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Pediatric Hematology and Oncology

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https://www.readbyqxmd.com/read/29148865/metronomic-therapy-has-low-toxicity-and-is-as-effective-as-current-standard-treatment-for-recurrent-high-risk-neuroblastoma
#1
Frank Berthold, Marc Hömberg, Inna Proleskovskaya, Pavel Mazanek, Margarita Belogurova, Angela Ernst, Jaroslav Sterba
The metronomic therapy concept uses low doses of continuously applied chemotherapeutic, anti-angiogenetic, and immunomodulating drugs. Twenty patients with recurrent and 3 with refractory high-risk neuroblastoma were treated by the metronomic concept using celecoxib, cyclophosphamide, vinblastine, and etoposide for up to 24 months. The outcome was compared to 274 matched patients with a first recurrence from stage 4 neuroblastoma using the variables time from diagnosis to first recurrence, number of organs involved, and MYCN amplification...
November 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29144828/prognostic-impact-of-cd200-and-cd56-expression-in-pediatric-b-cell-acute-lymphoblastic-leukemia-patients
#2
Salah Aref, Emad Azmy, Kadry El-Bakry, Lobna Ibrahim, Sherin Abdel Aziz
This study aimed to determine the prognostic impact of CD200 and CD56 expression in pediatric B cell acute lymphoblastic leukemia (B-ALL) patients, both of which have been implicated in immune tolerance and previously suggested as independent risk factors. CD200 has a central role in immune tolerance that protects stem cells and other critical tissues from immune damage. The expression of CD200/CD56 in leukemic blasts were assessed in leukemic blasts before chemotherapy in 43 bone marrow (BM) and/or peripheral blood (PB) samples by flow cytometry...
November 16, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29120691/therapeutic-advances-in-childhood-leukemia-and-lymphoma-tacl-overview-and-introduction-to-the-proceedings-of-the-2016-tacl-investigator-meeting
#3
Alan S Wayne, Erika Shin-Kashiyama, Richard Sposto, Paul Gaynon
Despite great success in the development of curative therapies for pediatric hematologic malignancies, new approaches are needed to overcome resistance to treatment and to reduce associated side effects. The Therapeutic Advances in Childhood Leukemia and Lymphoma (TACL) Consortium is an early phase clinical trial group dedicated to developing innovative therapies for currently incurable pediatric leukemias and lymphomas ( https://tacl.chla.usc.edu/tacl/ ). In November of 2016, a TACL Investigator Meeting was held, the proceedings of which appear in this edition of Pediatric Hematology Oncology...
November 9, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29065267/the-prognostic-importance-of-tgf-%C3%AE-tgf-%C3%AE-receptor-and-fascin-in-childhood-solid-tumors
#4
Hikmet Gulsah Tanyildiz, Gulsah Kaygusuz, Emel Unal, Nurdan Tacyildiz, Handan Dincaslan, Gulsan Yavuz
Fascin plays a role in tumor metastasis under the influence of TGF-β, each potentiating the effect of the other. We retrospectively investigated whether there was a prognostic relationship between TGF-β and fascin, and disease stage, local recurrence, metastasis tendency, and response to treatment. Twelve neuroblastomas, 17 osteosarcomas, 14 Ewing's sarcomas, 15 rhabdomyosarcoma cases, and 8 rare solid tumors were included. Serum TGF-β levels were high at the time of diagnosis in all groups (p = .015) and decreased significantly during remission (p = ...
