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Chordomas

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https://www.readbyqxmd.com/read/29148152/u-ch17p-m-and-s-a-new-cell-culture-system-for-tumour-diversity-and-progression-in-chordoma
#1
D Jäger, A Lechel, U Tharehalli, C Seeling, P Möller, T F E Barth, K Mellert
Chordoma is a rare bone tumour with a known intrinsic heterogeneity. Here, we address this tumour heterogeneity in a new cell culture model for tumour diversity and progression in chordoma. The three cell lines U-CH17P, U-CH17M, and U-CH17S were established from a primary sacral chordoma and its derived metastases, a soft tissue and a skin metastasis, respectively. The lesions had divergent differentiation patterns which are conserved in the derived cell lines making them a suitable in vitro model for the analysis of tumorigenesis in chordoma...
November 16, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29142772/rare-case-of-an-extraosseous-cervical-chordoma-with-both-intradural-and-extensive-extraspinal-involvement
#2
Victor Awuor, Christopher E Stewart, Albert Camma, Julie Renner, J M Tongson
Background: Chordomas must be considered among the differential diagnoses for extradural spinal tumors, especially involving the clival or sacrococcygeal regions. They are often locally invasive and destructive to the osseous structures from which they arise, but rarely extend intradurally. Here, we report a unique chordoma that was intradural and spanned nearly four subaxial cervical vertebral levels. Case Description: We report the case of an atypical intradural chordoma that spanned four subaxial levels of the cervical spine in an 81-year-old female...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/29141919/rare-tumors-in-kids-may-respond-to-tazemetostat
#3
(no author information available yet)
In a phase I trial of the EZH2 inhibitor tazemetostat, children with INI1-deficient tumors-including relapsed or refractory malignant rhabdoid tumors, atypical teratoid rhabdoid tumors, epithelioid sarcomas, and poorly differentiated chordomas-responded well to treatment, with some experiencing durable responses.
November 15, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/29138702/a-case-of-c5-vertebral-chordoma-in-a-73-year-old-patient-with-more-than-8-years-of-follow-up-after-total-piecemeal-spondylectomy
#4
Keigo Tanaka, Harutoshi Sakakima, Kazutoshi Hida, Kanako Hatanaka, Kosei Ijiri
Chordoma arising from the cervical spine is rare and the traditional long-term prognosis is typically poor. Total en bloc spondylectomy with a wide margin is generally accepted to be the most appropriate management for thoracic and lumbar malignant tumors. However, this method is still challenging for the cervical spine because of the proximity of the tumor to the vertebral arteries and neural elements. Here, we report a 73-year-old man with a C5 vertebral chordoma treated with total piecemeal spondylectomy...
2017: Case Reports in Orthopedics
https://www.readbyqxmd.com/read/29126120/cranial-chordoma-a-new-preoperative-grading-system
#5
Harley Brito da Silva, David Straus, Jason K Barber, Robert C Rostomily, Manuel Ferreira, Laligam N Sekhar
BACKGROUND: Chordomas are rare but challenging neoplasms involving the skull base. A preoperative grading system will be useful to identify both areas for treatment and risk factors, and correlate to the degree of resection, complications, and recurrence. OBJECTIVE: To propose a new grading system for cranial chordomas designed by the senior author. Its purpose is to enable comparison of different tumors with a similar pathology to clivus chordoma, and statistically correlate with postoperative outcomes...
November 3, 2017: Neurosurgery
https://www.readbyqxmd.com/read/29119645/high-sensitivity-of-fish-analysis-in-detecting-homozygous-smarcb1-deletions-in-poorly-differentiated-chordoma-a-clinicopathologic-and-molecular-study-of-9-cases
#6
Adepitan A Owosho, Lei Zhang, Marc K Rosenblum, Cristina R Antonescu
Poorly differentiated chordomas (PDCs) represent a rare subset of notochordal neoplasms, affecting primarily children and associated with an aggressive outcome. In contrast to conventional chordomas, PDC show solid growth and increased cellularity, cytologic atypia, and mitotic activity. Recent studies have shown that PDCs are characterized by recurrent deletions encompassing the SMARCB1 locus, resulting in consistent loss of nuclear SMARCB1 expression. Thus PDC joined the expanding family of SMARCB1-deficient tumors characterized by various SMARCB1 structural abnormalities, ranging from large homozygous deletions to small intragenic mutations...
