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https://www.readbyqxmd.com/read/29333026/renal-lymphoma-primary-or-first-manifestation-of-aggressive-pediatric-b-cell-lymphoma
#1
Pragnya Coca, Vijay Gandhi Linga, Sadashivudu Gundeti, Ashwani Tandon
Renal lymphoma is an uncommon renal tumor in children. Unlike renal lymphomas presenting as bilateral disease and renal failure, we report a boy who presented with unilateral renal involvement. After initial nephrectomy, he achieved remission with multiagent chemotherapy but relapsed systemically within 3 months. He was initiated on salvage chemotherapy with autologous bone marrow transplant. Even though the initial manifestation was localized lymphoma eventually, it turned out to be a systemic disease. He succumbed to disease at 14 months from diagnosis...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29296780/interleukin-6-levels-predict-event-free-survival-in-pediatric-aml-and-suggest-a-mechanism-of-chemotherapy-resistance
#2
Alexandra M Stevens, Jennifer M Miller, Jaime O Munoz, Amos S Gaikwad, Michele S Redell
The tumor microenvironment can protect cancer cells from conventional anticancer therapies. Thus, targeting these protective mechanisms could eradicate therapy-resistant cancer cells and improve outcomes. Interleukin-6 (IL-6) provides extrinsic protection for several solid tumors and multiple myeloma. In pediatric acute myeloid leukemia (AML), IL-6-induced STAT3 signaling frequently becomes stronger at relapse, and increases in IL-6-induced STAT3 activity are associated with inferior survival after relapse...
August 8, 2017: Blood Advances
https://www.readbyqxmd.com/read/29290072/operations-for-suspected-neoplasms-in-a-resource-limited-setting-experience-and-challenges-in-the-eastern-democratic-of-congo
#3
Luc Malemo Kalisya, Jacques Fadhili Bake, Richard Bigabwa, David H Rothstein, Sarah B Cairo
INTRODUCTION: Surgery is an essential component of a functional health system, with surgical conditions accounting for nearly 11-15% of world disability. While communicable diseases continue to burden low- and low-middle-income countries, non-communicable diseases, such as cancer, are an important cause of morbidity and mortality worldwide. Preliminary data on malignancies in low- and middle-income countries, specifically in Africa, suggest a higher mortality compared to other regions of the world, a difference partially explained by limited availability of screening and early detection systems as well as poorer access to treatment...
December 31, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/29285586/intracranial-ewing-sarcoma-four-pediatric-examples
#4
Michael J Yang, Ros Whelan, Jennifer Madden, Jean M Mulcahy Levy, B K Kleinschmidt-DeMasters, Todd C Hankinson, Nicholas K Foreman, Michael H Handler
BACKGROUND: Ewing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis...
December 28, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29279699/endocrine-health-problems-detected-in-764-patients-evaluated-in-a-late-effects-clinic
#5
Maria Conceição Pereira, Joana S Pereira, Daniela Dias, Sara Donato, Duarte Salgado
Background: Many pediatric cancer survivors have endocrine conditions. After treatment with alkylating agents, steroids, methotrexate, and radiation, several endocrine dysfunctions may appear. Surveillance for late effects is recommended by guidelines worldwide. Objective: The objective of this study was to describe the endocrine outcomes of 764 patients followed during a 20 years' period in our out-patient clinic. Design: We retrospectively reviewed the medical records...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29277722/the-pathogenic-roles-of-heparan-sulfate-deficiency-in-hereditary-multiple-exostoses
#6
REVIEW
Maurizio Pacifici
Heparan sulfate (HS) is an essential component of cell surface and matrix proteoglycans (HS-PGs) that include syndecans and perlecan. Because of their unique structural features, the HS chains are able to specifically interact with signaling proteins -including bone morphogenetic proteins (BMPs)- via their HS-binding domain, regulating protein availability, distribution and action on target cells. Hereditary Multiple Exostoses (HME) is a rare pediatric disorder linked to germline heterozygous loss-of-function mutations in EXT1 or EXT2 that encode Golgi-resident glycosyltransferases responsible for HS synthesis, resulting in a systemic HS deficiency...
December 22, 2017: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29274929/intrapericardial-neurofibromatosis-with-coronary-involvement-a-case-report
#7
E A Moghadam, M A Navabi Shirazi, M R Mirzaaghayan, M Nikoufar, A Ghamari
Neurocutaneous syndromes are heterogenous diseases that are diagnosed in the presence of skin and central nervous system disorders. Neurofibromatosis (NF) is one of these disorders, with autosomal dominant inheritance, that causes tumors that grow on nerves as well as other abnormalities such as skin changes and bone deformities. The most common form of NF is type I. A 6-year-old Iranian boy with neurofibromatosis was referred to the pediatric cardiology clinic due to a soft holosystolic murmur discovered on routine examination...