October 24, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29040054/pediatric-nut-midline-carcinoma-therapeutic-success-employing-a-sarcoma-based-multimodal-approach
#5
Simone Storck, Alyssa L Kennedy, Karen J Marcus, Lisa Teot, Jennifer Vaughn, Astrid K Gnekow, Bruno Märkl, Ivo Leuschner, Steven G DuBois, Christopher A French, Michael C Frühwald
A subset of poorly differentiated squamous cell carcinomas, NUT midline carcinomas (NMC) are characterized by a translocation t(15;19)(q13;p13) [ 1 ]. The prognosis is generally dismal [ 2 ] and therapeutic success has been limited to exceptional cases [ 3 ]. We present two cases of pediatric NMC from two different institutions treated according to a multimodal sarcoma approach involving surgery, chemotherapy, and focal radiotherapy. One patient has remained in complete continuous remission for over 6 years, while the other is in CR in early follow-up off therapy...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29040023/dramatic-clinical-and-radiographic-response-to-braf-inhibition-in-a-patient-with-progressive-disseminated-optic-pathway-glioma-refractory-to-mek-inhibition
#6
Abhishek Bavle, Jeremy Jones, Frank Y Lin, Amy Malphrus, Adekunle Adesina, Jack Su
While clinical and radiographic responses to agents targeting the mitogen-activated protein kinases (MAPK) pathway have been repor-ted in pediatric low-grade gliomas (LGG), early phase trials indicate refractoriness to these medications in some of these patients. We report a patient with disseminated LGG with the BRAFV600E mutation, which was refractory to selumetinib, a MEK inhibitor, but subsequently showed immediate clinical and radiographic response to dabrafenib, a BRAF inhibitor, with sustained effect for 9 months prior to clinical progression...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29040012/outcome-of-acute-lymphoblastic-leukemia-in-children-with-down-syndrome-polish-pediatric-leukemia-and-lymphoma-study-group-report
#7
Joanna Zawitkowska, Teresa Odój, Katarzyna Drabko, Agnieszka Zaucha-Prażmo, Julia Rudnicka, Michał Romiszewski, Michał Matysiak, Kinga Kwiecińska, Magdalena Ćwiklińska, Walentyna Balwierz, Joanna Owoc-Lempach, Katarzyna Derwich, Jacek Wachowiak, Maciej Niedźwiecki, Elżbieta Adamkiewicz-Drożyńska, Joanna Trelińska, Wojciech Młynarski, Andrzej Kołtan, Mariusz Wysocki, Renata Tomaszewska, Tomasz Szczepański, Marcin Płonowski, Maryna Krawczuk-Rybak, Tomasz Ociepa, Tomasz Urasiński, Agnieszka Mizia-Malarz, Grażyna Sobol-Milejska, Grażyna Karolczyk, Jerzy Kowalczyk
Children with Down syndrome (DS) have a 20-fold increased risk of developing leukemia compared with the general population. The aim of the study was to analyze the outcome of patients diagnosed with Down syndrome and acute lymphoblastic leukemia (ALL) in Poland between the years 2003 and 2010. A total of 1848 children were diagnosed with ALL (810 females and 1038 males). Of those, 41 (2.2%) had DS. The children were classified into three risk groups: a standard-risk group-14 patients, an intermediate-risk group-24, a high-risk group-3...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29040002/fak-src-paxillin-system-expression-and-disease-outcome-in-human-neuroblastoma
#8
Panagiotis Kratimenos, Ioannis Koutroulis, Vasiliki Syriopoulou, Christina Michailidi, Maria Delivoria-Papadopoulos, Jerzy Klijanienko, Stamatios Theocharis
BACKGROUND: Neuroblastoma (NB) often presents with metastatic disease and poor survival. The need for new prognostic markers remains invaluable. The FAK-Src-Paxillin protein system is associated with aggressive phenotype in adult malignancies but is largely unexplored in pediatric NB. OBJECTIVE: To assess FAK-Src-Paxillin protein expression in human NB cell lines and clinical cytology material and to delineate its association with survival. DESIGN/METHODS: Western blot and immunohistochemistry were applied for FAK-Src-Paxillin expression in NB cell lines and 23 human cytology specimens, respectively...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29039999/treatment-and-survival-of-childhood-neuroblastoma-evidence-from-a-population-based-study-in-the-united-states
#9
Diarmuid Coughlan, Matthew Gianferante, Charles F Lynch, Jennifer L Stevens, Linda C Harlan
BACKGROUND: Childhood neuroblastoma describes a heterogeneous group of extracranial solid tumors, that are treated per risk profile. We sought to describe treatment patterns and survival using population-based data from throughout the United States. MATERIALS AND METHODS: Using the National Cancer Institute (NCI)'s Patterns of Care data, we analyzed treatment provided to newly diagnosed, histologically confirmed neuroblastoma patients in 2010 and 2011, registered to one of 14 Surveillance, Epidemiology, and End Results (SEER) cancer registries...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29039989/combination-of-clofarabine-cyclophosphamide-and-etoposide-for-relapsed-or-refractory-childhood-and-adolescent-acute-myeloid-leukemia
#10
Yoav Messinger, Jessica Boklan, John Goldberg, Steven G DuBois, Javier Oesterheld, Oussama Abla, Alissa Martin, Joanna Weinstein, Nobuko Hijiya
Relapsed/refractory acute myeloid leukemia (AML) has an extremely poor prognosis. We describe 17 children and adolescents with relapsed/refractory AML who received clofarabine, cyclophosphamide, and etoposide. Seven patients (41%) responded: 4 with a complete response (CR); 1 with CR with incomplete platelet recovery; and 2 with a partial response. Additionally, 4 developed hypocellular marrow without evidence of leukemia; 5 patients had resistant disease; and 1 suffered early toxic death. After further therapy including transplantation, 4 patients (24%) are alive without evidence of disease at a median of 60 months...