November 9, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29113181/microrna-profiling-and-bioinformatics-analyses-reveal-the-potential-roles-of-micrornas-in-chordoma
#7
Kangwu Chen, Hao Chen, Kai Zhang, Siwei Sun, Jianqiang Mo, Jian Lu, Zhonglai Qian, Huilin Yang
Chordoma is a rare aggressive bone tumor arising from remnants of the notochord, and patients with chordoma have a poor prognosis. However, the unique expression profiles of microRNAs (miRNAs/miRs) and their downstream signaling pathways in chordoma remain incompletely characterized. The aim of the present study was to delineate the global miRNA expression profile and associated signaling networks in chordoma. miRNA profiling was performed on chordoma and fetal notochord tissues. Differentially expressed miRNAs in chordoma were analyzed using microarrays with hierarchical clustering analysis...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29110879/a-diagnostic-approach-to-bone-tumours
#8
REVIEW
Adrienne M Flanagan, Daniel Lindsay
In this review we discuss an approach to diagnosing primary bone tumours, namely the cartilaginous, bone-forming, giant cell-rich, fibro-osseous and round cell neoplasms. Less common lesions including chordoma are also discussed. The value of integrating clinical, histopathological and relevant radiological features is emphasised with a view to providing the general histopathologist with a methodical approach to reaching an accurate diagnosis.
October 27, 2017: Pathology
https://www.readbyqxmd.com/read/29104156/endoscopic-approach-to-clival-chordomas-the-northwestern-experience
#9
Rudy J Rahme, Omar M Arnaout, Olabisi R Sanusi, Kartik Kesavabhotla, James P Chandler
INTRODUCTION: Chordomas are rare primary bone tumors with a low-grade histology but an aggressive clinical behavior characterized by local invasion and recurrence. When occurring in the skull base, their treatment is limited by proximity to critical neurovascular structures. Open surgical approaches can carry high morbidity making the development of alternative approaches desirable. We describe our experience with endoscopic endonasal approaches to clival chordomas over 13 years. MATERIALS AND METHOD: We performed a retrospective chart review of patients diagnosed with clival chordomas and treated with an endoscopic endonasal approach between the years 2003 and 2015 at Northwestern Memorial Hospital...
November 2, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29063744/tailored-surgical-strategy-in-clival-chordomas-an-extraordinary-selection-bias-that-limits-approach-comparison
#10
Emanuele LA Corte, Morgan Broggi, Lorenzo Bosio, Giovanni Danesi, Paolo Ferroli
No abstract text is available yet for this article.
October 23, 2017: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/29061463/presentation-treatment-and-long-term-outcome-of-intrasellar-chordoma-a-pooled-analysis-of-institutional-seer-and-published-data
#11
Abdul-Kareem Ahmed, Hassan Y Dawood, Omar M Arnaout, Edward R Laws, Timothy R Smith
BACKGROUND: Chordoma that occurs primarily in the sella turcica is rare and presents unique treatment challenges. OBJECTIVE: The purpose of this study was to determine common features, treatment approaches and survival characteristics of intrasellar chordoma. METHODS: Institutional databases, the Surveillance, Epidemiology, and End Results (SEER) database, and PubMed/EMBASE were queried for chordoma with a primarily intrasellar component. The SEER database was also queried for adult skull base chordoma...
October 20, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29051990/clinicopathologic-implications-of-cd8-foxp3-ratio-and-mir-574-3p-pd-l1-axis-in-spinal-chordoma-patients
#12
Ming-Xiang Zou, Ke-Miao Guo, Guo-Hua Lv, Wei Huang, Jing Li, Xiao-Bin Wang, Yi Jiang, Xiao-Ling She
Currently, little is known about the interactions between microRNAs (miRNAs) and the PD-1/PD-L1 signaling pathway in chordoma, and data discussing the role of the immune milieu in chordoma prognosis are limited. We aimed to analyze the relationship between PD-L1, miR-574-3p, microenvironmental tumor-infiltrating lymphocytes (TILs) and clinicopathological features of spinal chordoma patients. PD-L1 expression and TILs (including Foxp3(+), CD8(+), PD-1(+) and PD-L1(+)) were assessed by immunohistochemistry in tumor specimens of 54 spinal chordoma patients...
October 20, 2017: Cancer Immunology, Immunotherapy: CII
https://www.readbyqxmd.com/read/29050071/-poorly-differentiated-chordoma-with-ini1-loss-a-clinicopathologic-study
#13
J Y Feng, L Chen, Y Y Ma, H W Yang, L Chen
Objective: To analyze the clinicopathologic characteristics of poorly-differentiated chordoma with INI1 loss in children and to discuss the differential diagnosis. Methods: The clinical, radiological, histopathological profiles and molecular pathologic characteristics of two pediatric poorly differentiated chordoma cases with INI1 loss were reviewed. Results: The patients were a girl and a boy. Both lesions involved the slope. Both patients were presented with progressive muscle weakness or neck pain. Radiological examination showed clivus bone destruction and compression of the brain stem and cervical spinal cord...
October 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29045855/prognostic-factors-in-skull-base-chordoma-a-systematic-literature-review-and-meta-analysis
#14
REVIEW
Ming-Xiang Zou, Guo-Hua Lv, Qian-Shi Zhang, Shao-Fu Wang, Jing Li, Xiao-Bin Wang
OBJECTIVE: Currently, there are still lack of reviews assessing the complete range of prognostic factors in skull base chordoma (SBC). This study aimed to systematically review the published literature on prognostic factors in SBC and to establish pooled hazard ratios (HRs) of such factors. METHODS: MEDLINE and EMBASE search (inception to April 04, 2017). Two reviewers independently selected papers involving SBC prognostic factors, and studied them for methodological quality and valuable factors...
