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https://www.readbyqxmd.com/read/28643220/denosumab-an-emerging-therapy-in-pediatric-bone-disorders
#1
REVIEW
Alison M Boyce
PURPOSE OF REVIEW: Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand (RANKL), and has emerged as an important novel therapy for skeletal disorders. This article examines the use of denosumab in children. RECENT FINDINGS: Considerable safety and efficacy data exists for denosumab treatment of adults with osteoporosis, bone metastases, and giant cell tumors. Pediatric data is limited; however, evidence suggests denosumab may be beneficial in decreasing bone turnover, increasing bone density, and preventing growth of certain skeletal neoplasms in children...
June 22, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28624811/altered-erythropoiesis-and-decreased-number-of-erythrocytes-in-children-with-neuroblastoma
#2
Fabio Morandi, Sebastiano Barco, Sara Stigliani, Michela Croce, Luca Persico, Corrado Lagazio, Francesca Scuderi, Maria Luisa Belli, Mariapina Montera, Giuliana Cangemi, Sarah Pozzi, Valentina Rigo, Paola Scaruffi, Loredana Amoroso, Giovanni Erminio, Vito Pistoia, Silvano Ferrini, Maria Valeria Corrias
Neuroblastoma (NB) is a pediatric tumor presenting at diagnosis either as localized or metastatic disease, which mainly involves the bone marrow (BM). The physical occupancy of BM space by metastatic NB cells has been held responsible for impairment of BM function. Here, we investigated whether localized or metastatic NB may alter hematopoietic lineages' maturation and release of mature cells in the periphery, through gene expression profiling, analysis of BM smears, cell blood count and flow cytometry analysis...
May 30, 2017: Oncotarget
https://www.readbyqxmd.com/read/28619077/what-is-the-impact-of-local-control-in-ewing-sarcoma-analysis-of-the-first-brazilian-collaborative-study-group-ewing1
#3
Ricardo G Becker, Lauro J Gregianin, Carlos R Galia, Reynaldo Jesus-Garcia Filho, Eduardo A Toller, Gerardo Badell, Suely A Nakagawa, Alexandre David, André M Baptista, Eduardo S Yonamime, Osvaldo A Serafini, Valter Penna, Julie Francine C Santos, Algemir L Brunetto
BACKGROUND: Relapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1). METHODS: Seventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included...
June 15, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28607872/vascularized-fibula-based-physis-transfer-a-follow-up-study-of-longitudinal-bone-growth-and-complications
#4
Ronnie L Shammas, Yash J Avashia, Alfredo E Farjat, Anthony A Catanzano, L Scott Levin, William C Eward, Brian E Brigman, Detlev Erdmann
BACKGROUND: The vascularized free fibula epiphyseal transfer provides an option for the preservation of limb lengthening after resection of the proximal humerus in pediatric sarcoma patients. The purpose of this study was to provide a long-term follow-up of longitudinal growth patterns and outcomes after free fibula epiphyseal transfer in upper extremity reconstruction. METHODS: A retrospective review of 4 patients who underwent free fibula epiphyseal transfer after oncologic resection of the proximal humerus for osteosarcoma was performed...
May 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28598265/robot-assisted-endoscopic-third-ventriculostomy-institutional-experience-in-9-patients
#5
Reid Hoshide, Mark Calayag, Hal Meltzer, Michael L Levy, David Gonda
OBJECTIVE The endoscopic third ventriculostomy (ETV) is an established and effective treatment for obstructive hydrocephalus. In its most common application, surgeons plan their entry point and the endoscope trajectory for the procedure based on anatomical landmarks, then control the endoscope freehand. Recent studies report an incidence of neural injuries as high as 16.6% of all ETVs performed in North America. The authors have introduced the ROSA system to their ETV procedure to stereotactically optimize endoscope trajectories, to reduce risk of traction on neural structures by the endoscope, and to provide a stable mechanical holder of the endoscope...
June 9, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28597942/copy-number-alterations-determined-by-single-nucleotide-polymorphism-array-testing-in-the-clinical-laboratory-are-indicative-of-gene-fusions-in-pediatric-cancer-patients
#6
Tracy M Busse, Jacquelyn J Roth, Donna Wilmoth, Luanne Wainwright, Laura Tooke, Jaclyn A Biegel
Gene fusions resulting from structural rearrangements are an established mechanism of tumorigenesis in pediatric cancer. In this clinical cohort, 1,350 single nucleotide polymorphism (SNP)-based chromosomal microarrays from 1,211 pediatric cancer patients were evaluated for copy number alterations (CNAs) associated with gene fusions. Karyotype or fluorescence in situ hybridization studies were performed in 42% of the patients. Ten percent of the bone marrow or solid tumor specimens had SNP array-associated CNAs suggestive of a gene fusion...