October 17, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29035641/analysis-of-clinical-features-of-lipoblastoma-in-children
#11
Ji-Won Han, Hongbeom Kim, Joong Kee Youn, Chaeyoun Oh, Sung-Eun Jung, Kwi-Won Park, Seong-Cheol Lee, Hyun-Young Kim
OBJECTIVE: A lipoblastoma is pathologically benign but often recurs. Due to its rarity, studies are scarce. The purpose of this study was to investigate the clinical characteristics of lipoblastoma occurring in children and to detect any correlations with the expression of Ki-67. PARTICIPANTS: From 1998 to 2010, 33 patients were diagnosed with lipoblastoma at Seoul National University Children's Hospital. METHODS: Ki-67 immunohistochemistry staining of the tumor tissue was performed...
October 16, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28872997/treatment-of-tumor-lysis-syndrome-in-children-with-leukemia-lymphoma-in-resource-limited-settings-efficacy-of-a-fixed-low-dose-rasburicase
#12
K G Gopakumar, Priyakumari Thankamony, Shwetha Seetharam, Kusumakumary P
Rasburicase is a novel drug used during the management of tumor lysis syndrome. In countries with limited resources, it is frequently given at a lower dose and only for the treatment of established tumor lysis syndrome and not as prophylaxis. A retrospective study was conducted in the department of pediatric oncology at a tertiary referral oncology center in south India to analyze the use of rasburicase over the past 3 years. Data of all the 18 children (< 14 years of age) who were given rasburicase for the management of hyperuricemia were collected and analyzed...
September 5, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28590156/hearing-loss-after-platinum-treatment-is-irreversible-in-noncranial-irradiated-childhood-cancer-survivors
#13
Eva Clemens, Andrica Ch de Vries, Antoinette Am Zehnhoff-Dinnesen, Wim Je Tissing, Jacqueline J Loonen, Saskia Fm Pluijm, Eline van Dulmen-den Broeder, Dorine Bresters, Birgitta Versluys, Leontien Cm Kremer, Helena J van der Pal, Sebastian Jccm Neggers, Martine van Grotel, Marry M van den Heuvel-Eibrink
Cisplatin and carboplatin are effective antineoplastic agents. They are also considered to be potentially highly ototoxic. To date, no long-term follow-up data from well-documented cohorts with substantial numbers of childhood cancer survivors (CCS) with platinum-related hearing loss are available. Therefore, in this study, we studied the reversibility of ototoxicity from discontinuation of treatment onwards in a national cohort of platinum-treated survivors with hearing loss at the end of cancer treatment...
June 7, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28570120/characterization-of-the-coagulation-profile-in-children-with-liver-disease-and-extrahepatic-portal-vein-obstruction-or-shunt
#14
William Beattie, Maria Magnusson, Winita Hardikar, Paul Monagle, Vera Ignjatovic
INTRODUCTION: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. MATERIALS AND METHODS: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children...
June 1, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28532265/encephaloduroarteriosynangiosis-edas-in-young-patients-with-cerebrovascular-complications-of-sickle-cell-disease-single-institution-experience
#15
Michael Winstead, Peter P Sun, Kenneth Martin, Janice Earl, Lynne Neumayr, Carolyn Hoppe, Elliott Vichinsky
Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established.  Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not...
