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Chordomas

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https://www.readbyqxmd.com/read/29348851/comprehensive-analysis-of-mrna-lncrna-co-expression-profile-revealing-crucial-role-of-imprinted-gene-cluster-dlk1-meg3-in-chordoma
#1
Hao Chen, Kai Zhang, Jian Lu, Guizhong Wu, Huilin Yang, Kangwu Chen
Chordoma is a rare bone tumor with high recurrence rate, but the mechanism of its development is unclear. Long non-coding RNAs(lncRNAs) are recently revealed to be regulators in a variety of biological processed by targeting on mRNA transcription. Their expression profile and function in chordoma have not been investigated yet. In this study, we firstly performed the comprehensive analysis of the lncRNA and coding genes expression analysis with three chordoma samples and three fetal nucleus pulposus tissues...
December 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29327864/limits-of-endoscopic-endonasal-approach-for-cranio-vertebral-junction-tumors
#2
Matteo Zoli, Nicolò Rossi, Filippo Friso, Carmelo Sturiale, Giorgio Frank, Ernesto Pasquini, Diego Mazzatenta
INTRODUCTION: The endoscopic endonasal approach has been recently proposed for cranio-vertebral junction lesions. The more common indication for this sagittal extension of the endonasal route is represented by odontoidectomy for irreducible ventral brainstem compression due to congenital or degenerative conditions. However, in an increasing number of studies its adoption for tumors involving the cranio-cervical junction has been reported. The aim of this study is to consider retrospectively our surgical series, focusing on the advantages and limits of this approach...
January 10, 2018: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/29327709/insm1-expression-and-its-diagnostic-significance-in-extraskeletal-myxoid-chondrosarcoma
#3
Akihiko Yoshida, Naohiro Makise, Susumu Wakai, Akira Kawai, Nobuyoshi Hiraoka
Extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that affects the soft tissue and bones in middle-aged and elderly adults. Its diagnosis can be challenging, with the differential diagnoses including a wide variety of mesenchymal tumors. The line of differentiation of extraskeletal myxoid chondrosarcoma has been controversial, but recent evidence suggests a neuroendocrine phenotype. INSM1 is a zinc-finger transcription factor that plays a pivotal role in neuroendocrine differentiation, and has been proposed as a promising immunohistochemical marker of neuroendocrine carcinoma...
January 12, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29325600/clival-chordoma-with-brainstem-invasion
#4
Amit R L Persad, Bradford Mechor, Yves Starreveld
No abstract text is available yet for this article.
January 12, 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/29318761/suitability-of-the-cellienttm-cell-block-method-for-diagnosing-soft-tissue-and-bone-tumors
#5
W Song, B M van Hemel, A J H Suurmeijer
BACKGROUND: The diagnosis of tumors of soft tissue and bone (STB) heavily relies on histological biopsies, whereas cytology is not widely used. CellientTM cell blocks often contain small tissue fragments. In addition to Hematoxylin and Eosin (H&E) interpretation of histological features, immunohistochemistry (IHC) can be applied after optimization of protocols. The objective of this retrospective study was to see whether this cytological technique allowed us to make a precise diagnosis of STB tumors...
January 10, 2018: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29317233/c-cbl-and-cbl-b-expression-in-skull-base-chordomas-is-associated-with-tumor-progression-and-poor-prognosis
#6
Peng Luo, Xin Wang, Jinpeng Zhou, Long Li, Zhitao Jing
Chordomas are rare, locally aggressive malignancies that are often difficult to eradicate. Surgery and radiotherapy are the first-line treatments, but the probability of local recurrence is high. According to our previous research, c-Cbl and Cbl-b have been linked to tumor progression and poor prognosis of glioma. However, their role in skull base chordomas is unclear. To clarify this issue, in the present study we analyzed the expression of c-Cbl and Cbl-b in relation to the clinicopathological features and clinical outcome of skull base chordomas patients (n=70)...
January 6, 2018: Human Pathology
https://www.readbyqxmd.com/read/29313091/intradural-cervical-chordoma-with-diffuse-spinal-leptomeningeal-spread-case-report-and-review-of-the-literature
#7
Jing Zhang, Chuan-Ping Gao, Xue-Jun Liu, Wen-Jian Xu
PURPOSE: Chordoma is a low-grade malignant bone tumor derived from embryonic notochord remnants along the axial skeleton. About 50% of chordomas occur in the sacral vertebrae and 35% in the skull base. Most chordomas are extradural and cause extensive bone destruction. Intradural spinal tumors without bone involvement are rare. METHODS: We herein describe the clinical features of a patient with a chordoma as well as the imaging and pathological manifestations of the tumor...
