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https://www.readbyqxmd.com/read/28302003/liver-transplant-for-nonhepatocellular-carcinoma-malignancy
#1
Nihan Haberal Reyhan
Liver transplant is now an acceptable and effective treatment for specific nonhepatocellular malignancies. Worldwide, hilar cholangiocarcinoma accounts for 3% of all primary gastrointestinal malignancies and for 10% of primary hepatobiliary malignancies. For patients who have early-stage, unresectable cholangiocarcinoma, liver transplant preceded by neoadjuvant radiotherapy can result in tumor-free margins, accomplish a radical resection, and treat the underlying primary sclerosing cholangitis when present...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28288067/modified-associating-liver-partition-and-portal-vein-ligation-for-staged-hepatectomy-for-hepatoblastoma-in-a-small-infant-how-far-can-we-push-the-envelope
#2
Johnny C Hong, Joohyun Kim, Meghen Browning, Amy Wagner, Stacee Lerret, Annette D Segura, Michael A Zimmerman
For patients with hepatoblastoma, a timely and complete resection of the tumor is critical to the patient's tumor recurrence-free survival. Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS), a 2-stage hepatectomy procedure, has revolutionized the surgical management of large hepatic tumors with insufficient future liver remnant (FLR) at presentation. Although existing data support the utility of ALPPS in adults with primary and metastatic hepatobiliary malignancy, the literature in children is scarce...
March 10, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28203453/association-of-osteoprotegerin-with-obesity-insulin-resistance-and-non-alcoholic-fatty-liver-disease-in-children
#3
Meltem Erol, Ozlem Bostan Gayret, Hikmet Tekin Nacaroglu, Ozgul Yigit, Oguzhan Zengi, Mehmet Salih Akkurt, Mehmet Tasdemir
BACKGROUND: Osteoprotegerin (OPG) is a member of the tumor necrosis factor superfamily. Reduced OPG levels are related to obesity, insulin resistance, and non-alcoholic fatty liver disease (NAFLD). OBJECTIVES: The aim of this study was to evaluate the relationship between OPG levels, obesity, insulin resistance, and NAFLD in pediatric patients. METHODS: This was a prospective, cross-sectional, controlled study that was conducted in the department of pediatrics at Bagcilar training and research hospital in Istanbul, Turkey, between April and August 2015...
November 2016: Iranian Red Crescent Medical Journal
https://www.readbyqxmd.com/read/28191813/acute-lymphoblastic-leukemia-mimicking-wilms-tumor-at-presentation
#4
Amitabh Singh, Anirban Mandal, Vijay Guru, Rachna Seth
BACKGROUND: Acute lymphoblastic leukemia (ALL), the commonest malignancy of childhood, is known to manifest with a myriad of atypical presentations. Nephromegaly is a rare presentation of childhood ALL with hepatic mass being even rarer. CASE PRESENTATION: We present a 3 year-old child with unilateral renal mass and hepatic mass lesion with normal blood counts, initially suspected to have metastatic Wilms tumor based on clinical, radiological and WT1 positivity on immunocytochemistry of renal mass...
September 2016: Gulf Journal of Oncology
https://www.readbyqxmd.com/read/28144858/a-case-of-adult-undifferentiated-embryonal-sarcoma-of-the-liver-successfully-treated-with-right-trisectionectomy-a-case-report
#5
Akiko Mori, Koji Fukase, Kunihiro Masuda, Naoaki Sakata, Masamichi Mizuma, Hideo Ohtsuka, Takanori Morikawa, Kei Nakagawa, Hiroki Hayashi, Fuyuhiko Motoi, Takeshi Naitoh, Keigo Murakami, Michiaki Unno
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. CASE PRESENTATION: Here, we describe the case of a 65-year-old woman who presented with a huge cystic lesion in the liver. Laboratory studies performed on admission showed modest inflammation, poor nutrition, and elevated levels of total bilirubin, alkaline phosphatase, and γ-glutamyl transferase...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28104133/liver-transplantation-for-biliary-rhabdomyosarcoma-with-liver-metastasis-report-of-one-case
#6
C-H Shen, K-R Dong, Y-F Tao, G Chen, R-D Li, Q-B Zhang, X-F Zhang, S Zheng, Z-X Wang
BACKGROUND: Liver transplantation in combination with chemotherapy in postoperative biliary rhabdomyosarcoma recurrence of children was evaluated. METHODS: An 8-year-old girl with biliary rhabdomyosarcoma underwent pancreatico-duodenectomy with postoperative vincristine (VCR), adriamycin (Act-D), and cyclophosphamide (CTX) (VAC chemotherapy) (VCR, 1 mg; Act-D, 0.7 mg; CTX, 1500 mg). Two years later, liver metastasis in the left and right lobes was found and was followed by VAC chemotherapy (CTX, 1800 mg; Act-D, 0...
