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Chordomas

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https://www.readbyqxmd.com/read/28919999/first-report-of-clinical-responses-to-immunotherapy-in-3-relapsing-cases-of-chordoma-after-failure-of-standard-therapies
#1
Denis Migliorini, Nicolas Mach, Diego Aguiar, Rémi Vernet, Basile Nicolas Landis, Minerva Becker, Thomas McKee, Valérie Dutoit, Pierre-Yves Dietrich
Chordoma is a rare tumor of notochordal origin, currently principally treated by surgery and/or irradiation. Here, we describe the clinical outcome of 3 consecutive patients with metastatic and locally advanced chordoma, treated with different immunotherapeutic approaches. All patients presented fast growing tumors and failure of standard therapies. One was treated with a tumor-based vaccine, the 2 others with anti-PD1 antibodies, all with impressive clinical and radiological responses. We therefore propose that chordoma is an immunogenic tumor and thus that translational and clinical research is necessary to develop rationally designed immunotherapy approaches...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28914872/-the-efficacy-of-desmopressin-in-the-treatment-of-central-diabetes-insipidus-after-resection-of-chiasmo-sellar-region-tumors
#2
L I Astaf'eva
Central diabetes insipidus (CDI) is a neuroendocrine disease, the pathogenesis of which is associated with abnormal secretion of the antidiuretic hormone. One of the specific causes of CDI is neurosurgical resection of chiasmatic-sellar region tumors. AIM: to study the efficacy and safety of desmopressin in CDI patients after resection of chiasmatic-sellar region (CSR) tumors. MATERIAL AND METHODS: Examination and treatment of patients were performed at a hospital for 7-14 days after surgery and then were continued after discharge...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28914866/-extended-endoscopic-endonasal-posterior-transclival-approach-to-tumors-of-the-clival-region-and-ventral-posterior-cranial-fossa-part-1-topographic-and-anatomical-features-of-the-clivus-and-adjacent-structures
#3
A N Shkarubo, K V Koval', G F Dobrovol'skiy, M A Shkarubo, V V Karnaukhov, B A Kadashev, D N Andreev, I V Chernov, O A Gadzhieva, O Yu Aleshkina, E A Anisimova, P L Kalinin, M A Kutin, D V Fomichev, O I Sharipov, D B Ismailov, E S Selivanov
OBJECTIVE: to describe the main topographic and anatomical features of the clival region and its adjacent structures for improvement and optimization of the extended endoscopic endonasal posterior (transclival) approach for resection of tumors of the clival region and ventral posterior cranial fossa. MATERIAL AND METHODS: We performed a craniometric study of 125 human skulls and a topographic anatomical study of heads of 25 cadavers, the arterial and venous bed of which was stained with colored silicone (the staining technique was developed by the authors) to visualize bed features and individual variability...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28912284/t1-weighted-dynamic-contrast-enhanced-mr-perfusion-imaging-characterizes-tumor-response-to-radiation-therapy-in-chordoma
#4
P Santos, K K Peck, J Arevalo-Perez, S Karimi, E Lis, Y Yamada, A I Holodny, J Lyo
BACKGROUND AND PURPOSE: Chordomas notoriously demonstrate a paucity of changes following radiation therapy on conventional MR imaging. We hypothesized that dynamic contrast-enhanced MR perfusion imaging parameters of chordomas would change significantly following radiation therapy. MATERIALS AND METHODS: Eleven patients with pathology-proved chordoma who completed dynamic contrast-enhanced MR perfusion imaging pre- and postradiation therapy were enrolled. Quantitative tumor measurements were obtained by 2 attending neuroradiologists...
September 14, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28904574/multicentric-chordoma-in-a-child
#5
Shighakolli Ramesh, Raju Subodh, Srinadh Boppana, Erukkambattu Jayashankar
Chordomas are primary malignant bone tumors that arise in the axial skeleton, believed to originate from remnants of embryologic notochordal cell rests. Multicentric origin of chordoma is extremely rare. To our literature search, we found only three cases of multicentric chordoma in adults. We report a first case of multicentric chordoma in pediatric age group. A 14-month-old child presented with torticolis and left upper limb monoparesis, imaging showed expansile bony destructive lesion in clivus and dorsal spine simultaneously...
April 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28889042/marginal-en-bloc-resection-of-c2-3-chordoma-with-bilateral-vertebral-artery-preservation-and-mesh-cage-reconstruction-with-review-of-previously-published-cases
#6
Steven O Tenny, Landon D Ehlers, J Will Robbins, Christopher C Gillis
BACKGROUND: Chordomas arise from remnants of the notocord and occur throughout the neuroaxis. En bloc resection of chordomas can prove especially challenging in the upper cervical spine secondary to the unique structural anatomy and intimate relationship to the vertebral arteries and spinal cord. CASE DESCRIPTION: We describe the resection of a C2-3 chordoma in a 55 year old female in two stage fashion with preservation of the vertebral arteries. First a posterior instrumented fusion and removal of the posterior elements was performed, followed by an anterior transmandibular en bloc resection with cage reconstruction with kick-plate support achievable with off-the-shelf products...
