIVE: Visceral adiposity index (VAI) is a mathematical model associated with cardiometabolic risk in adults, but studies on children failed to support this association. Our group has proposed a pediatric VAI model using pediatric ranges, but it has not yet been evaluated and needs further adjustments. The objective of this study was to further adjust the proposed pediatric VAI by age, creating a new pediatric metabolic index (PMI), and assess the correlation of the PMI with insulin resistance indexes and hepatic enzymes...
March 2018: Endocrine Practice
Alexandra Okihiro, Rachana Hasija, Lillia Fung, Bonnie Cameron, Brian M Feldman, Ronald Laxer, Rayfel Schneider, Earl Silverman, Lynn Spiegel, Rae S M Yeung, Shirley M L Tse
BACKGROUND: Anti-TNF (Tumor necrosis factor) therapy is effective in treating pediatric patients with refractory rheumatic disease. There is however a concern that anti-TNF usage may increase the risk of malignancy. Reports on specific types of malignancy in this patient population have been emerging over the past decade, but there is a need for additional malignancy reports, as these events are rare. Therefore, a retrospective chart review was performed on the biologic database of pediatric rheumatology patients at The Hospital for Sick Children (SickKids) from 1997 to 2013 for neoplasms, patient demographic information and rheumatologic treatment course...
March 14, 2018: Pediatric Rheumatology Online Journal
Revathi Rajagopal, Marianne Phillips, Nicholas G Gottardo
The initial signs of hepatic sinusoidal obstruction syndrome (HSOS) can be challenging to recognize in children, especially outside the hematopoietic stem cell transplantation setting. To assist clinicians to promptly identify HSOS, the European Society for Blood and Marrow Transplantation has proposed pediatric HSOS diagnostic criteria which emphasize unexplained consumptive and transfusion-refractory thrombocytopenia. To highlight the importance of these "bellwether" early signs of HSOS and the efficacy of pre-emptive treatment with defibrotide, we describe the case of a child with a right 11th rib primitive neuroectodermal tumor who developed HSOS following focal radiotherapy and actinomycin-D treatment...
March 14, 2018: Pediatric Blood & Cancer
Dehong Huang, Lei Jin, Zhengkang Li, Ji Wu, Ni Zhang, Dianrong Zhou, Xiaorong Ni, Tieying Hou
Hepatoblastoma (HB) is the most common malignant liver tumor in children. DNA and DNA-associated processes are one of the most important targets of chemotherapeutic agents. Isoorientin (Iso), a natural flavonoid compound, can be extracted from several plant species. The effects of Iso and its molecular mechanisms on hepatic malignancies remain unclear. Herein, the anti-tumor effects of Iso in HB and its underlying mechanisms were explored. We found that Iso significantly inhibited the proliferation of HB cells both in vitro and in vivo...
March 7, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Linda Kievit, Pia Kræmer, Stephen Hamilton-Dutoit, Henning Grønbæk
A 37-year-old male, who at the age of 8 years had been treated for right-sided Wilms' tumor with nephrectomy, radiotherapy, and chemotherapy, presented with noncirrhotic portal hypertension (NCPH), grade 2 esophageal varices, and ascites. A CT scan demonstrated hypoplasia of liver segments 2 and 3. A liver biopsy showed portal tract fibrosis without cirrhosis, with histological features of NCPH. Liver vein catheterization showed a normal portal pressure gradient of 5 mm Hg while spleen to hepatic vein pressure was 29 mm Hg...
January 2018: Case Reports in Gastroenterology
Saravana Kumar Kailasam Mani, Ourania Andrisani
Chronic Hepatitis B Virus (HBV) infection is linked to hepatocellular carcinoma (HCC) pathogenesis. Despite the availability of a HBV vaccine, current treatments for HCC are inadequate. Globally, 257 million people are chronic HBV carriers, and children born from HBV-infected mothers become chronic carriers, destined to develop liver cancer. Thus, new therapeutic approaches are needed to target essential pathways involved in HCC pathogenesis. Accumulating evidence supports existence of hepatic cancer stem cells (hCSCs), which contribute to chemotherapy resistance and cancer recurrence after treatment or surgery...
March 2, 2018: Genes
Joshua Casaos, Sakibul Huq, Tarik Lott, Raphael Felder, John Choi, Noah Gorelick, Michael Peters, Yuanxuan Xia, Russell Maxwell, Tianna Zhao, Chenchen Ji, Thomas Simon, Julie Sesen, Sarah J Scotland, Richard E Kast, Jeffrey Rubens, Eric Raabe, Charles G Eberhart, Eric M Jackson, Henry Brem, Betty Tyler, Nicolas Skuli
Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive, malignant tumors and are the most common malignant brain tumor in children under 6 months of age. Currently, there is no standard treatment for AT/RT. Recent studies have reported potential anti-tumoral properties of ribavirin, a guanosine analog and anti-viral molecule approved by the Food and Drug Administration for treatment of hepatitis C. We previously demonstrated that ribavirin inhibited glioma cell growth in vitro and in vivo . Based on these results and the fact that no pre-clinical model of ribavirin in AT/RT exists, we decided to investigate the effect of ribavirin on several human AT/RT cell lines (BT12, BT16, and BT37) both in vitro and in vivo ...
