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Liver tumor children

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
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
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
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)
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
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...
September 16, 2016: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
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
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
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
Sue Creviston Kaste, Scott E Snyder, Monika L Metzger, John T Sandlund, Scott Howard, Matthew Krasin, Barry L Shulkin
BACKGROUND: Methionine transport across plasma membranes occurs via large amino acid transporter ( LAT1), which is overexpressed in malignant cells leading to tracer accumulation within tumors. We investigated the uptake of (11)C-methionine in children and young adults newly diagnosed with Hodgkin lymphoma (HL) or non-Hodgkin lymphoma (NHL) and compared the biodistribution of (11)C-methionine PET-CTs ((11)C- METPET) to that of (18)F-FDG PET-CT ((18)F-FDGPET). METHODS & MATERIALS: Conducted under an IND, we prospectively enrolled patients with newly diagnosed HL (N = 19) and NHL (N = 2) onto the IRB-approved investigation of (11)C-METPET...
September 8, 2016: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
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
Wanbo Liu, Sheng Chen, Bing Liu
BACKGROUND: Hepatoblastoma (HB) is the most common primary malignant tumor of the liver in young children. The aim of this study is to identify the diagnostic and prognostic values of serum exosomal miR-21 in Chinese patients with HB. METHODS: We retrospectively reviewed 32 children with HB. The expressions of miR-21 were detected by real-time PCR. The comparison of diagnostic performance of plasmatic, exosomal miR-21 and AFP levels was measured using the Area Under ROC Curve...
November 2016: Pediatric Surgery International
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
Gang Zhang, Xian-Jun Zhou, Cheng-Zhan Zhu, Qian Dong, Lin Su
BACKGROUND: Hepatoblastoma (HB) is the most common malignant liver tumor in childhood. Complete HB surgical resection which is technically demanding is the cornerstone of effective therapy with a good prognosis. The aim of our study is to evaluate the usefulness of 3D simulation software in assisting hepatectomy in pediatric patients with HB. METHODS: 21 children with HB who underwent hepatectomy were enrolled in this study. All patients underwent computer tomography (CT) imaging preoperatively...
September 2016: Surgical Oncology
Phunnipit Wongmeerit, Krittika Suwanrungruang, Arunee Jetsrisuparb, Patcharee Komvilaisak, Surapon Wiangnon
BACKGROUND: In Thailand, a national treatment protocol for childhood leukemia and lymphoma (LL) was implemented in 2006. Access to treatment has also improved with the National Health Security system. Since these innovations, survival of childhood LL has not been fully described. MATERIALS AND METHODS: Trends and survival of children under 15 with childhood cancers diagnosed between 1993 and 2012 were investigated using the hospitalbased data from the Khon Kaen Cancer Registry, Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand...
2016: Asian Pacific Journal of Cancer Prevention: APJCP
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
Daniel Satgé, Motoi Nishi, Nicolas Sirvent, Michel Vekemans
Constitutional trisomy 18 causes Edwards syndrome, which is characterized by intellectual disability and a particular set of malformations. Although this condition carries high mortality during prenatal and early postnatal life, some of the rare infants who survive the first months develop benign and malignant tumors. To determine the tumor profile associated with Edwards syndrome, we performed a systematic review of the literature. This review reveals a tumor profile differing from those of Down (trisomy 21) and Patau (trisomy 13) syndromes...
September 2016: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
Jamie Harris, Andre Kajdacsy-Balla, Bill Chiu
AIM: To create an orthotopic hepatoma model with local metastasis monitored with ultrasound could be created as a platform for testing new treatments. BACKGROUND: Hepatoma accounts for 25% of liver tumors in children with poor overall survival. Intraabdominal metastasis are present in 35% of patients at time of diagnosis. We hypothesized that an orthotopic tumor model with local metastasis could be created as a platform for testing treatment modalities and could be monitored with ultrasound...
2016: Gastroenterology and Hepatology From Bed to Bench
Dumitru Cristinel Badiu, Cristina Alexandra Manea, Vlad Porojan, Marius Paraschiv, Claudia Mehedintu, Ionut Simion Coman, Valentin Titus Grigorean
Osteosarcomas are the most frequent primary malignant bone tumors in children and adolescents. Like brain metastases in osteosarcomas, the bowel metastases are very rare. We present the case of a 23-year-old female patient, diagnosed and operated in 2008 of osteosarcoma at the tibia, for which she had sessions of neoadjuvant and adjuvant chemotherapy, but presented lungs metastases for which she underwent surgery in 2014. Then, in March 2015, she was diagnosed with an intracranial expansive process, an osteosarcoma metastasis, for which a total ablation of the tumor was performed during the early postoperatory period, being transferred to the General Surgery Clinic for abdominal pain, abdominal distention, vomiting, and lack of intestinal transit regarding faeces and intestinal gas...
May 2016: Chirurgia
Ahmet Ali Tuncer, Adnan Narcı, Fatma Hüsniye Dilek, Didem Baskın Embleton, Salih Çetinkurşun
BACKGROUND: Benign cystic mesothelioma (BCM) is a rare tumor with benign characteristic. There are only 8 child cases reported in the English literature. In this report, we present this rare entity a brief review of the literature. CASE REPORT: A two year-old boy presenting with abdominal swelling was hospitalized. Physical examination revealed a mass filling the abdomen. Laboratory findings were not specific. Abdominal computerized tomography showed a 15×13×11 cm cystic mass extending from the bladder to the liver with no solid components and no infiltration to adjacent organs...
March 2016: Balkan Medical Journal
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