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Pediatric osteosarcoma

Matthew J Thompson, John C Shapton, Stephanie E Punt, Christopher N Johnson, Ernest U Conrad
BACKGROUND: The quantitative accuracy of MRI in predicting the intraosseous extent of primary sarcoma of bone has not been definitively confirmed, although MRI is widely accepted as an accurate tool to plan limb salvage resections. Because inaccuracies in MRI determination of tumor extent could affect the ability of a tumor surgeon to achieve negative margins and avoid local recurrence, we thought it important to assess the accuracy of MR-determined tumor extent to the actual extent observed pathologically from resected specimens in pediatric patients treated for primary sarcomas of bone...
March 2018: Clinical Orthopaedics and related Research
Zhenqin Zhang, Zhenzhu Zhu, Cheng Luo, Chengcheng Zhu, Changli Zhang, Zijian Guo, Xiaoyong Wang
Osteosarcoma (OS) is the most common primary pediatric bone tumor lethal to children and adolescents. Chemotherapeutic agents such as cisplatin are not effective for OS because of their poor accessibility to this cancer and severe systemic toxicity. In this study, a lipophilic platinum(II) complex bearing a bisphosphonate bone-targeting moiety, cis-[PtL(NH3 )2 Cl]NO3 {BPP; L = tetraethyl [2-(pyridin-2-yl)ethane-1,1-diyl]bisphosphonate}, was prepared and characterized by NMR, electrospray ionization mass spectrometry, and single-crystal X-ray crystallography...
March 7, 2018: Inorganic Chemistry
Suoyuan Li, Tao Zhang, Weiguo Xu, Jianxun Ding, Fei Yin, Jing Xu, Wei Sun, Hongsheng Wang, Mengxiong Sun, Zhengdong Cai, Yingqi Hua
PURPOSE: Osteosarcoma is the most common primary bone cancer and is notorious for pulmonary metastasis, representing a major threat to pediatric patients. An effective drug targeting osteosarcoma and its lung metastasis is urgently needed. DESIGN: In this study, a sarcoma-targeting peptide-decorated disulfide-crosslinked polypeptide nanogel (STP-NG) was exploited for enhanced intracellular delivery of shikonin (SHK), an extract of a medicinal herb, to inhibit osteosarcoma progression with minimal systemic toxicity...
2018: Theranostics
Jun Wu, Cui Wang, Ming Li, Cong Luo, Xing Liu, Lina Xie, Ling He, Mengjia Gong, Zujie Hu, Anthony J Bleyer, Yuanyuan Zhang
OBJECTIVE: This study aimed to investigate the application of 3D computed tomography (CT) angiography with a novel post-processing technique in diagnosis of malignant bone tumors in children. METHODS: Twenty-seven pediatric patients (15 males and 12 females; average age: 10±3.4 years old, with a range from 2 months to 14 years old) with suspected bone tumors were evaluated histopathologically using 3D CT angiography and a multislice scanner. CT angiography image data were analyzed with a novel post-processing technique that included separating, fusing opacifying false-coloring, and volume rendering...
2018: Journal of X-ray Science and Technology
Junhua Cao, Qi An, Lei Wang
Sarcomas arise from primitive mesenchymal cells, which are classified, into two main groups: Bone and soft tissue sarcomas. We have searched all-important electronic databases including Google scholar and PubMed for the collection of latest literature pertaining to pediatric sarcomas. Latest literature confirmed that these tumors are relatively rare and represent only 1% of all malignancies but they have higher incidence in children. Pediatric sarcomas comprise about 13% of all pediatric malignancies and are ranked third in childhood cancers...
February 2018: Oncology Letters
Reghu Kesavapillai Sukumaran, Binitha Rajeshwari, Subin Sugath, S Guruprasad Chellappan, Priyakumari Thankamony, Kusumakumary Parukuttyamma
Background: Osteosarcoma (OS) is the most common primary malignant tumor of bone. The survival of OS patients has steadily improved from <20% in the early 20th century to around 70% with current treatment. There are very few studies in pediatric OS from India analyzing various aspects of the disease. This study focuses on the clinical profile, treatment options, and their complications and survival outcomes in pediatric osteosarcoma (OS) patients. Materials and Methods: This was a retrospective observational study which included pediatric patients <14 years of age, with newly diagnosed OS confirmed by histological diagnosis...
January 2018: Indian Journal of Orthopaedics
Sujing Jiang, Guannan Wang, Ying Dong
RATIONALE: Angiogenesis is a key factor for tumor growth and metastasis both in cancer and sarcoma. Endostar, a novel safe and well-tolerated recombinant human endostatin, can suppress the expression of VEGF and the activation of ERK, MAPK, and AKT, and then inhibit tumor progression. PATIENT CONCERNS: A pediatric osteosarcoma with pulmonary metastasis and malignant pleural effusion. DIAGNOSES: Osteosarcoma with pulmonary metastasis and malignant pleural effusion...
December 2017: Medicine (Baltimore)
X-D Zhang, Y-N Wang, X-Y Feng, J-Y Yang, Y-Y Ge, W-Q Kong
OBJECTIVE: Osteosarcoma is one of the commonest malignant bone tumors, which frequently occurs in children all over the world. To find out methods to improve the therapeutic effect of osteosarcoma, it is necessary to detect the functioning mechanism of miR-30c to regulate the proliferation and metastasis of osteosarcoma cell. PATIENTS AND METHODS: In order to reveal the expression level of miR-30c, quantitative Real-time PCR (qRT-PCR) method was chosen. To evaluate cell viability and proliferation rates, colony formation and cell counting kit-8 (CCK8) assay were introduced...
January 2018: European Review for Medical and Pharmacological Sciences
Hyung-Jun Im, Yi Zhang, Huiyun Wu, Jianrong Wu, Najat C Daw, Fariba Navid, Barry L Shulkin, Steve Y Cho
Purpose To preliminarily assess the potential prognostic value of various fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography (PET) parameters before, during, and after neoadjuvant chemotherapy (NCT). Materials and Methods Thirty-four patients with osteosarcoma were enrolled prospectively from 2008 to 2012 and underwent FDG PET/computed tomography (CT) imaging before (baseline scan), during (interim scan) and after NCT (posttherapy scan). The study was approved by the institutional review board and informed consent was received from patients...
January 19, 2018: Radiology
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
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
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
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
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
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 is no study on preoperative use of gabapentin in pediatric population in terms 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 PLP in pediatric patients undergoing amputation for malignant bone tumors in observation period of 60 days postoperatively...
March 2018: Journal of Pain and Symptom Management
Caitlin D Lowery, Wayne Blosser, Michele Dowless, Shelby Knoche, Jennifer Stephens, Huiling Li, David Surguladze, Nick Loizos, Debra Luffer-Atlas, Gerard J Oakley, Qianxu Guo, Seema Iyer, Brian P Rubin, Louis Stancato
Purpose: Platelet-derived growth factor receptor α (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 mAb 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. In this study, we investigated olaratumab in preclinical models of pediatric bone and soft tissue tumors...
February 15, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
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...
February 2018: Journal of Pediatric Orthopedics
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
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
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
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