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Chordoma

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https://www.readbyqxmd.com/read/28218953/glial-papillary-tumour-of-the-spinal-cord-with-smarcb1-ini1-loss-and-favourable-long-term-outcome
#1
Martin Hasselblatt, Anastasia Dewi Kurniawan, Stephanie Rozsnoki, Pascal D Johann, Susanne Bens, Florian Oyen, Reinhard Schneppenheim, Reiner Siebert, David Capper, Marcel Kool, Christoph Schul, Werner Paulus
Rhabdoid phenotype and biallelic mutations of the SMARCB1 gene causing loss of SMARCB1/INI1 protein expression are the hallmark of atypical teratoid/rhabdoid tumour (AT/RT), a highly malignant central nervous system tumour mainly affecting infants [1]. Loss of SMARCB1/INI1 protein expression has also been described in a variety of other rhabdoid and non-rhabdoid tumour entities including cribriform neuroepithelial tumour (CRINET) [2], poorly differentiated chordoma [3] as well as rhabdoid peripheral nerve sheath tumour [4] and familial schwannomatosis [5]...
February 20, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28216213/expression-of-cathepsin-k-in-skull-base-chordoma
#2
Kaibing Tian, Junpeng Ma, Liang Wang, Ke Wang, Da Li, Shuyu Hao, Yang Yang, Jiang Du, Guijun Jia, Liwei Zhang, Zhen Wu, Junting Zhang
OBJECTIVE: The objective of this study is to explore the association between cathepsin K and the clinical characteristics of skull base chordoma (SBC). METHODS: In this study, 58 paraffin-embedded samples and 85 frozen samples of 94 patients were included. All clinical data corresponding to these patients were available. Immunohistochemical staining and Quantitative Real-time polymerase chain reaction were performed. Positive rate of Immunohistochemical staining slices and delta CT value of Quantitative Real-time polymerase chain reaction represent the cathepsin K expression level in protein and gene level separately...
February 12, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28188186/nccn-guidelines-insights-bone-cancer-version-2-2017
#3
J Sybil Biermann, Warren Chow, Damon R Reed, David Lucas, Douglas R Adkins, Mark Agulnik, Robert S Benjamin, Brian Brigman, G Thomas Budd, William T Curry, Aarati Didwania, Nicola Fabbri, Francis J Hornicek, Joseph B Kuechle, Dieter Lindskog, Joel Mayerson, Sean V McGarry, Lynn Million, Carol D Morris, Sujana Movva, Richard J O'Donnell, R Lor Randall, Peter Rose, Victor M Santana, Robert L Satcher, Herbert Schwartz, Herrick J Siegel, Katherine Thornton, Victor Villalobos, Mary Anne Bergman, Jillian L Scavone
The NCCN Guidelines for Bone Cancer provide interdisciplinary recommendations for treating chordoma, chondrosarcoma, giant cell tumor of bone, Ewing sarcoma, and osteosarcoma. These NCCN Guidelines Insights summarize the NCCN Bone Cancer Panel's guideline recommendations for treating Ewing sarcoma. The data underlying these treatment recommendations are also discussed.
February 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28184416/best-practices-for-the-management-of-local-regional-recurrent-chordoma-a-position-paper-by-the-chordoma-global-consensus-group
#4
S Stacchiotti, A Gronchi, P Fossati, T Akiyama, C Alapetite, M Baumann, J Y Blay, S Bolle, S Boriani, P Bruzzi, R Capanna, A Caraceni, R Casadei, V Colia, J Debus, T Delaney, A Desai, P Dileo, S Dijkstra, F Doglietto, A Flanagan, S Froelich, P A Gardner, H Gelderblom, Z L Gokaslan, R Haas, C Heery, N Hindi, P Hohenberger, F Hornicek, R Imai, L Jeys, R L Jones, B Kasper, A Kawai, M Krengli, A Leithner, I Logowska, J Martin Broto, D Mazzatenta, C Morosi, P Nicolai, O J Norum, S Patel, N Penel, P Picci, S Pilotti, S Radaelli, F Ricchini, P Rutkowski, S Scheipl, C Sen, E Tamborini, K A Thornton, B Timmermann, V Torri, P U Tunn, M Uhl, Y Yamada, D C Weber, D Vanel, P P Varga, C LA Vleggeert-Lankamp, P G Casali, J Sommer
No abstract text is available yet for this article.
