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https://www.readbyqxmd.com/read/27884679/risk-stratified-staging-in-paediatric-hepatoblastoma-a-unified-analysis-from-the-children-s-hepatic-tumors-international-collaboration
#1
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/27825058/huge-focal-nodular-hyperplasia-presenting-in-a-6-year-old-child-a-case-presentation
#2
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
#3
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
#4
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/27775819/genomic-analysis-of-hepatoblastoma-identifies-distinct-molecular-and-prognostic-subgroups
#5
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/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
#6
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
#7
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
#8
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
#9
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
#10
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/27610342/history-of-the-infantile-hepatic-hemangioma-from-imaging-to-generating-a-differential-diagnosis
#11
REVIEW
Maria Gnarra, Gerald Behr, Alison Kitajewski, June K Wu, Sudha A Anupindi, Carrie J Shawber, Nick Zavras, Dimitrios Schizas, Chris Salakos, Konstantinos P Economopoulos
We aim to provide an up-to-date summary of infantile hepatic hemangioma (IHH) and its misnomers and to dialectically present the differential diagnosis of these rare entities of the liver. Eligible peer-reviewed articles on hepatic infantile hemangiomas, published between 2000 and 2015, were reviewed for this study. IHH is the most common hepatic vascular tumor in children. Once a liver mass is identified in an infant, the differential diagnosis ranges from vascular malformations to benign and malignant tumors including mesenchymal hamartoma, hepatoblastoma, metastatic neuroblastoma, so careful physical examination, imaging studies, and, if indicated, tumor markers and biopsy, are of pivotal importance to ascertain the correct diagnosis...
August 8, 2016: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/27605415/yap-subcellular-localization-and-hippo-pathway-transcriptome-analysis-in-pediatric-hepatocellular-carcinoma
#12
Michael J LaQuaglia, James L Grijalva, Kaly A Mueller, Antonio R Perez-Atayde, Heung Bae Kim, Ghazaleh Sadri-Vakili, Khashayar Vakili
Pediatric hepatocellular carcinoma (HCC) is a rare tumor which is associated with an extremely high mortality rate due to lack of effective chemotherapy. Recently, the Hippo pathway and its transcriptional co-activator Yes-associated protein (YAP) have been shown to play a role in hepatocyte proliferation and development of HCC in animal models. Therefore, we sought to examine the activity of YAP and the expression of Hippo pathway components in tumor and non-neoplastic liver tissue from 7 pediatric patients with moderately differentiated HCC...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27566042/hepatoblastoma-of-the-adult-a-systematic-review-of-the-literature
#13
Andrea Celotti, Giuseppe D'Amico, Marco Ceresoli, Matteo Tomasoni, Stefano Raimondo, Paolo Baggi, Gian Luca Baiocchi
Hepatoblastoma is the most common malignant liver tumor in children. On the other hand in the adult HB is very rare and characterized by unfavorable prognosis. A review of the entire literature was performed: 58 articles and 63 cases of HB were found. The patient's data were collected and analyzed. No correlation with hepatitis virus was found and AFP was elevated in most cases. Usually HB forms a large single mass in the liver and presents aggressive behavior, with local invasiveness and metastatic spread...
September 2016: Surgical Oncology
https://www.readbyqxmd.com/read/27530723/clinical-features-and-management-of-multifocal-hepatic-hemangiomas-in-children-a-retrospective-study
#14
Yi Ji, Siyuan Chen, Bo Xiang, Zhicheng Xu, Xiaoping Jiang, Xingtao Liu, Qi Wang, Guoyan Lu, Li Yang
Multifocal hepatic hemangioma (MHH) is a benign hepatic tumor that is commonly diagnosed in children with multiple cutaneous infantile hemangiomas (IHs). We present a review of all children with MHH at our institutions. Of the 42 patients, the median age at presentation of MHH was 2.5 months. Thirty-six (85.7%) patients had cutaneous IHs. Twelve (28.6%) patients were symptomatic at presentation. There was no significant association between the number of hepatic hemangiomas and the number of cutaneous IHs. Fourteen (33...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27509487/residential-proximity-to-environmental-pollution-sources-and-risk-of-rare-tumors-in-children
#15
Javier García-Pérez, Antonio Morales-Piga, Diana Gómez-Barroso, Ibon Tamayo-Uria, Elena Pardo Romaguera, Gonzalo López-Abente, Rebeca Ramis
BACKGROUND: Few epidemiologic studies have explored risk factors for rare tumors in children, and the role of environmental factors needs to be assessed. OBJECTIVES: To ascertain the effect of residential proximity to both industrial and urban areas on childhood cancer risk, taking industrial groups into account. METHODS: We conducted a population-based case-control study of five childhood cancers in Spain (retinoblastoma, hepatic tumors, soft tissue sarcomas, germ cell tumors, and other epithelial neoplasms/melanomas), including 557 incident cases from the Spanish Registry of Childhood Tumors (period 1996-2011), and 3342 controls individually matched by year of birth, sex, and region of residence...
