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https://www.readbyqxmd.com/read/27895749/a-single-center-retrospective-study-of-pediatric-hepatoblastoma
#1
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/27884679/risk-stratified-staging-in-paediatric-hepatoblastoma-a-unified-analysis-from-the-children-s-hepatic-tumors-international-collaboration
#2
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/27845967/what-is-new-in-pediatric-surgical-oncology
#3
Roshni Dasgupta, Deborah Billmire, Jennifer H Aldrink, Rebecka L Meyers
PURPOSE OF REVIEW: As pediatric oncology has become more complex, designing and maintaining pediatric surgical protocols require greater expertise. The primary purpose of this review is to summarize the changes in protocols and new study findings, which have changed surgical practice for children with solid tumors. RECENT FINDINGS: The most common solid tumors treated by surgery are reviewed. There are new methods of surgical techniques such as the use of sentinel lymph node techniques for the assessment of lymph nodes in pediatric sarcoma...
November 14, 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27825058/huge-focal-nodular-hyperplasia-presenting-in-a-6-year-old-child-a-case-presentation
#4
Lin Zhuang, Chuangye Ni, Wenbing Din, Feng Zhang, Yi Zhuang, Yawei Sun, Dong Xi
INTRODUCTION: Focal nodular hyperplasia (FNH) is a benign lesion of the liver which is usually found in healthy adults, however, FNH is rare in children, and comprises only 2% of all pediatric liver tumors. Herein, we report the case of a 6-year-old child (male) with a huge FNH which size is more than 10cm. This could be the biggest FNH among all children's FNH cases ever reported. CASE PRESENTATION: A 6-year-old boy was found a hepatic space-occupying lesion two years ago...
October 27, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27815224/minimal-hepatic-encephalopathy-in-children-with-chronic-liver-disease-prevalence-pathogenesis-and-magnetic-resonance-based-diagnosis
#5
Anshu Srivastava, Saurabh Chaturvedi, Rakesh Kumar Gupta, Rohan Malik, Amrita Mathias, Naranamangalam R Jagannathan, Sunil Jain, Chandra Mani Pandey, Surender Kumar Yachha, Ram Kishor Singh Rathore
BACKGROUND AND AIMS: Data on minimal hepatic encephalopathy (MHE) in children is scanty. We evaluated children with chronic liver disease (CLD) to determine prevalence of MHE, its correlation with changes in brain metabolites by magnetic resonance spectroscopy ((1)HMRS), diffusion tensor imaging (DTI) derived metrics, blood ammonia (BA) and inflammatory cytokines and the accuracy of MR based investigations for diagnosis METHODS: 67(38 boys; age 13[7-18] years) CLD and 37 healthy children were evaluated with neuropsychological tests (NPT), BA, interleukin-6 [IL6], tumor necrosis factor alpha [TNFα], magnetic resonance imaging (MRI),(1)HMRS and DTI...
November 1, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27783314/space-occupying-lesions-in-the-liver
#6
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/27781381/characteristics-and-outcomes-in-children-with-undifferentiated-embryonal-sarcoma-of-the-liver-a-report-from-the-national-cancer-database
#7
Yan Shi, Yesenia Rojas, Wei Zhang, Elizabeth A Beierle, John J Doski, Melanie Goldfarb, Adam B Goldin, Kenneth W Gow, Monica Langer, Rebecka L Meyers, Jed G Nuchtern, Sanjeev A Vasudevan
OBJECTIVE: To examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi-institutional database. SUMMARY BACKGROUND DATA: UESL is a rare disease (incidence is one per million). Therefore, the current literature is mostly limited to small case series. METHODS: The National Cancer Database was queried for primary UESL diagnosed between 1998 and 2012. RESULTS: A total of 103 patients (<18 years) were identified...
October 26, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27781375/multidisciplinary-management-of-hepatoblastoma-in-children-experience-from-a-developing-country
#8
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
#9
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/27775293/-thoracoscopic-resection-of-lung-metastases-guided-by-hook-wire
#10
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/27775284/-congenital-portosistemic-shunt-importance-of-early-treatment
#11
N Vicente, M Pérez, R Gander, A Segarra, C Leganés, J Bueno
AIM: Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition. PATIENTS AND METHODS: Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007...
April 15, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27764132/can-we-spare-the-pancreas-and-other-abdominal-organs-at-risk-a-comparison-of-conformal-radiotherapy-helical-tomotherapy-and-proton-beam-therapy-in-pediatric-irradiation
#12
Emmanuel Jouglar, Antoine Wagner, Grégory Delpon, Loïc Campion, Philippe Meingan, Valérie Bernier, Charlotte Demoor-Goldschmidt, Marc-André Mahé, Thomas Lacornerie, Stéphane Supiot
OBJECTIVES: Late abdominal irradiation toxicity during childhood included renal damage, hepatic toxicity and secondary diabetes mellitus. We compared the potential of conformal radiotherapy (CRT), helical tomotherapy (HT) and proton beam therapy (PBT) to spare the abdominal organs at risk (pancreas, kidneys and liver- OAR) in children undergoing abdominal irradiation. METHODS: We selected children with abdominal tumors who received more than 10 Gy to the abdomen...
2016: PloS One
https://www.readbyqxmd.com/read/27734029/hepatoblastoma-modeling-in-mice-places-nrf2-within-a-cancer-field-established-by-mutant-%C3%AE-catenin
#13
Sarah A Comerford, Elizabeth A Hinnant, Yidong Chen, Hima Bansal, Shawn Klapproth, Dinesh Rakheja, Milton J Finegold, Dolores Lopez-Terrada, Kathryn A O'Donnell, Gail E Tomlinson, Robert E Hammer
Aberrant wnt/β-catenin signaling and amplification/overexpression of Myc are associated with hepatoblastoma (HB), the most prevalent type of childhood liver cancer. To address their roles in the pathogenesis of HB, we generated mice in which Myc and mutant β-catenin were targeted to immature cells of the developing mouse liver. Perinatal coexpression of both genes promoted the preferential development of HBs over other tumor types in neonatal mice, all of which bore striking resemblance to their human counterparts...