January 8, 2018: European Spine Journal
https://www.readbyqxmd.com/read/29310903/proton-beam-therapy-for-skull-base-chordomas-in-106-patients-a-dose-adaptive-radiation-protocol
#8
Vivien Fung, Valentin Calugaru, Stéphanie Bolle, Hamid Mammar, Claire Alapetite, Philippe Maingon, Ludovic De Marzi, Sébastien Froelich, Jean-Louis Habrand, Rémi Dendale, Georges Noël, Loïc Feuvret
BACKGROUND AND PURPOSE: To evaluate clinical results and safety of a dose adaptive protocol based on tumor volume coverage and critical structure constraints, for the treatment of skull base chordomas. MATERIAL AND METHODS: Between May 2006 and October 2012, 106 patients with skull base chordoma were treated by combined photon and proton irradiation. Prescribed dose levels were 68.4, 70.2, 72 and 73.8 Gy(RBE) in once daily fractionation of 1.8 Gy(RBE). Dose level and dosimetric constraints to organs at risk depended on postoperative residual Gross Tumor Volume (GTV) coverage...
January 6, 2018: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/29310398/an-unusual-case-of-oropharyngeal-chordoma-a-case-report-and-literature-review
#9
Xiang Li, Yufan Wang, Feng Wang, Bowen Li, Shuai Sun, Hongyu Yang
RATIONALE: Chordomas are rare malignant neoplasms derived from incomplete regression of notochordal tissue along the cranio-coccygeal axis. Chordomas that develop in an atypical position are called ectopic chordomas, such as oropharyngeal chordomas (OCs). OCs are exceedingly rare; only 11 cases have been reported to date. Preoperative diagnosis is challenging, and an accurate diagnosis thus is based on postoperative pathologic examination findings and immunohistochemistry. Although surgical therapy and radiotherapy is performed in some patients, the 5-year survival rate is low...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29303447/analysis-of-clinical-factors-and-pdgfr-%C3%AE-in-predicting-prognosis-of-patients-with-clival-chordoma
#10
Yixuan Zhai, Jiwei Bai, Shuai Wang, Hua Gao, Mingxuan Li, Chuzhong Li, Songbai Gui, Yazhuo Zhang
OBJECTIVE In this study, the authors' aim was to research clinical features and prognostic factors in patients harboring clival chordomas and explore the relationship between platelet-derived growth factor receptor-β (PDGFR-β) expression and tumor invasion and prognosis of clival chordoma. METHODS A total of 242 patients were retrospectively analyzed. Clinical information, including extent of resection, Al-Mefty classification, postoperative complications, and postoperative radiotherapy, was reviewed. Kaplan-Meier analysis was used to estimate survival time...
January 5, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#11
Bruce R Pawel
The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT)...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29279794/surgical-nuances-of-partial-sacrectomy-for-chordoma
#12
Sergei Terterov, Daniel Diaz-Aguilar, Rudi Scharnweber, Alex Tucker, Tianyi Niu, Jos'lyn Woodard, Harsimran Brara, Melissa Poh, Catherine Merna, Stephanie Wang, Shayan Rahman
Background: Sacral chordomas are rare, slow growing, locally aggressive tumors. Unfortunately, aggressive surgical resection is often associated with increased neurological morbidity. Methods: This technical note focuses on the utilization of partial sacrectomy for the resection of complex spinal chordomas. Results: The case presented documents the potential range of postoperative morbidity seen in patients undergoing partial sacrectomy for chordomas...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/29279241/mri-evaluation-of-sacral-chordoma-treated-with-carbon-ion-radiotherapy-alone
#13
Lorenzo Preda, Davide Stoppa, Maria Rosaria Fiore, Giulia Fontana, Sofia Camisa, Roberto Sacchi, Michele Ghitti, Gisela Viselner, Piero Fossati, Francesca Valvo, Viviana Vitolo, Maria Bonora, Alberto Iannalfi, Barbara Vischioni, Alessandro Vai, Edoardo Mastella, Guido Baroni, Roberto Orecchia
BACKGROUND AND PURPOSE: To compare RECIST 1.1 with volume modifications in patients with sacral chordoma not suitable for surgery treated with carbon ions radiotherapy (CIRT) alone. To evaluate patients pain before and after CIRT. To detect if baseline Apparent Diffusion Coefficient values (ADC) from Diffusion Weighted sequences could predict response to treatment. MATERIAL AND METHODS: Patients included had one cycle of CIRT and underwent MRI before and after treatment...
December 23, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/29239140/acquired-myospherulosis-secondary-to-gluteal-augmentation-on-fine-needle-aspiration-cytology-a-diagnostic-challenge
#14
Susan Alperstein, Thomas Dilcher, Kartik Viswanathan, Rema A Rao, Momin T Siddiqui, Tamara Giorgadze
A 30-year-old female presented with a three-month history of a multilocular cystic lesion over the lumbosacral spine. Fine-needle aspiration biopsy (FNA) of the lesion was performed at an outside institution, and a cytologic diagnosis, suspicious for chordoma, was rendered. The patient presented for surgical consultation at our institution. Repeat FNA demonstrated an unusual fat-like material. Upon further inquiry, the patient provided a recent history of gluteal contour improvement with fibroadipose tissue implants...