June 9, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28590782/rhabdomyosarcoma-in-children-and-adolescents-patterns-and-risk-factors-of-distant-metastasis
#7
Jeong Rye Kim, Hee Mang Yoon, Kyung-Nam Koh, Ah Young Jung, Young Ah Cho, Jin Seong Lee
OBJECTIVE: The objective of this study is to evaluate patterns of distant metastasis and identified factors that may increase the risk of distant metastasis in pediatric patients with rhabdomyosarcoma. MATERIALS AND METHODS: This retrospective study included 69 patients (age, ≤ 20 years) who had rhabdomyosarcoma diagnosed between January 2000 and February 2016. Various imaging features, including distant metastasis, were evaluated on initial and follow-up imaging studies...
June 7, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28589056/vaginal-ewing-sarcoma-an-uncommon-clinical-entity-in-pediatric-patients
#8
Nathan M Cross, A Luana Stanescu, Erin R Rudzinski, Douglas S Hawkins, Marguerite T Parisi
Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28578183/central-precocious-puberty-secondary-to-adrenocortical-adenoma-in-a-female-child-case-report-and-review-of-the-literature
#9
Betül Ersoy, Deniz Kizilay, Hasan Çayirli, Peyker Temiz, Cüneyt Günşar
BACKGROUND: Pediatric adrenocortical tumors are rare but significant causes of virilization and peripheral precocious puberty (PPP). CASE: A 4-year-old girl presented with development of breast, pubic hair, and facial acne. Her bone age was advanced, and gonadotropins did not elevate in gonadotropin-releasing hormone (GnRH) test. High levels of dehydroepiandrosterone-sulfate, estradiol, and testosterone and detection of a tumor in the left adrenal gland of the abdomen by computed tomography led to a diagnosis of PPP due to adrenal tumor...
May 31, 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28572263/recommendations-for-surveillance-for-children-with-leukemia-predisposing-conditions
#10
REVIEW
Christopher C Porter, Todd E Druley, Ayelet Erez, Roland P Kuiper, Kenan Onel, Joshua D Schiffman, Kami Wolfe Schneider, Sarah R Scollon, Hamish S Scott, Louise C Strong, Michael F Walsh, Kim E Nichols
Leukemia, the most common childhood cancer, has long been recognized to occasionally run in families. The first clues about the genetic mechanisms underlying familial leukemia emerged in 1990 when Li-Fraumeni syndrome was linked to TP53 mutations. Since this discovery, many other genes associated with hereditary predisposition to leukemia have been identified. Although several of these disorders also predispose individuals to solid tumors, certain conditions exist in which individuals are specifically at increased risk to develop myelodysplastic syndrome (MDS) and/or acute leukemia...
June 1, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28561686/advances-in-the-treatment-of-pediatric-bone-sarcomas
#11
Patrick J Grohar, Katherine A Janeway, Luke D Mase, Joshua D Schiffman
Bone tumors make up a significant portion of noncentral nervous system solid tumor diagnoses in pediatric oncology patients. Ewing sarcoma and osteosarcoma, both with distinct clinical and pathologic features, are the two most commonly encountered bone cancers in pediatrics. Although mutations in the germline have classically been more associated with osteosarcoma, there is recent evidence germline alterations in patients with Ewing sarcoma also play a significant role in pathogenesis. Treatment advances in this patient population have lagged behind that of other pediatric malignancies, particularly targeted interventions directed at the biologic underpinnings of disease...
2017: American Society of Clinical Oncology Educational Book
https://www.readbyqxmd.com/read/28544777/the-prognostic-significance-of-circulating-serum-amyloid-a-and-cxc-chemokine-ligand-4-in-osteosarcoma
#12
Ricardo J Flores, Aaron J Kelly, Yiting Li, Xiang Chen, Colin McGee, Mark Krailo, Donald A Barkauskas, John Hicks, Tsz-Kwong Man
BACKGROUND: Osteosarcoma (OS) is the most common pediatric bone cancer.  Despite advances in treatment regimens, the survival rate remains 60-70%.  There is an urgent need to identify prognostic biomarkers, so that targeted therapies can be developed to improve the outcome. PROCEDURE: Our laboratory has previously identified that circulating serum amyloid A (SAA) and CXC chemokine ligand 4 (CXCL4) are upregulated in patients with OS.  In this study, we tested if they could be used as prognostic biomarkers...
May 24, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28534008/a-comparison-of-pediatric-vs-adult-patients-with-the-ewing-sarcoma-family-of-tumors
#13
Vivek Verma, Kyle A Denniston, Christopher J Lin, Chi Lin
PURPOSE: This study sought to identify differences in clinical characteristics, outcomes, and treatments between adult and pediatric patients with the Ewing sarcoma family of tumors (ESFT). METHODS: By using the Surveillance, Epidemiology, and End Results database from 1983 to 2013, 1,870 patients were analyzed (n = 976 pediatric, n = 894 adult). Between the two groups, demographic, tumor, and treatment characteristics were collated and compared. The chi-square test determined differences in proportions of the variables between groups...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28530458/-early-symptoms-of-childhood-malignant-diseases
#14
Zsuzsanna Erzsébet Papp, Izabella Kelemen, Adrienne Horváth
INTRODUCTION: Childhood malignant diseases are rare in pediatric pathology. Early symptoms are not specific, fatigue, pallor, compression signs and bone marrow failure are often mentioned. AIM: To summarize the most frequent early symptoms of childhood malignancies in order to help the physicians in the early recognition. METHOD: In our retrospective study, we processed a period of 5 years between 2012-2016, with an accent on the onset manifestations of malignancies...