August 7, 2016: Environmental Research
https://www.readbyqxmd.com/read/27501172/post-text-iii-and-iv-hepatoblastoma-extended-hepatic-resection-avoids-liver-transplantation-in-selected-cases
#16
Jörg Fuchs, Seher Cavdar, Gunnar Blumenstock, Martin Ebinger, Jürgen F Schäfer, Bence Sipos, Steven W Warmann
OBJECTIVE: To analyze the outcome of hepatoblastoma (HB) patients presenting with post treatment extent of disease (POST-TEXT) stages III and IV after neoadjuvant chemotherapy. BACKGROUND: Primary liver transplantation has been advocated as surgical treatment for children with HB involving 3 or 4 sectors at diagnosis. However, in some cases, tumors seem resectable after chemotherapy through aggressive use of nontransplant surgical procedures. METHODS: Data of 27 HB patients were reviewed, undergoing extended liver resection for POST-TEXT III or IV tumors after chemotherapy between 1992 and 2015...
August 5, 2016: Annals of Surgery
https://www.readbyqxmd.com/read/27432853/infectious-complications-with-the-use-of-biologic-response-modifiers-in-infants-and-children
#17
H Dele Davies
Biologic response modifiers (BRMs) are substances that interact with and modify the host immune system. BRMs that dampen the immune system are used to treat conditions such as juvenile idiopathic arthritis, psoriatic arthritis, or inflammatory bowel disease and often in combination with other immunosuppressive agents, such as methotrexate and corticosteroids. Cytokines that are targeted include tumor necrosis factor α; interleukins (ILs) 6, 12, and 23; and the receptors for IL-1α (IL-1A) and IL-1β (IL-1B) as well as other molecules...
August 2016: Pediatrics
https://www.readbyqxmd.com/read/27392999/pediatric-hepatocellular-carcinoma-in-a-developing-country-is-the-etiology-changing
#18
Kumar Palaniappan, Vibhor V Borkar, Mohamed Safwan, Mukul Vij, Sanjay Govil, Naresh Shanmugam, Mohamed Rela
HCC is the second most common malignant liver tumor of childhood. It typically affects children with a median age of 10-14 yr on background hepatitis B-related liver disease and is often metastatic or locally advanced at diagnosis. Children below the age of five yr typically constitute <10% of all children with HCC. In these children, it occurs on a background of congenital or metabolic liver disease. The records of all children with HCC who presented to our department over a six-yr study period were reviewed...
July 8, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27382436/fgf19-functions-as-autocrine-growth-factor-for-hepatoblastoma
#19
David J Elzi, Meihua Song, Barron Blackman, Susan T Weintraub, Dolores López-Terrada, Yidong Chen, Gail E Tomlinson, Yuzuru Shiio
Hepatoblastoma is the most common liver cancer in children, accounting for over 65% of all childhood liver malignancies. Hepatoblastoma is distinct from adult liver cancer in that it is not associated with hepatitis virus infection, cirrhosis, or other underlying liver pathology. The paucity of appropriate cell and animal models has been hampering the mechanistic understanding of hepatoblastoma pathogenesis. Consequently, there is no molecularly targeted therapy for hepatoblastoma. To gain insight into cytokine signaling in hepatoblastoma, we employed mass spectrometry to analyze the proteins secreted from Hep293TT hepatoblastoma cell line we established and identified the specific secretion of fibroblast growth factor 19 (FGF19), a growth factor for liver cells...
March 2016: Genes & Cancer
https://www.readbyqxmd.com/read/27325360/benefit-of-early-inflow-exclusion-during-living-donor-liver-transplantation-for-unresectable-hepatoblastoma
#20
Hajime Uchida, Akinari Fukuda, Kengo Sasaki, Yoshihiro Hirata, Takanobu Shigeta, Hiroyuki Kanazawa, Atsuko Nakazawa, Osamu Miyazaki, Shunsuke Nosaka, Vidyadhar Padmakar Mali, Seisuke Sakamoto, Mureo Kasahara
BACKGROUND: Hepatoblastoma (HB) is a highly malignant primary liver tumor in children. Although liver transplantation (LT) is an effective treatment for unresectable HB with good long-term outcomes, post-transplant survival is mainly affected by recurrence, despite adjuvant chemotherapy. Novel strategies are needed to improve the outcomes in patients undergoing LT for unresectable HB. PATIENTS AND METHODS: Twelve children received LT for unresectable HB. In 9 patients, we applied early exclusion of hepatic inflow (hepatic artery and portal vein) and creation of a temporary portocaval shunt during LT...
May 31, 2016: Journal of Pediatric Surgery
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