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Pediatric osteosarcoma

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https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#1
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29313943/pediatric-oncologist-willingness-to-offer-germline-tp53-testing-in-osteosarcoma
#2
Eliana Shaul, Michael Roth, Yungtai Lo, David S Geller, Bang Hoang, Rui Yang, David Malkin, Richard Gorlick, Jonathan Gill
BACKGROUND: Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by mutations in the tumor-suppressor gene TP53. Osteosarcoma is a sentinel cancer in LFS. Prior studies using Sanger sequencing platforms have demonstrated that 3% of individuals with osteosarcoma harbor a mutation in TP53. New data from next-generation sequencing have demonstrated that 3.8% of patients with osteosarcoma have a known pathogenic variant, and an additional 5.7% carry exonic variants of unknown significance in TP53...
January 3, 2018: Cancer
https://www.readbyqxmd.com/read/29237527/-a-rhabdomyosarcoma-patient-from-a-li-fraumeni-syndrome-family-a-case-report-and-literature-review
#3
Yao Xie, Wei-Hong Zhao, Ying Hua, Qing Sun, Peng-Hui Wu
Li-Fraumeni syndrome (LFS) is a hereditary cancer predisposition syndrome, with the characteristics of early onset of cancer and high cancer incidence. TP53 is widely accepted as a pathogenic gene of LFS. A 2 years and 6 months old boy is reported in this article, who was diagnosed with embryonal rhabdomyosarcoma (RMS) in the left submandibular region. His brother died of RMS, and his grandmother was diagnosed with breast cancer. TP53 gene mutation detection was performed in this patient and some family members, indicating a missense mutation in exon 8 of the patient: c...
December 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29226089/oncolytic-adenoviruses-as-a-therapeutic-approach-for-osteosarcoma-a-new-hope
#4
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/29224821/a-multicenter-study-of-malignant-oral-and-maxillofacial-lesions-in-children-and-adolescents
#5
José Alcides Almeida de Arruda, Leni Verônica de Oliveira Silva, Camila de Nazaré Alves de Oliveira Kato, Lauren Frenzel Schuch, Aline Carvalho Batista, Nádia Lago Costa, Sandra Beatriz Chaves Tarquinio, Elena Riet Correa Rivero, Vinícius Coelho Carrard, Manoela Domingues Martins, Ana Paula Veras Sobral, Ricardo Alves Mesquita
OBJECTIVES: To investigate the frequency of malignant oral and maxillofacial lesions among children and adolescents from representative geographic regions of Brazil. MATERIALS AND METHODS: A retrospective study was conducted on biopsies obtained from 1990 to 2016 at six Brazilian oral and maxillofacial pathology referral centers. A total of 85,105 biopsy specimens from children and adolescents were analyzed. Gender, age, anatomical location, symptomatology and histopathological diagnosis were evaluated...
December 2017: Oral Oncology
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
#6
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/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
#7
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/29176457/a-frame-free-vascularized-fibular-graft-and-femoral-lengthening-for-osteosarcoma-pediatric-patients
#8
Megan Cashin, Christopher Coombs, Ian Torode
BACKGROUND: Pediatric limb reconstruction after resection of a malignant tumor presents specific challenges. Multiple surgical techniques have been used to treat these patients. This paper describes a staged surgical technique for the reconstruction of large distal femoral defects due to tumor resection in skeletally immature patients. METHODS: Three pediatric patients with osteosarcoma of the distal femur underwent staged reconstruction. Neoadjuvant chemotherapy was followed by en bloc tumor resection and immediate reconstruction of the distal femoral defect with a vascularized free fibular autograft utilizing a unique A-frame construct combined with intramedullary nail fixation...
November 21, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29168356/single-institution-experience-on-cancer-among-adolescents-15-19-years-of-age
#9
Begül Yağcı-Küpeli, Canan Akyüz, Bilgehan Yalçın, Ali Varan, Tezer Kutluk, Münevver Büyükpamukçu
Yağcı-Küpeli B, Akyüz C, Yalçın B, Varan A, Kutluk T, Büyükpamukçu M. Single institution experience on cancer among adolescents 15-19 years of age. Turk J Pediatr 2017; 59: 1-5. Adolescent cancers differ from other age groups in terms of cancer types, treatment and outcome. We aimed to present our institutional data on survival of certain types of cancer in adolescents. Hospital files were retrospectively evaluated for distribution of tumor types, clinical features, and overall and event-free survival (OS and EFS) rates in adolescents with malignant tumors...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29163677/trim37-promotes-tumor-cell-proliferation-and-drug-resistance-in-pediatric-osteosarcoma
#10
Yanling Tao, Meiyun Xin, Huanchen Cheng, Zongxuan Huang, Tiantian Hu, Teng Zhang, Jianlong Wang
Osteosarcoma (OS) is among the most frequently occurring bone tumors, particularly in children. Clinical treatment of OS is limited due to several factors including resistance to chemotherapy drugs and metastasis, and the underlying molecular mechanisms remain unclear. In the present study, tripartite motif containing 37 (TRIM37) expression levels were upregulated in tumor samples and associated with the development of drug resistance in OS. Furthermore, chemotherapy drug treatment (doxorubicin, cisplatin and methotrexate) induced TRIM37 expression in OS cells in vitro...