February 9, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28182320/proton-beam-therapy-for-bone-sarcomas-of-the-skull-base-and-spine-a-retrospective-nationwide-multicenter-study-in-japan
#5
Yusuke Demizu, Masashi Mizumoto, Tsuyoshi Onoe, Naoki Nakamura, Yasuhiro Kikuchi, Tetsushi Shibata, Tomoaki Okimoto, Sakurai Hideyuki, Tetsuo Akimoto, Kota Ono, Takashi Daimon, Shigeyuki Murayama
We conducted a retrospective, nationwide multicenter study to evaluate the clinical outcomes of proton beam therapy for bone sarcomas of the skull base and spine in Japan. Eligibility criteria included: (1) histologically proven bone sarcomas of the skull base or spine, (2) no metastases, (3) ≥20 years of age, and (4) no prior treatment with radiotherapy. Of the 103 patients treated between January 2004 and January 2012, we retrospectively analyzed data from 96 patients who were followed-up for >6 months or had died within 6 months...
February 9, 2017: Cancer Science
https://www.readbyqxmd.com/read/28179707/cervical-chordoma-involving-c3-c4-a-case-report
#6
Muhammad Imran, Atiq Ahmed Khan, Syed Muneeb Younus
Chordoma is a rare, indolent but locally invasive, osteolytic, slow growing, low grade, primary bone malignancy, derived from the embryonic remnants of the notochord. It is a midline tumour and it predominantly emerges from the axial skeleton. The most commonly observed location of a chordoma is in the sacrococcygeal region ( 50 to 55% ) followed by the cranio occipital region ( 25 to 30% )We present a case of a 30 years old lady who came to us with complaints of difficulty in walking and inability to hold objects in both hands...
December 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28175529/365%C3%A2-epigenetic-profiling-reveals-a-unique-histone-code-in-chordoma
#7
Nelson Moussazadeh, Samuel H Berman, Ilya Laufer, Mrinal Gounder, Yupeng Zheng, Joshua Sommer, Mark H Bilsky, Neil L Kelleher, Cameron Brennan
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28175422/130%C3%A2-skull-base-chordomas-in-children-and-young-adults
#8
M Maher Hulou, Marcio S Rassi, Kaith Almefty, Wenya Linda Bi, Ian F Dunn, Timothy R Smith, Ossama Al-Mefty
No abstract text is available yet for this article.
August 1, 2016: Neurosurgery
https://www.readbyqxmd.com/read/28166150/68ga-dota-tate-pet-ct-for-molecular-imaging-of-somatostatin-receptor-expression-in-metastasizing-chordoma-comparison-with-18f-fdg
#9
Thorsten Derlin, Jan M Sohns, Katja Hueper
Chordoma is a rare slow-growing neoplasm of neuroectodermal origin, which frequently recurs after removal and has the potential to metastasize. We present the case of a 53-year-old man with metastasizing chordoma who underwent F-FDG and Ga-DOTA-TATE PET/CT for restaging of disease and for evaluation of targeted radionuclide therapy potential. On both F-FDG and Ga-DOTA-TATE PET scans, increased tracer accumulation was observed in chordoma metastases. Besides the increased glucose metabolism in chordoma, this case highlights the potential of Ga-DOTA-TATE PET/CT for restaging of chordoma as well the option of targeted radionuclide therapy in this entity...