January 30, 2018: Oncotarget
Alexander J Towbin, Rebecka L Meyers, Helen Woodley, Osamu Miyazaki, Christopher B Weldon, Bruce Morland, Eiso Hiyama, Piotr Czauderna, Derek J Roebuck, Greg M Tiao
Imaging is crucial in the assessment of children with a primary hepatic malignancy. Since its inception in 1992, the PRETEXT (PRE-Treatment EXTent of tumor) system has become the primary method of risk stratification for hepatoblastoma and pediatric hepatocellular carcinoma in numerous cooperative group trials across the world. The PRETEXT system is made of two components: the PRETEXT group and the annotation factors. The PRETEXT group describes the extent of tumor within the liver while the annotation factors help to describe associated features such as vascular involvement (either portal vein or hepatic vein/inferior vena cava), extrahepatic disease, multifocality, tumor rupture and metastatic disease (to both the lungs and lymph nodes)...
February 9, 2018: Pediatric Radiology
C Matthew Hawkins, Alexander J Towbin, Derek J Roebuck, Eric J Monroe, Anne E Gill, Avnesh S Thakor, Richard B Towbin, Anne Marie Cahill, Matthew P Lungren
Hepatoblastoma and hepatocellular carcinoma (HCC) are the most common pediatric liver malignancies, with hepatoblastoma occurring more commonly in younger children and HCC occurring more commonly in older children and adolescents. Although surgical resection (including transplant when necessary) and systemic chemotherapy have improved overall survival rate for hepatoblastoma to approximately 80% from 30%, a number of children with this tumor type are not eligible for operative treatment. In contradistinction, pediatric HCC continues to carry a dismal prognosis with an overall 5-year survival rate of 30%...
February 2, 2018: Pediatric Radiology
Nicolas Berte, Alya Filfilan, Laurence Mainard, Ludovic Mansuy, Jean-Louis Lemelle
Infantile hepatic hemangioma (IHH) and mesenchymal hamartoma (MH) form the first and second most common benign hepatic tumors in children. In this case report, we present a newborn child in whom a growing hepatic mass was discovered at the age of 7 days. She suffered also from anemia, respiratory and renal failure. No signs of heart disease or cutaneous lesions were detected. Alpha-fetoprotein was elevated for her age (3562.2 ng/ml). Imaging studies showed multifocal large cystic lesions associated with heterogeneous small solid lesions with arterial enhancement of the liver...
January 2018: Journal of Surgical Case Reports
BinBin Zhang, GongBao Liu, XiangQi Liu, Shan Zheng, Kuiran Dong, Rui Dong
OBJECTIVES: Hepatoblastoma (HB) is the most common pediatric liver malignancy, typically affecting children within the first 4 years of life. However, only a few validated blood biomarkers are used in clinical evaluation. The current study explored the clinical application and significance of D-dimer levels in patients with HB. METHOD: Forty-four patients with HB were included in this retrospective study. D-dimer and plasma fibrinogen levels were examined, and their correlation with other clinical features was analyzed...
November 2017: Medicine (Baltimore)
Piotr Czauderna, Hanna Garnier
Introduction: Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children. Its increasing survival rate is related to the progress in modern imaging, surgical techniques, and new chemotherapy regimens. Clinical approach: One of the past achievements was the development of the pretreatment extension of disease (PRETEXT) system. Gradually, the HB therapeutic approach has become more individualized with better stratification of patients. Controversies: These include the need for preoperative chemotherapy and its optimal duration; intensity of preoperative chemotherapy required for locally advanced cases (PRETEXT 4); optimal surgical treatment for locally advanced tumors: aggressive hepatic resections versus liver transplantation; the role of postoperative chemotherapy in the post-transplant setting; the timing and role of metastasectomy in patients with disseminated disease who undergo partial liver resection; and the prognostic significance of several HB pathology variants...
2018: F1000Research
Matthew P Lungren, Alexander J Towbin, Derek J Roebuck, Eric J Monroe, Anne E Gill, Avnesh Thakor, Richard B Towbin, Anne Marie Cahill, C Matthew Hawkins
Primary liver malignancies are rare in children. Hepatoblastoma and hepatocellular carcinoma (HCC) together represent the overwhelming majority of cases. Overall survival of hepatoblastoma approaches 80% with multimodal treatment approaches that include chemotherapy, surgery and transplantation. However, there remains a subset of children with hepatoblastoma in whom resection or transplantation is not possible. The 5-year survival for children diagnosed with HCC is less than 30% and remains a significant therapeutic challenge...