December 2017: Oncology Letters
https://www.readbyqxmd.com/read/29113313/whole-exome-analysis-in-osteosarcoma-to-identify-a-personalized-therapy
#11
Caterina Chiappetta, Massimiliano Mancini, Francesca Lessi, Paolo Aretini, Veronica De Gregorio, Chiara Puggioni, Raffaella Carletti, Vincenzo Petrozza, Prospero Civita, Sara Franceschi, Antonio G Naccarato, Carlo Della Rocca, Chiara M Mazzanti, Claudio Di Cristofano
Osteosarcoma is the most common pediatric primary non-hematopoietic bone tumor. Survival of these young patients is related to the response to chemotherapy and development of metastases. Despite many advances in cancer research, chemotherapy regimens for osteosarcoma are still based on non-selective cytotoxic drugs. It is essential to investigate new specific molecular therapies for osteosarcoma to increase the survival rate of these patients. We performed exomic sequence analyses of 8 diagnostic biopsies of patients with conventional high grade osteosarcoma to advance our understanding of their genetic underpinnings and to correlate the genetic alteration with the clinical and pathological features of each patient to identify a personalized therapy...
October 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/29065267/the-prognostic-importance-of-tgf-%C3%AE-tgf-%C3%AE-receptor-and-fascin-in-childhood-solid-tumors
#12
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/28954696/the-effect-of-immune-checkpoint-inhibitors-on-lung-metastases-of-osteosarcoma
#13
Takahiro Shimizu, Yasushi Fuchimoto, Kazumasa Fukuda, Hajime Okita, Yuko Kitagawa, Tatsuo Kuroda
BACKGROUND/PURPOSE: The prognosis of patients with metastases remains unsatisfactory in certain pediatric solid tumors. In this study, we evaluated the efficacy of immune checkpoint inhibitors against such metastases using a murine model of osteosarcoma. METHODS: Murine osteosarcoma LM8 cells were transplanted subcutaneously into C3H mice. The primary tumor lesion was surgically resected 11 days after transplantation. Two hundred micrograms of three antibodies (anti-PD-1, anti-PD-L1, and anti-OX-86) or an isotype antibody were administered intraperitoneally on post-transplantation days 11, 14, 18, and 21...
September 4, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28944406/downregulated-adhesion-associated-micrornas-as-prognostic-predictors-in-childhood-osteosarcoma
#14
L E A Delsin, G M Roberto, P F Fedatto, E E Engel, C A Scrideli, L G Tone, M S Brassesco
miRNAs have been identified as key regulators of almost all cellular processes, therefore, their dysregulation is involved with several diseases, including cancer. miRNAs specifically related to the metastastic cascade are called metastamiRs and can be involved with different steps of this process, including loss of adhesion. Osteosarcoma (OS) is the most common primary malignant pediatric bone tumor that often presents metastatic disease at diagnosis; therefore, a deeper study of adhesion-associated miRNAs could shed light on its pathophysiology...
September 25, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28938163/tumor-cell-targeted-delivery-of-crispr-cas9-by-aptamer-functionalized-lipopolymer-for-therapeutic-genome-editing-of-vegfa-in-osteosarcoma
#15
Chao Liang, Fangfei Li, Luyao Wang, Zong-Kang Zhang, Chao Wang, Bing He, Jie Li, Zhihao Chen, Atik Badshah Shaikh, Jin Liu, Xiaohao Wu, Songlin Peng, Lei Dang, Baosheng Guo, Xiaojuan He, D W T Au, Cheng Lu, Hailong Zhu, Bao-Ting Zhang, Aiping Lu, Ge Zhang
Osteosarcoma (OS) is a highly aggressive pediatric cancer, characterized by frequent lung metastasis and pathologic bone destruction. Vascular endothelial growth factor A (VEGFA), highly expressed in OS, not only contributes to angiogenesis within the tumor microenvironment via paracrine stimulation of vascular endothelial cells, but also acts as an autocrine survival factor for tumor cell themselves, thus making it a promising therapeutic target for OS. CRISPR/Cas9 is a versatile genome editing technology and holds tremendous promise for cancer treatment...