February 3, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28153624/the-adult-apical-ligament-of-the-dens-does-not-contain-notochordal-tissue-application-to-better-understanding-the-origins-of-skull-base-chordomas
#10
Christian Fisahn, Cameron Schmidt, Steven Rostad, Rong Li, Tarush Rustagi, Fernando Alonso, Mohammadali M Shoja, Joe Iwanaga, Jens R Chapman, Rod J Oskouian, R Shane Tubbs
INTRODUCTION: The apical ligament has long been reported to contain notochord remnants and thus might serve as a site of origin of chordoma formation at the skull base. However, to our knowledge, the histological study of the apical ligament using histological staining specific for notochordal tissue has not been previously performed. Therefore, the current study was undertaken. METHODS: Fifteen apical ligament samples underwent histological examination with specific markers for notochordal differentiation...
January 30, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28153134/distinctive-head-and-neck-bone-and-soft-tissue-neoplasms
#11
REVIEW
Bibianna Purgina, Chi K Lai
Benign and malignant primary bone and soft tissue lesions of the head and neck are rare. The uncommon nature of these tumors, combined with the complex anatomy of the head and neck, pose diagnostic challenges to pathologists. This article describes the pertinent clinical, radiographic, and pathologic features of selected bone and soft tissue tumors involving the head and neck region, including angiofibroma, glomangiopericytoma, rhabdomyosarcoma, biphenotypic sinonasal sarcoma, chordoma, chondrosarcoma, and osteosarcoma...
March 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28149092/isolated-sphenoid-sinus-lesions-experience-with-a-few-rare-pathologies
#12
Nishanth Sadashiva, B N Nandeesh, Dhaval Shukla, Dhananjaya Bhat, Sampath Somanna, Bhagavatula Indira Devi
INTRODUCTION: The sphenoid sinus is often neglected because of its difficult access. The deep position of the sphenoid sinus hinders early diagnosis of pathologies in that location. Delayed diagnosis can cause serious complications due to proximity to many important structures. OBJECTIVES: The aim of this study is to demonstrate different pathologies which can affect the sphenoid sinus and elucidate the findings. METHODS: Cases of isolated sphenoid sinus lesions encountered in the neurosurgical setting which had rare pathologies are discussed...
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28145559/myoepithelial-tumor-of-soft-tissue-and-bone-current-perspective
#13
REVIEW
Anuj Verma, Bharat Rekhi
Myoepithelial tumor (MET) of soft tissue and bone is an unusual tumor of uncertain differentiation and histogenesis, but lately has been recognized as a distinct tumor entity. This tumor forms a morphologic continuum with a mixed tumor and a parachordoma, but is different from an extra-axial chordoma or chordoma periphericium. METs display a range of histopathologic features, including architectural arrangements/growth patterns, cell types and intervening stroma, leading to their several differential diagnoses...
February 1, 2017: Histology and Histopathology
https://www.readbyqxmd.com/read/28143487/helical-tomotherapy-of-spinal-chordomas-french-multicentric-retrospective-study-of-a-cohort-of-30-cases
#14
Maxime Bobin, Christina Zacharatou, Paul Sargos, Véronique Brouste, Albert Lisbona, Marc-André Mahé, Georges Noël, Amandine Halley, Loïc Feuvret, Louis Gras, Stéphanie Hoppe, Bénédicte Henriques de Figueiredo, Guy Kantor
PURPOSE: To evaluate the efficacy and toxicity of helical tomotherapy (HT) in the management of spine chordomas when proton therapy is unavailable or non-feasible. METHODS AND MATERIALS: Between 2007 and 2013, 30 patients with biopsy-proven chordomas were treated by HT in five French institutions. Information regarding local control (LC), overall survival (OS), progression-free survival (PFS) and metastasis-free survival (MFS) was collected. Clinical efficacy, toxicity and treatment quality were evaluated...
January 31, 2017: Radiation Oncology
https://www.readbyqxmd.com/read/28137565/integrated-and-quantitative-proteomics-of-human-tumors
#15
Y Yakkioui, Y Temel, E Chevet, L Negroni
Quantitative proteomics represents a powerful approach for the comprehensive analysis of proteins expressed under defined conditions. These properties have been used to investigate the proteome of disease states, including cancer. It has become a major subject of studies to apply proteomics for biomarker and therapeutic target identification. In the last decades, technical advances in mass spectrometry have increased the capacity of protein identification and quantification. Moreover, the analysis of posttranslational modification (PTM), especially phosphorylation, has allowed large-scale identification of biological mechanisms...