October 6, 2016: JCI Insight
https://www.readbyqxmd.com/read/27723922/hepatocellular-carcinoma-referral-to-a-transplantation-unit
#14
Paloma Triana, Mariela Dore, Martha Muñoz Romo, Javier Jimenez Gomez, Alba Sánchez Galán, Francisco Hernandez, Ane M Andres Moreno, Jose Luis Encinas, Leopoldo Martinez, Manuel Lopez Santamaria
Aim Hepatocellular carcinoma (HCC), although being infrequent, is the second-most common primary hepatic malignancy in children, after hepatoblastoma (HB). The prognosis is very poor. We present our series of children with HCC referred to our transplant unit to be assessed as candidates for liver transplantation (LT). Methods A retrospective review of HCCs referred to our transplant unit in the past 20 years (1994-2015) was performed. Age at diagnosis, disease-free survival, location of recurrence, initial treatment, secondary treatment, and mortality were noted...
October 10, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27721967/multi-visceral-transplantation-in-a-21-year-old-man-with-prior-pancreatoblastoma
#15
R Girlanda, A Pozzi, C S Matsumoto, T M Fishbein
Organ transplantation in patients with prior malignancy increases the risk of tumor recurrence post-transplantation due to immunosuppression. Only two cases of liver transplantation have so far been reported in children with hepatic metastases from pancreatoblastoma, a rare malignant neoplasm originating from the epithelial exocrine cells of the pancreas. Herein, we describe a case of a successful multi-visceral transplant in a man with intestinal failure after surgical resection of pancreatoblastoma.
2016: International Journal of Organ Transplantation Medicine
https://www.readbyqxmd.com/read/27684838/uncommon-liver-tumors-case-report-and-literature-review
#16
Chia-Hung Wu, Nai-Chi Chiu, Yi-Chen Yeh, Yu Kuo, Sz-Shian Yu, Ching-Yao Weng, Chien-An Liu, Yi-Hong Chou, Yi-You Chiou
BACKGROUND: Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms. METHODS: From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27639430/prognostic-factors-and-survival-in-non-central-nervous-system-rhabdoid-tumors
#17
Benjamin A Farber, Neerav Shukla, Irene Isabel P Lim, Jennifer M Murphy, Michael P La Quaglia
INTRODUCTION: Non-central nervous system (non-CNS) rhabdoid tumors tend to present at a young age and have an extremely aggressive course, with dismal overall survival rates. Inactivation of the tumor suppressor gene SMARCB1 has been shown in rhabdoid tumors regardless of anatomic location, suggesting a common genetic basis. We retrospectively analyzed our institutional experience with non-CNS rhabdoid tumors to determine overall survival and prognostic variables. METHODS: We reviewed records of pediatric patients (age<22y) with non-CNS rhabdoid tumor at our institution between 1980 and 2014...
August 31, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27638340/zinc-and-zinc-containing-biomolecules-in-childhood-brain-tumors
#18
Jan Hrabeta, Tomas Eckschlager, Marie Stiborova, Zbynek Heger, Sona Krizkova, Vojtech Adam
Zinc ions are essential cofactors of a wide range of enzymes, transcription factors, and other regulatory proteins. Moreover, zinc is also involved in cellular signaling and enzymes inhibition. Zinc dysregulation, deficiency, over-supply, and imbalance in zinc ion transporters regulation are connected with various diseases including cancer. A zinc ion pool is maintained by two types of proteins: (i) zinc-binding proteins, which act as a buffer and intracellular donors of zinc and (ii) zinc transporters responsible for zinc fluxes into/from cells and organelles...
November 2016: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/27635950/connectivity-map-identifies-hdac-inhibition-as-a-treatment-option-of-high-risk-hepatoblastoma
#19
Alexander Beck, Corinna Eberherr, Michaela Hagemann, Stefano Cairo, Beate Häberle, Christian Vokuhl, Dietrich von Schweinitz, Roland Kappler
Hepatoblastoma (HB) is the most common liver tumor of childhood, usually occurring in children under the age of 3 y. The prognosis of patients presenting with distant metastasis, vascular invasion and advanced tumor stages remains poor and children that do survive often face severe late effects from the aggressive chemotherapy regimen. To identify potential new therapeutics for high risk HB we used a 1,000-gene expression signature as input for a Connectivity Map (CMap) analysis, which predicted histone deacetylase (HDAC) inhibitors as a promising therapy option...
September 16, 2016: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/27621753/undifferentiated-embryonal-sarcoma-of-the-liver-in-a-young-female-treatment-with-portal-vein-embolization-and-liver-trisectonectomy
#20
Dimitrios E Giakoustidis, Athanasios A Gargavanis, Evangelia D Katsiki, Nikolaos T Salveridis, Nikolaos A Antoniadis, Vasileios Papanikolaou
Undifferentiated Embryonal Sarcoma of the Liver (UESL) is a tumor highly malignant, of mesenchymal origin. It is a rare finding in adults, though less rare in children. The strategy to be followed and the therapeutic targets to be reached for this tumor, in adult cases, remain ambiguous and controversial. Herein we report the case of a 29 year old female patient with a massive UESL and we describe our therapeutic approach. A 29 year-old female patient was referred to our center with severe intermittent epigastric pain and fever due to a voluminous liver tumor: Needle biopsy was of no specific findings and surgical excision was decided...
August 2016: Korean Journal of Hepato-biliary-pancreatic Surgery
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