September 13, 2017: Biomaterials
https://www.readbyqxmd.com/read/28937522/ambulatory-high-dose-methotrexate-administration-in-pediatric-osteosarcoma-patients-at-a-single-institution-in-argentina
#16
Jose A Villegas Rubio, Walter Cacciavillano, Adriana Rose, Pedro Zubizarreta, Marcelo Scopinaro
BACKGROUND: The purpose of this study was to evaluate the feasibility and safety of ambulatory high-dose methotrexate (HDMTX) administration with oral hydration, alkalinization, and leucovorin rescue. HDMTX (12 g/m) was given intravenously over 4 hours after urine alkalinization. Families and patients were instructed to continue ambulatory oral hydration and alkalinization to monitor urine pH and to adjust bicarbonate according to our institution's treatment algorithm. Clinical status and MTX levels were controlled every 24 hours, and oral leucovorin dose was adjusted accordingly...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28887617/fdg-pet-ct-appearance-of-local-osteosarcoma-recurrences-in-pediatric-patients
#17
Susan E Sharp, Barry L Shulkin, Michael J Gelfand, M Beth McCarville
BACKGROUND: Osteosarcoma is the most common pediatric malignant bone tumor, frequently surgically managed with limb salvage rather than amputation. Local recurrences are seen in up to 9% of osteosarcoma patients, with CT and MRI imaging often limited by metal artifacts. OBJECTIVE: To describe the [F-18]2-fluoro-2-deoxyglucose (FDG) PET/CT appearance of local osteosarcoma recurrences with correlation to findings on other imaging modalities. MATERIALS AND METHODS: A retrospective review of pediatric osteosarcoma patients imaged with FDG PET/CT was performed in patients with pathologically proven local recurrences...
December 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28841702/prognostic-value-of-fdg-pet-indices-for-the-assessment-of-histological-response-to-neoadjuvant-chemotherapy-and-outcome-in-pediatric-patients-with-ewing-sarcoma-and-osteosarcoma
#18
Clement Bailly, Rodolphe Leforestier, Loic Campion, Estelle Thebaud, Anne Moreau, Francoise Kraeber-Bodere, Thomas Carlier, Caroline Bodet-Milin
PURPOSE: The objective of this retrospective work was to evaluate the prognostic value on histological response and survival of quantitative indices derived from FDG-PET performed before and after chemotherapy (CHT), in a homogeneous pediatric Ewing sarcoma (EWS) and Osteosarcoma (OST) population. METHODS: Thirty-one patients with EWS and 31 with OST were included. All patients were treated with neoadjuvant CHT, and underwent surgery for local control. All patients had FDG-PET at diagnosis and after CHT, prior to surgery...
2017: PloS One
https://www.readbyqxmd.com/read/28806574/quantitative-diffusion-weighted-magnetic-resonance-imaging-assessment-of-chemotherapy-treatment-response-of-pediatric-osteosarcoma-and-ewing-sarcoma-malignant-bone-tumors
#19
Andrew J Degnan, Chul Y Chung, Amisha J Shah
OBJECTIVE: Assessment of tumor response to chemotherapy is essential in managing malignant pediatric bone tumors prior to resection. SUBJECTS AND METHODS: Pre-chemotherapy and post-chemotherapy osteosarcoma and Ewing sarcoma cases (n=18) were analyzed with apparent diffusion coefficient (ADC) values measured by two readers. RESULTS: Treated tumors demonstrated significantly greater ADC values compared to untreated tumors (p<0.001). Intraclass correlation coefficients ranged between 0...
August 5, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28802390/pediatric-head-and-neck-bone-sarcomas-an-analysis-of-204-cases
#20
Jacob S Brady, Sei Y Chung, Emily Marchiano, Jean Anderson Eloy, Soly Baredes, Richard Chan Woo Park
OBJECTIVES: To analyze the demographics, survival, and treatment efficacy of pediatric sarcomas of the facial skeleton and skull. METHODS: Retrospective study of cases from the US National Cancer Institute's Surveillance, Epidemiology, and End Results database. Pediatric patients between the ages of 0 and 18 diagnosed with a malignant sarcoma of either the mandible or the bones of skull, face, and associated joints from 1973 to 2013 were studied. RESULTS: In total, 204 patients were included in the analysis...
September 2017: International Journal of Pediatric Otorhinolaryngology
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