2017: Methods in Enzymology
https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#16
M N Hayes, D M Langenau
Sarcoma is a type of cancer affecting connective, supportive, or soft tissue of mesenchymal origin. Despite rare incidence in adults (<1%), over 15% of pediatric cancers are sarcoma. Sadly, both adults and children with relapsed or metastatic disease have devastatingly high rates of mortality. Current treatment options for sarcoma include surgery, radiation, and/or chemotherapy; however, significant limitations exist with respect to the efficacy of these strategies. Strong impetus has been placed on the development of novel therapies and preclinical models for uncovering mechanisms involved in the development, progression, and therapy resistance of sarcoma...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28109490/risk-factors-for-cerebrospinal-fluid-leak-in-pediatric-patients-undergoing-endoscopic-endonasal-skull-base-surgery
#17
Amanda L Stapleton, Elizabeth C Tyler-Kabara, Paul A Gardner, Carl H Snyderman, Eric W Wang
OBJECTIVES: To determine the risk factors associated with cerebrospinal fluid (CSF) leak following endoscopic endonasal surgery (EES) for pediatric skull base lesions. METHODS: Retrospective chart review of pediatric patients (ages 1 month to 18 years) treated for skull base lesions with EES from 1999 to 2014. Five pathologies were reviewed: craniopharyngioma, clival chordoma, pituitary adenoma, pituitary carcinoma, and Rathke's cleft cyst. Fisher's exact tests were used to evaluate the different factors to determine which had a statistically higher risk of leading to a post-operative CSF leak...
February 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28106524/the-utility-of-3d-printing-for-surgical-planning-and-patient-specific-implant-design-for-complex-spinal-pathologies-case-report
#18
Ralph J Mobbs, Marc Coughlan, Robert Thompson, Chester E Sutterlin, Kevin Phan
OBJECTIVE There has been a recent renewed interest in the use and potential applications of 3D printing in the assistance of surgical planning and the development of personalized prostheses. There have been few reports on the use of 3D printing for implants designed to be used in complex spinal surgery. METHODS The authors report 2 cases in which 3D printing was used for surgical planning as a preoperative mold, and for a custom-designed titanium prosthesis: one patient with a C-1/C-2 chordoma who underwent tumor resection and vertebral reconstruction, and another patient with a custom-designed titanium anterior fusion cage for an unusual congenital spinal deformity...
January 20, 2017: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/28105164/mir-219-5p-inhibits-proliferation-and-clonogenicity-in-chordoma-cells-and-is-associated-with-tumor-recurrence
#19
Wei Wei, Qiuhang Zhang, Zhenlin Wang, Bo Yan, Yanjun Feng, Pu Li
Chordoma is a rare malignant bone tumor that is usually localized to the skull base, vertebral column and sacrum. The transcription factor brachyury, which is encoded by the T gene, has a critical role in the development and progression of chordoma, although the mechanisms underlying brachyury regulation remain unclear. The aim of the current study was to identify and characterize microRNAs (miRs) that regulate brachyury expression in chordoma. MicroRNAs that target brachyury were predicted using miRanda and TargetScan...
December 2016: Oncology Letters
https://www.readbyqxmd.com/read/28094431/chordoma-immunohistochemical-analysis-of-brachury
#20
İbrahim Sun, Mustafa Güdük, Bülent Güçyetmez, Özlem Yapicier, M Necmettin Pamir
AIM: Chordomas are rare, slow growing but locally aggressive malignancies of the axial skeleton. Skull base chordomas, due to their intricate anatomical localization pose significant challenges to managing physician. In classical and chondroid chordomas the disease course cannot be reliably determined using only morphological criteria. Brachyury (T Gene) was shown to play a central role in chordoma pathogenesis and several studies have also shown that this gene also caries potential as a prognostic biomarker...
December 12, 2016: Turkish Neurosurgery
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