May 22, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28976242/persistent-positive-metaiodobenzylguanidine-scans-after-autologous-peripheral-blood-stem-cell-transplantation-may-indicate-maturation-of-stage-4-neuroblastoma
#16
Yasuhiro Okamoto, Yuichi Kodama, Takuro Nishikawa, Almitra Rindiarti, Takayuki Tanabe, Shunsuke Nakagawa, Takako Yoshioka, Koji Takumi, Tatsuru Kaji, Yoshifumi Kawano
Metaiodobenzylguanidine (MIBG) scans are sensitive testing tools for neuroblastoma. Persistent positive MIBG scans in patients with stage 3 neuroblastoma have previously been found to indicate maturation rather than regression. We assessed the significance of this finding in stage 4 neuroblastoma in the present study. Fifteen consecutive pediatric patients with stage 4 neuroblastoma treated between 2004 and 2014 at the Kagoshima University Hospital were retrospectively examined. Treatment involved a combination of multiagent chemotherapy, resection, autologous peripheral blood stem cell transplantation (PBSCT), radiotherapy, and maintenance therapy with retinoic acid...
April 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28922050/how-to-interpret-high-levels-of-distress-when-using-the-distress-thermometer-in-the-long-term-follow-up-clinic-a-study-with-acute-lymphoblastic-leukemia-survivors
#17
A J Pépin, S Lippé, M Krajinovic, C Laverdière, B Michon, D Sinnett, S Sultan
OBJECTIVES: Recent guidelines recommend to assess emotional distress in pediatric oncology during treatment and in after care. One tool used to do this is the distress thermometer (DT), a simple tool which has almost exclusively been studied in its screening abilities. Given its increased used as a measure of distress per se, it is necessary to document its concurrent validity. The goal of this study was to identify clinical domains (eg, depression, anxiety) and individual symptoms associated with pediatric cancer survivors' rating on the DT...
April 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28727494/efficacy-of-standard-prophylaxis-versus-on-demand-treatment-with-bayer-s-sucrose-formulated-recombinant-fviii-rfviii-fs-in-chinese-children-with-severe-hemophilia-a
#18
Yongqiang Zhao, Juan Xiao, Renchi Yang, Runhui Wu, Yu Hu, Horst Beckmann, Junde Wu, Qingsong Hou, Jing Sun
In China, care of patients with severe hemophilia primarily involves insufficient dosing of on-demand treatment and secondary low-dose prophylaxis (10 IU/kg 2× /wk). We sought to evaluate 3× /wk, standard-dose prophylaxis with sucrose-formulated recombinant factor VIII (rFVIII-FS; Bayer) compared with on-demand treatment in Chinese children with severe hemophilia A. Children and adolescents aged 2-16 years with severe hemophilia A, no inhibitors, and no prophylaxis for >6 consecutive months before study entry were eligible for this 24-week, interventional, sequential-treatment study...
April 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28727480/pilot-study-of-propranolol-premedication-to-reduce-fdg-uptake-in-brown-adipose-tissue-on-pet-scans-of-adolescent-and-young-adult-oncology-patients
#19
Anil George, Partha Sinha, Gary Conrad, Aum A Memon, Emily V Dressler, Lars M Wagner
OBJECTIVE: Physiologic uptake of (18)F-fluorodeoxyglucose (FDG) in brown adipose tissue (adipose tissue) of cancer patients may confound interpretation of positron emission tomography (PET) scans. Uptake in adipose tissue occurs in up to half of pediatric oncology patients undergoing PET scans, and is especially common in adolescents. adipose tissue is innervated by the sympathetic nervous system, and beta blockers such as propranolol have shown efficacy in reducing adipose tissue uptake on PET scans done in older adult oncology patients...
April 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28662353/neuroblastoma-in-children-update-on-clinicopathologic-and-genetic-prognostic-factors
#20
Atif A Ahmed, Lei Zhang, Naresh Reddivalla, Maxine Hetherington
Neuroblastoma is the most common extracranial solid tumor in childhood accounting for 8-10% of all childhood malignancies. The tumor is characterized by a spectrum of histopathologic features and a heterogeneous clinical phenotype. Modern multimodality therapy results in variable clinical response ranging from cure in localized tumors to limited response in aggressive metastatic disease. Accurate clinical staging and risk assessment based on clinical, surgical, biologic and pathologic criteria are of pivotal importance in assigning prognosis and planning effective treatment approaches...
April 2017: Pediatric Hematology and Oncology
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