December 20, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29274153/oral-cancer-a-multicenter-study
#8
K Dhanuthai, S Rojanawatsirivej, W Thosaporn, S Kintarak, A Subarnbhesaj, M Darling, E Kryshtalskyj, C-P Chiang, H-I Shin, S-Y Choi, S-S Lee, P-A Shakib
BACKGROUND: To determine the prevalence and clinicopathologic features of the oral cancer patients. MATERIAL AND METHODS: Biopsy records of the participating institutions were reviewed for oral cancer cases diagnosed from 2005 to 2014. Demographic data and site of the lesions were collected. Sites of the lesion were subdivided into lip, tongue, floor of the mouth, gingiva, alveolar mucosa, palate, buccal/labial mucosa, maxilla and mandible. Oral cancer was subdivided into 7 categories: epithelial tumors, salivary gland tumors, hematologic tumors, bone tumors, mesenchymal tumors, odontogenic tumors, and others...
December 23, 2017: Medicina Oral, Patología Oral y Cirugía Bucal
https://www.readbyqxmd.com/read/29260287/change-in-liver-spleen-and-bone-marrow-magnetic-resonance-imaging-signal-intensity-over-time-in-children-with-solid-abdominal-tumors
#9
Michael Sirignano, Jonathan R Dillman, Brian D Weiss, Charles T Quinn, Bin Zhang, Weizhe Su, Andrew T Trout
BACKGROUND: Reticuloendothelial system MRI signal hypointensity is common in pediatric oncology patients with solid abdominal tumors. OBJECTIVE: To assess changes in liver, spleen and bone marrow T2-weighted MRI signal intensity over time and their relationship to blood transfusion history in children with solid abdominal tumors. MATERIALS AND METHODS: In this retrospective study we measured liver, spleen and bone marrow signal intensity on axial T2-weighted MR images obtained December 2009 through February 2016 in children with hepatoblastoma, neuroblastoma, ganglioneuroblastoma and Wilms tumor...
December 19, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/29236139/-notochordal-tumors-benign-notochordal-tumors-and-chordomas
#10
REVIEW
T F E Barth, A von Witzleben, P Möller, S Scheil-Bertram
Benign notochordal tumors (BNCT) and chordomas are primary bone tumors of the spine with a predominant localization in the sacrum and clival region followed by the vertebral bodies. Besides the most common variant (NOS [not otherwise specified] with hepatoid or renal carcinoma cell-like differentiation) chordomas with chondroid, and polymorphic to anaplastic morphology are described. An unfavorable variant are pediatric chordomas with a loss of INI-1. BNCT and chordomas are characterized by the following immunohistological profile: vimentin+, cytokeratin+/-, epithelial membrane antigen (EMA)+/-, S100 protein+/-, brachyury+...
December 13, 2017: Der Pathologe
https://www.readbyqxmd.com/read/29228868/solitary-fibrous-tumors-in-pediatric-patients-a-rare-and-potentially-overdiagnosed-neoplasm-confirmed-by-stat6-immunohistochemistry
#11
Serena Y Tan, Linda J Szymanski, Carlos Galliani, David Parham, Eduardo Zambrano
Pathological diagnosis of solitary fibrous tumor (SFT) in the pediatric population is challenging, as it occurs uncommonly in this age-group and resembles other spindle cell neoplasms. SFT contains a NAB2-STAT6 fusion gene, which can be reliably detected using STAT6 immunohistochemistry. Positive staining is highly sensitive and specific. We sought to investigate the utility of STAT6 immunohistochemistry, to show how commonly SFT was historically recognized at 3 academic pediatric institutions, to reclassify them when appropriate, and to demonstrate features of major mimics of SFT...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29227372/osteofibrous-dysplasia-of-the-tibia-in-children-outcome-without-resection
#12
Daniel Westacott, Peter Kannu, Jennifer Stimec, Sevan Hopyan, Andrew Howard
BACKGROUND: The proposed association between osteofibrous dysplasia and adamantinoma has led some to advocate resection of the entire lesion, which can require major subsequent reconstruction. However, this link remains unproven and there is some support in more recent literature for a less aggressive approach. This study aims to describe our experience managing pediatric tibial osteofibrous dysplasia with such an approach and to report functional outcomes in children treated thus. METHODS: A total of 28 cases of osteofibrous dysplasia in 25 patients were managed at a referral center for pediatric bone tumors with observation in the first instance, then limited surgical intervention if required to address pain and deformity...
December 8, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29226854/diagnostic-and-prognostic-relevance-of-serum-mir-195-in-pediatric-acute-myeloid-leukemia
#13
Ze Hong, Rongrong Zhang, Haixiao Qi
BACKGROUND: MicroRNA-195 acts as a tumor suppressor in a variety of cancers. However, its clinical significance in pediatric acute myeloid leukemia (AML) remains largely undefined. OBJECTIVE: To investigate the diagnostic and prognostic relevance of miR-195 in this malignancy. METHODS: Expression levels of miR-195 in peripheral blood and bone marrow samples of patients with pediatric AML and normal controls were detected by real-time quantitative PCR...
November 27, 2017: Cancer Biomarkers: Section A of Disease Markers
https://www.readbyqxmd.com/read/29226200/long-term-results-of-osteoarticular-allograft-reconstruction-in-children-with-distal-femoral-bone-tumors
#14
Khodamorad Jamshidi, Mehrdad Bahrabadi, Alireza Mirzaei
Background: There is no consensus regarding the best method of reconstruction in pediatric population following the wide resection of malignant bone tumors. More exploration of the complications of osteoarticular reconstruction leads to less existing controversy of this type of reconstruction, which is the main point of this article. Methods: Long-term outcomes and complications of osteoarticular allograft reconstruction of primary distal femoral bone sarcomas in 22 children with mean age of 10...