October 15, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29040778/multimodality-management-of-recurrent-skull-base-chordomas-factors-impacting-tumor-control-and-disease-specific-survival
#15
Shaan M Raza, Diana Bell, Jacob L Freeman, David R Grosshans, Gregory N Fuller, Franco DeMonte
BACKGROUND: Limited data exist to guide the management of recurrent chordomas arising in the skull base. OBJECTIVE: To determine factors affecting tumor control rates and disease-specific survival (DSS) in recurrent disease. METHODS: A retrospective review was performed of 29 patients with 55 recurrences treated at our institution. Tumor and treatment factors were assessed for impact on freedom from progression (FFP; primary outcome) and DSS (secondary outcome)...
October 10, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29040677/navigation-linked-heads-up-display-in-intracranial-surgery-early-experience
#16
Justin R Mascitelli, Leslie Schlachter, Alexander G Chartrain, Holly Oemke, Jeffrey Gilligan, Anthony B Costa, Raj K Shrivastava, Joshua B Bederson
BACKGROUND: The use of intraoperative navigation during microscope cases can be limited when attention needs to be divided between the operative field and the navigation screens. Heads-up display (HUD), also referred to as augmented reality, permits visualization of navigation information during surgery workflow. OBJECTIVE: To detail our initial experience with HUD. METHODS: We retrospectively reviewed patients who underwent HUD-assisted surgery from April 2016 through April 2017...
October 10, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/29038849/malignant-bone-tumors-other-than-ewing-s-clinical-practice-guidelines-for-diagnosis-treatment-and-follow-up-by-spanish-group-for-research-on-sarcomas-geis
#17
Andrés Redondo, Silvia Bagué, Daniel Bernabeu, Eduardo Ortiz-Cruz, Claudia Valverde, Rosa Alvarez, Javier Martinez-Trufero, Jose A Lopez-Martin, Raquel Correa, Josefina Cruz, Antonio Lopez-Pousa, Aurelio Santos, Xavier García Del Muro, Javier Martin-Broto
Primary malignant bone tumors are uncommon and heterogeneous malignancies. This document is a guideline developed by the Spanish Group for Research on Sarcoma with the participation of different specialists involved in the diagnosis and treatment of bone sarcomas. The aim is to provide practical recommendations with the intention of helping in the clinical decision-making process. The diagnosis and treatment of bone tumors requires a multidisciplinary approach, involving as a minimum pathologists, radiologists, surgeons, and radiation and medical oncologists...
October 16, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/29029887/long-term-clinical-safety-of-high-dose-proton-radiation-therapy-delivered-with-pencil-beam-scanning-technique-for-extracranial-chordomas-and-chondrosarcomas-in-adult-patients-clinical-evidence-of-spinal-cord-tolerance
#18
Sonja Stieb, James W Snider, Lorenzo Placidi, Ulrike Kliebsch, Anthony J Lomax, Ralf A Schneider, Damien C Weber
PURPOSE: To assess the radiation dose tolerance of the spinal cord by reviewing our institutional experience regarding the incidence of radiation-induced spinal cord toxicity after high-dose pencil beam scanning proton therapy (PBSPT). METHODS AND MATERIALS: Seventy-six patients (median age 53 years; range, 23-79 years) treated for spinal chordoma (n=55) or chondrosarcoma (n=21) met the following criteria and were retrospectively analyzed: PBSPT only, no reirradiation or concomitant chemotherapy, maximum dose (Dmax) to the spinal cord of ≥45 Gy(relative biological effectiveness [RBE]), ≥18 years of age, and follow-up of ≥12 months...
September 4, 2017: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/29029249/prognostic-factors-in-patients-with-spinal-chordoma-an-integrative-analysis-of-682-patients
#19
(no author information available yet)
No abstract text is available yet for this article.
November 1, 2017: Neurosurgery
https://www.readbyqxmd.com/read/29026317/high-expression-of-sam68-in-sacral-chordomas-is-associated-with-worse-clinical-outcomes
#20
Hai Wen, Pengzhi Li, Hong Ma, Jiaoyun Zheng, Yipin Yu, Guohua Lv
Src-associated in mitosis of 68 kDa (Sam68), also known as KHDRBS1 (KH domain-containing, RNA-binding, signal transduction-associated 1), is a member of the signal transduction and activation of RNA family. Previous studies have demonstrated that the aberrant expression of Sam68 is associated with the progression and prognosis of a variety of cancers, but little is known about its expression and role in chordomas, which are rare and aggressive bone neoplasms. In this study, we analyzed 40 tumor tissues and 20 distant normal tissues obtained from 40 patients with sacral chordoma using immunohistochemistry, and observed the expression of Sam68 was significantly upregulated in sacral chordomas compared with normal tissues (P=0...
2017: OncoTargets and Therapy
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