January 23, 2018: Pediatric Radiology
Zuhal Keskin Yildirim, Mustafa Buyukavci
BACKGROUND: Cancer survival rates and longevity of patients after therapy have significantly improved during the last few decades. Therefore, lasting protection against infections should be provided. PROCEDURE: A total of 162 children diagnosed with acute lymphoblastic leukemia, acute myelogenous leukemia, solid tumors, non-Hodgkin lymphoma, and Hodgkin lymphoma were enrolled in the study. Antibody levels against hepatitis B virus was assessed both at the time of diagnosis and within 6 months after completion of chemotherapy...
January 5, 2018: Journal of Pediatric Hematology/oncology
Amanda Ricciuto, Binita M Kamath, Thomas D Walters, Karen Frost, Nicholas Carman, Peter C Church, Simon C Ling, Anne M Griffiths
OBJECTIVES: To evaluate a large anti-tumor necrosis factor (TNF)-treated pediatric inflammatory bowel disease cohort for drug-induced liver injury (DILI) following presentation of an index case with suspected DILI with autoimmune features after infliximab exposure. To characterize the incidence, natural history, and risk factors for liver enzyme elevation with anti-TNF use. STUDY DESIGN: We reviewed the index case and performed a retrospective cohort study of 659 children receiving anti-TNF therapy between 2000 and 2015 at a tertiary pediatric inflammatory bowel disease center...
December 21, 2017: Journal of Pediatrics
Michael Sirignano, Jonathan R Dillman, Brian D Weiss, Charles T Quinn, Bin Zhang, Weizhe Su, Andrew T Trout
BACKGROUND: Reticuloendothelial system MRI signal hypointensity is common in pediatric oncology patients with solid abdominal tumors. OBJECTIVE: To assess changes in liver, spleen and bone marrow T2-weighted MRI signal intensity over time and their relationship to blood transfusion history in children with solid abdominal tumors. MATERIALS AND METHODS: In this retrospective study we measured liver, spleen and bone marrow signal intensity on axial T2-weighted MR images obtained December 2009 through February 2016 in children with hepatoblastoma, neuroblastoma, ganglioneuroblastoma and Wilms tumor...
December 19, 2017: Pediatric Radiology
Marina Weber Pasa, Rafael Selbach Scheffel, André Borsatto Zanella, Ana Luiza Maia, José Miguel Dora
Objectives: To provide a comprehensive description of consumptive hypothyroidism syndrome (CHS), a severe form of hypothyroidism that occurs due to the high expression levels of thyroid hormone inactivation enzyme type 3 deiodinase (D3) in tumors. Study Design: Case report and systematic review. Results: A 7-month-old girl with a diagnosis of massive hepatic hemangioendotheliomas was treated with high doses of thyroid hormones and tumor-directed chemotherapy with vincristine...
November 2017: European Thyroid Journal
Alexander J Towbin, Fernanda D C Braojos Braga, Bin Zhang, James I Geller, Greg M Tiao, Daniel J Podberesky
BACKGROUND: Hepatoblastoma is the most common primary hepatic malignancy in children. We have recently noticed an increased incidence of unsuspected fractures in children with newly diagnosed hepatoblastoma. This association has been suggested in the past, but the incidence and pathophysiology remain uncertain. OBJECTIVE: To define the incidence and imaging features of fractures in children with newly diagnosed hepatoblastoma. MATERIALS AND METHODS: We searched the oncology database and the radiology picture archiving and communication system of our large tertiary care children's hospital between January 2000 and August 2013 for all patients who presented to our institution with newly diagnosed hepatoblastoma...
December 8, 2017: Pediatric Radiology
Emma C Hamilton, Julius Balogh, Duc T Nguyen, Edward A Graviss, Andras A Heczey, Mary T Austin
BACKGROUND/PURPOSE: The purpose of this study was to determine factors associated with patient and graft survival following orthotopic liver transplantation (OLT) in children and adolescents with primary hepatic malignancies. METHODS: The United Network for Organ Sharing (UNOS) database was queried for all patients <18years old who received an OLT with a primary malignant liver tumor between 1987 and 2012 (n=544). Five-year patient and graft survival were determined using Kaplan-Meier methodology, and independent predictors of survival were determined using multivariate Cox proportional hazards model...
October 12, 2017: Journal of Pediatric Surgery
Luisa Ferrero, Riccardo Guanà, Giulia Carbonaro, Maria Grazia Cortese, Luca Lonati, Elisabetta Teruzzi, Jurgen Schleef
Benign intra-abdominal cystic masses in infancy are fairly uncommon and their etiopathogenesis, histology and clinical presentation differ significantly. Our aim is to report our experience in their treatment in order to discuss the best diagnostic and treatment modality. The medical records of 5 children (2M, 3F) with cystic intraabdominal masses referred to our hospital between November 2012 and September 2016, were retrospectively reviewed. All patients underwent open surgery and subsequent histopathologic analysis...
October 6, 2017: Pediatric Reports
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