September 2017: Archives of Bone and Joint Surgery
https://www.readbyqxmd.com/read/29226089/oncolytic-adenoviruses-as-a-therapeutic-approach-for-osteosarcoma-a-new-hope
#15
REVIEW
Marc Garcia-Moure, Naiara Martinez-Vélez, Ana Patiño-García, Marta M Alonso
Osteosarcoma is the most common bone cancer among those with non-hematological origin and affects mainly pediatric patients. In the last 50 years, refinements in surgical procedures, as well as the introduction of aggressive neoadjuvant and adjuvant chemotherapeutic cocktails, have increased to nearly 70% the survival rate of these patients. Despite the initial therapeutic progress the fight against osteosarcoma has not substantially improved during the last three decades, and almost 30% of the patients do not respond or recur after the standard treatment...
November 2017: Journal of Bone Oncology
https://www.readbyqxmd.com/read/29222262/pediatric-leukemia-susceptibility-disorders-manifestations-and-management
#16
REVIEW
Lisa J McReynolds, Sharon A Savage
The clinical manifestations of inherited susceptibility to leukemia encompass a wide phenotypic range, including patients with certain congenital anomalies or early-onset myelodysplastic syndrome (MDS) and some with no obvious medical problems until they develop leukemia. Leukemia susceptibility syndromes occur as a result of autosomal dominant, autosomal recessive, or X-linked recessive inheritance, or de novo occurrence, of germline pathogenic variants in DNA repair, ribosome biogenesis, telomere biology, hematopoietic transcription factors, tumor suppressors, and other critical cellular processes...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29221844/gabapentin-as-an-adjuvant-therapy-for-prevention-of-acute-phantom-limb-pain-in-pediatric-patients-undergoing-amputation-for-malignant-bone-tumors-a-prospective-double-blind-randomized-controlled-trial
#17
Xiandi Wang, Ying Yi, Dingbo Tang, Yi Chen, Yanhua Jiang, Jun Peng, Jiwei Xiao
CONTEXT: Gabapentin is reported to have an analgesic effect of reducing phantom-limb pain(PLP) in adult patients. There's no study on preoperative use of gabapentin in pediatric population in term of PLP prevention. OBJECTIVE: To determine whether gabapentin could be used as an adjuvant agent of opioid-based pain control to lower the rate of phantom-limb pain in pediatric patients undergoing amputation for malignant bone tumors in observation period of 60 days post-operatively...
December 5, 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/29192868/bullough-s-bump-unusual-protuberant-fibro-osseous-tumor-of-the-temporal-bone-case-report
#18
Bowen Jiang, Harry Mushlin, Lei Zhang, Aaron W James, Alan R Cohen
Scalp and calvarial masses are common in children and the differential diagnosis is broad. The authors recently cared for a child with an unusual fibro-osseous lesion of the temporal bone that has previously not been described in the pediatric neurosurgery literature. A 10-year-old girl presented with a firm, slowly enlarging protuberant mass arising from the squamous suture of the temporal bone without intracranial extension. The mass was removed en bloc through a temporal craniectomy and cranioplasty was performed...
December 1, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29191969/olaratumab-exerts-anti-tumor-activity-in-preclinical-models-of-pediatric-bone-and-soft-tissue-tumors-through-inhibition-of-platelet-derived-growth-factor-receptor-alpha
#19
Caitlin D Lowery, Wayne Blosser, Michele Dowless, Shelby Knoche, Jennifer Stephens, Huiling Li, David Surguladze, Nick Loizos, Debra Luffer-Atlas, Gerard Joseph Oakley, Qianxu Guo, Seema Iyer, Brian P Rubin, Louis F Stancato
PURPOSE: Platelet-derived growth factor receptor alpha (PDGFRα) is implicated in several adult and pediatric malignancies, where activated signaling in tumor cells and/or cells within the microenvironment drive tumorigenesis and disease progression. Olaratumab (LY3012207/IMC-3G3) is a human monoclonal antibody that exclusively binds to PDGFRα and recently received accelerated FDA approval and conditional EMA approval for treatment of advanced adult sarcoma patients in combination with doxorubicin...
November 30, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29177641/prolactinoma-through-the-female-life-cycle
#20
REVIEW
Deirdre Cocks Eschler, Pedram Javanmard, Katherine Cox, Eliza B Geer
Prolactinomas are the most common secretory pituitary adenoma. They typically occur in women in the 3rd-6th decade of life and rarely in the pediatric population or after menopause. Most women present with irregular menses and/or infertility. Dopamine (DA) agonists, used in their treatment, are safe during pregnancy, but in most cases are discontinued at conception with close monitoring for signs or symptoms of tumor growth. Breastfeeding is safe postpartum, provided there was no significant growth during pregnancy...
November 24, 2017: Endocrine
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