January 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28049229/tandem-high-dose-chemotherapy-and-autologous-stem-cell-transplantation-for-high-grade-gliomas-in-children-and-adolescents
#7
Ji Won Lee, Do Hoon Lim, Ki Woong Sung, Hyeong Jin Lee, Eun Sang Yi, Keon Hee Yoo, Hong Hoe Koo, Yeon Lim Suh, Hyung Jin Shin
With the aim to investigate the outcome of tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) for high-grade gliomas (HGGs), we retrospectively reviewed the medical records of 30 patients with HGGs (16 glioblastomas, 7 anaplastic astrocytomas, and 7 other HGGs) between 2006 and 2015. Gross or near total resection was possible in 11 patients. Front-line treatment after surgery was radiotherapy (RT) in 14 patients and chemotherapy in the remaining 16 patients including 3 patients less than 3 years of age...
February 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27955729/malignant-tumors-of-the-liver-in-children
#8
REVIEW
Daniel C Aronson, Rebecka L Meyers
This article aims to give an overview of pediatric liver tumors; in particular of the two most frequently occurring groups of hepatoblastomas and hepatocellular carcinomas. Focus lays on achievements gained through worldwide collaboration. We present recent advances in insight, treatment results, and future questions to be asked. Increasing international collaboration between the four major Pediatric Liver Tumor Study Groups (SIOPEL/GPOH, COG, and JPLT) may serve as a paradigm to approach rare tumors. This international effort has been catalyzed by the Children's Hepatic tumor International Collaboration (CHIC) formation of a large collaborative database...
October 2016: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/27938502/novel-advances-in-understanding-of-molecular-pathogenesis-of-hepatoblastoma-a-wnt-%C3%AE-catenin-perspective
#9
Danielle Bell, Sarangarajan Ranganathan, Junyan Tao, Satdarshan P Monga
Hepatoblastoma is the most common pediatric liver malignancy, typically striking children within the first 3 years of their young lives. While advances in chemotherapy and newer surgical techniques have improved survival in patients with localized disease, unfortunately, for the 25% of patients with metastasis, the overall survival remains poor. These tumors, which are thought to arise from hepatic progenitors or hepatoblasts, hence the name hepatoblastoma, can be categorized by histological subtyping based on their level of cell differentiation...
February 10, 2017: Gene Expression
https://www.readbyqxmd.com/read/27920819/co-administration-of-metformin-and-n-acetylcysteine-with-dietary-control-improves-the-biochemical-and-histological-manifestations-in-rats-with-non-alcoholic-fatty-liver
#10
Naglaa Mohamed El-Lakkany, Sayed Hassan Seif El-Din, Abdel-Nasser Abdel-Aal Sabra, Olfat Ali Hammam, Fatma Abdel-Latif Ebeid
Non-alcoholic fatty liver disease (NAFLD) is a burgeoning health problem that affects 1/3 of the adult population and an increasing number of children in developed countries. Oxidative stress and insulin resistance are the mechanisms that seem to be mostly involved in its pathogenesis. This study was conceived in a NAFLD rat model to evaluate the efficacy of both metformin (MTF) and N-acetylcysteine (NAC) with dietary control on biochemical and histologic liver manifestations. Rats were classified into nine groups; normal (I), NAFLD-induced by feeding high-fat diet (HFD; II) for 12 weeks, NAFLD switched to regular diet (RD; III), NAFLD-HFD or -RD treated with MTF in a dose of 150 mg/kg (IV, V), NAC in a dose of 500 mg/kg (VI, VII) or MTF+NAC (VIII, IX) respectively for 8 weeks...
October 2016: Research in Pharmaceutical Sciences
https://www.readbyqxmd.com/read/27884679/risk-stratified-staging-in-paediatric-hepatoblastoma-a-unified-analysis-from-the-children-s-hepatic-tumors-international-collaboration
#11
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...
January 2017: Lancet Oncology
https://www.readbyqxmd.com/read/27825058/huge-focal-nodular-hyperplasia-presenting-in-a-6-year-old-child-a-case-presentation
#12
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...
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
#13
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 & AIMS: Data on minimal hepatic encephalopathy (MHE) in children is scarce. We aimed to study MHE in children with chronic liver disease (CLD) and to validate non-invasive objective tests which can assist in its diagnosis. METHODS: We evaluated 67 children with CLD (38 boys; age 13 [7-18] years) and 37 healthy children to determine the prevalence of MHE. We also assessed the correlation of MHE with changes in brain metabolites by magnetic resonance spectroscopy ((1)HMRS), diffusion tensor imaging (DTI) derived metrics, blood ammonia and inflammatory cytokines (interleukin-6 [IL6], tumor necrosis factor alpha [TNF-α])...
March 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/27783314/space-occupying-lesions-in-the-liver
#14
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...
November 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27775819/genomic-analysis-of-hepatoblastoma-identifies-distinct-molecular-and-prognostic-subgroups
#15
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 pretreatment 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 pretreatment 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 nuclear factor, erythroid 2-like 2 activity; high lin-28 homolog B, high mobility group AT-hook 2, spalt-like transcription factor 4, and alpha-fetoprotein expression; and high coordinated expression of oncofetal proteins and stem-cell markers, while low-risk tumors had low lin-28 homolog B and lethal-7 expression and high hepatic nuclear factor 1 alpha activity...
January 2017: 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
#16
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
#17
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
#18
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...
February 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27721967/multi-visceral-transplantation-in-a-21-year-old-man-with-prior-pancreatoblastoma
#19
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
#20
REVIEW
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)
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