December 14, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29237806/afatinib-is-a-new-therapeutic-approach-in-chordoma-with-a-unique-ability-to-target-egfr-and-brachyury
#15
Paola Magnaghi, Barbara Salom, Liviana Cozzi, Nadia Amboldi, Dario Ballinari, Elena Tamborini, Fabio Gasparri, Alessia Montagnoli, Laura Raddrizzani, Alessio Somaschini, Roberta Bosotti, Christian Orrenius, Fabio Bozzi, Silvana Pilotti, Arturo Galvani, Josh Sommer, Silvia Stacchiotti, Antonella Isacchi
Chordomas are rare bone tumors with no approved therapy. These tumors express several activated tyrosine kinase receptors, which prompted attempts to treat patients with tyrosine kinase inhibitors. Although clinical benefit was observed in Phase II clinical trials with imatinib and sorafenib, and sporadically also with EGFR inhibitors, therapies evaluated to date have shown modest activity. With the goal of identifying new drugs with immediate therapeutic potential for chordoma patients, we collected clinically approved drugs and other advanced inhibitors of MET, PDGFRβ and EGFR tyrosine kinases, and assessed their anti-proliferative activity against a panel of chordoma cell lines...
December 13, 2017: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/29236139/-notochordal-tumors-benign-notochordal-tumors-and-chordomas
#16
REVIEW
T F E Barth, A von Witzleben, P Möller, S Scheil-Bertram
Benign notochordal tumors (BNCT) and chordomas are primary bone tumors of the spine with a predominant localization in the sacrum and clival region followed by the vertebral bodies. Besides the most common variant (NOS [not otherwise specified] with hepatoid or renal carcinoma cell-like differentiation) chordomas with chondroid, and polymorphic to anaplastic morphology are described. An unfavorable variant are pediatric chordomas with a loss of INI-1. BNCT and chordomas are characterized by the following immunohistological profile: vimentin+, cytokeratin+/-, epithelial membrane antigen (EMA)+/-, S100 protein+/-, brachyury+...
December 13, 2017: Der Pathologe
https://www.readbyqxmd.com/read/29222701/snf5-as-a-prognostic-factor-in-skull-base-chordoma
#17
Mingxuan Li, Yixuan Zhai, Jiwei Bai, Shuai Wang, Hua Gao, Chuzhong Li, Songbai Gui, Jiang Du, Yazhuo Zhang
The current study aimed to characterize SNF5 expression and investigate the relationship between SNF5 and clinicopathological features in skull base chordoma. 48 patients diagnosed with skull base chordoma were enrolled in this study. Tissue microarray and immunohistochemistry were performed to evaluate the expression of SNF5 in skull base chordoma. Kaplan-Meier survival analysis was used to assess survival. Multivariable Cox regression analysis was used to identify risk factors affecting patient survival. The H-scores for cytoplasmic SNF5 ranged from 124...
December 8, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29220289/osteosarcoma-chondrosarcoma-and-chordoma
#18
Jeremy S Whelan, Lara E Davis
Osteosarcoma (OS), chondrosarcoma, and chordoma are characterized by multiple challenges to the investigator, clinician, and patient. One consequence of their rarity among sarcomas, as well as their biologic and clinical heterogeneity, is that management guidelines are inadequate to inform the range of individual patient-treatment decisions from diagnosis, approaches to surgery, chemotherapy, radiotherapy, treatment of recurrence, palliative care, and quality of survivorship. Of high-grade sarcomas, OSs are among the most curable, with more than two-thirds of patients with localized disease likely to achieve long-term survival...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29207720/locally-aggressive-primary-intraosseous-paraganglioma-of-sacrum
#19
Parampalli Srinivas Srilatha, Lakshmi Rao
Primary Intraosseous Paraganglioma (PGL) of sacrum is highly uncommon. Few of the spinal PGL reported were mostly intradural mass. Paraganglionic tissue is usually not present in the bone. So far, only seven cases of primary intraosseous sacral PGL have been reported in the literature. There are no dependable prognostic histological features to differentiate benign from malignant PGL. The only unequivocal criterion for malignancy is metastasis to an organ where paraganglionic tissue is normally not present...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29194728/cdk4-expression-in-chordoma-a-potential-therapeutic-target-running-title-cdk4-expression-in-chordoma
#20
Tang Liu, Jacson K Shen, Edwin Choy, Yu Zhang, Henry J Mankin, Francis J Hornicek, Zhenfeng Duan
Chordomas are rare bone tumors and treatment is commonly based on a combination of surgery and radiotherapy. There is no standard chemotherapy for treatment for chordoma. The aim of this study was to determine the expression of cyclin-dependent kinase 4 (CDK4) in chordoma and its therapeutic implications. We evaluated CDK4 expression both in chordoma cell lines and in chordoma tissues. Also, we investigated the functional roles of CDK4 in chordoma cell growth and proliferation. Furthermore, the therapeutic implications of targeting CDK4 in chordoma were evaluated...
November 30, 2017: Journal of Orthopaedic Research: Official Publication of the Orthopaedic Research Society
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