May 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28528870/recurrent-multifocal-osteomyelitis-in-children-experience-in-a-tertiary-care-center
#15
Ana Belén Ariza Jiménez, Esmeralda Núñez Cuadros, Rocío Galindo Zavala, Leticia Núñez Caro, Gisela Díaz-Cordobés Rego, Antonio Urda Cardona
INTRODUCTION: Chronic recurrent multifocal osteomyelitis is a rare aseptic bone inflammation that affects pediatric patients. Its management and treatment have not yet been standardized. METHODS: Retrospective, descriptive study of patients under 14 years of age diagnosed with chronic nonbacterial osteomyelitis (CNBO) in a tertiary hospital. We included patients diagnosed over the last 6 years (2010-2015) who met the Jansson criteria. The clinical and radiological characteristics of CNBO were analyzed, as was the outcome after different therapeutic options...
May 19, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28508352/pediatric-precursor-b-acute-lymphoblastic-leukemia-are-t-helper-cells-the-missing-link-in-the-infectious-etiology-theory
#16
REVIEW
Simone Bürgler, David Nadal
Precursor B acute lymphoblastic leukemia (BCP-ALL), the most common childhood malignancy, arises from an expansion of malignant B cell precursors in the bone marrow. Epidemiological studies suggest that infections or immune responses to infections may promote such an expansion and thus BCP-ALL development. Nevertheless, a specific pathogen responsible for this process has not been identified. BCP-ALL cells critically depend on interactions with the bone marrow microenvironment. The bone marrow is also home to memory T helper (Th) cells that have previously expanded during an immune response in the periphery...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28493806/pediatric-benign-bone-tumors-what-does-the-radiologist-need-to-know-pediatric-imaging
#17
Jignesh N Shah, Harris L Cohen, Asim F Choudhri, Saurabh Gupta, Stephen F Miller
No abstract text is available yet for this article.
May 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28490517/atoh1-promotes-leptomeningeal-dissemination-and-metastasis-of-sonic-hedgehog-subgroup-medulloblastomas
#18
Katie B Grausam, Samuel Dr Dooyema, Laure Bihannic, Hasitha Premathilake, A Sorana Morrissy, Antoine Forget, Amanda M Schaefer, Justin H Gundelach, Slobodan Macura, Diane M Maher, Xin Wang, Alex H Heglin, Xijin Ge, Erliang Zeng, Stephanie Puget, Indra Chandrasekar, Kameswaran Surendran, Richard J Bram, Ulrich Schüller, Michael D Taylor, Olivier Ayrault, Haotian Zhao
Medulloblastoma (MB) arising from the cerebellum is the most common pediatric brain malignancy, with leptomeningeal metastases often present at diagnosis and recurrence associated with poor clinical outcome. In this study, we employed mouse MB models to explore the relationship of tumor pathophysiology and dysregulated expression of the Notch pathway transcription factor ATOH1, which is present in aggressive MB subtypes driven by aberrant Sonic Hedgehog/Patched (SHH/PTCH) signaling. In experiments with conditional Atoh1 mouse mutants crossed to Ptch1(+/-) mice which develop SHH-driven MB[63], animals with Atoh1 transgene expression developed highly penetrant MB at a young age with extensive leptomeningeal disease and metastasis to the spinal cord and brain, resembling xenografts of human SHH MB...
May 10, 2017: Cancer Research
https://www.readbyqxmd.com/read/28489628/lengthening-of-newly-formed-humerus-after-autologous-fibula-graft-transplantation-following-intercalary-tumor-resection
#19
Hakan Pilge, Martin Ruppert, Bernd Bittersohl, Bettina Westhoff, Rüdiger Krauspe
Secondary limb lengthening after intercalary bone resection in pediatric patients is still challenging. After the resection, a free fibula autograft can be used to reconstruct the osseous gap. However, in particular in young pediatric patients, insufficient growth of the epiphyseal plate after transplantation may lead to a significant limb-length discrepancy (LLD). In this case, the autograft was used for limb lengthening. We report on the lengthening of a humerus regenerate after fibula autograft transplantation into a humeral defect in a pediatric/young adolescent patient...
May 5, 2017: Journal of Pediatric Orthopedics. Part B
https://www.readbyqxmd.com/read/28477224/bone-tumors-of-the-pediatric-foot-imaging-appearances
#20
Pablo Caro-Domínguez, Oscar M Navarro
Tumors of the foot are rare in children. This review illustrates radiographic, CT and MR imaging findings of foot bone tumors in children based on all cases presented in a tertiary pediatric hospital during the 15-year period of 1999-2014. This search revealed 155 tumors of the foot, 72 of the bones and 83 of the soft tissues. Osteochondroma, bone cyst and fibrous dysplasia were the most frequent benign bone lesions. Ewing sarcoma was the most common malignant osseous tumor. Some tumors showed higher prevalence in certain age ranges and others showed predilection for specific bones...
May 2017: Pediatric Radiology
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