September 6, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28883093/role-of-the-ecm-in-notochord-formation-function-and-disease
#7
REVIEW
Valeria Trapani, Paolo Bonaldo, Diana Corallo
The notochord is a midline structure common to all chordate animals; it provides mechanical and signaling cues for the developing embryo. In vertebrates, the notochord plays key functions during embryogenesis, being a source of developmental signals that pattern the surrounding tissues. It is composed of a core of vacuolated cells surrounded by an epithelial-like sheath of cells that secrete a thick peri-notochordal basement membrane made of different extracellular matrix (ECM) proteins. The correct deposition and organization of the ECM is essential for proper notochord morphogenesis and function...
September 7, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28882523/role-of-intraoperative-radiotherapy-in-the-treatment-of-sacral-chordoma
#8
A C Jullien-Petrelli, J M Asencio, M I Orue-Echebarria, P Lozano, A Álvarez, J Serrano, F M Calvo, J A Calvo-Haro, J M Lasso, J L García-Sabrido
BACKGROUND CONTEXT: Sacral chordoma is a rare entity with high local recurrence rates when complete resection is not achieved. Till date, there are not any series available in literature combining surgery and intraoperative radiotherapy (IORT). PURPOSE: To report the experience of our Centre in the management of sacral chordoma combining radical resection with both external radiotherapy and intraoperative radiotherapy (IORT). STUDY DESIGN: Retrospective case series...
September 4, 2017: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/28872530/modified-en-bloc-spondylectomy-for-tumors-of-the-thoracic-and-lumbar-spine-surgical-technique-and-outcomes
#9
Akash A Shah, Nuno R Paulino Pereira, Frank X Pedlow, John C Wain, Sam S Yoon, Francis J Hornicek, Joseph H Schwab
BACKGROUND: Total en bloc spondylectomy (TES) for the treatment of spinal tumors decreases local recurrence and improves survival compared with intralesional resection. TES approaches vary in both the number of stages to complete the procedure and instruments with which osteotomies are performed. METHODS: We describe a 2-stage technique that employs the use of threadwire saws. We performed a retrospective review of cases of primary tumors and solitary metastases involving the thoracic or lumbar spine treated with use of our modified technique at our institution between 2010 and 2016, identifying eligible patients by searching for specific phrases in operative reports found in our oncologic database...
September 6, 2017: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/28866065/intradural-chordoma-of-the-cerebellopontine-angle-case-report-and-review
#10
Amir Goodarzi, Atrin Toussi, Mirna Lechpammer, Darrin Lee, Kiarash Shahlaie
No abstract text is available yet for this article.
August 30, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28859544/chordomas-technologies-techniques-and-treatment-strategies
#11
Tony Van Havenbergh
No abstract text is available yet for this article.
August 31, 2017: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/28853064/receptor-activator-of-nuclear-transcription-factor-nf-%C3%AE%C2%BAb-rank-its-ligand-rankl-and-natural-inhibitor-of-rankl-osteoprotegerin-opg-in-the-blood-serum-of-patients-with-primary-bone-tumors
#12
N E Kushlinskii, E S Gershtein, Yu N Solov'ev, Yu S Timofeev, I V Babkina, A O Dolinkin, A A Zuev, O I Kostyleva
The content of components of the RANK/RANKL/OPG system, the key regulator of homeostasis in the bone tissue, in blood serum samples from 199 patients with primary bone neoplasms and 131 practically healthy volunteers was measured by ELISA. Borderline giantcell tumor of the bone with high osteoclastogenic and osteolytic activity is characterized by an increase in the level of all components of this system and highest ratio of sRANKL/OPG in the blood serum. Study indexes in patients with various benign neoplasms and tumor-like bone lesions were lower than in patients with giant-cell tumor...
August 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28844110/transcriptome-comparison-identifies-potential-biomarkers-of-spine-and-skull-base-chordomas
#13
Achim H Bell, Franco DeMonte, Shaan M Raza, Laurence D Rhines, Claudio E Tatsui, Victor G Prieto, Gregory N Fuller, Diana Bell
Chordomas are rare, slowly growing, locally aggressive bone neoplasms that arise from embryonic remnants of the notochord, showing dual epithelial-mesenchymal differentiation. The high plasticity probably is the main reason for the high variety in phenotypes of chordoma, from its high heterogeneity on a cellular level to its subtype variations depending on tissue location, with its potential to develop from an inactive quiescent form to an aggressive cancer with extreme adaptability and resistance to drugs and other treatments...
August 27, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28838115/endoscopic-endonasal-approach-to-the-ventral-jugular-foramen-anatomical-basis-technical-considerations-and-clinical-series
#14
Francisco Vaz-Guimaraes, Ana Carolina I Nakassa, Paul A Gardner, Eric W Wang, Carl H Snyderman, Juan C Fernandez-Miranda
BACKGROUND: Surgical exposure of the jugular foramen (JF) is challenging given its complex regional anatomy and proximity to critical neurovascular structures. OBJECTIVE: To describe the anatomical basis, surgical technique, and outcomes of a group of patients who underwent the endoscopic endonasal approach to the JF. METHODS: Five silicon-injected anatomical specimens were prepared for dissection. Additionally, a chart review was conducted through our patient database, searching for endonasal exposure of the JF...
August 1, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/28835717/establishment-and-genomic-characterization-of-the-new-chordoma-cell-line-chor-in-1
#15
Roberta Bosotti, Paola Magnaghi, Sebastiano Di Bella, Liviana Cozzi, Carlo Cusi, Fabio Bozzi, Nicola Beltrami, Giovanni Carapezza, Dario Ballinari, Nadia Amboldi, Rosita Lupi, Alessio Somaschini, Laura Raddrizzani, Barbara Salom, Arturo Galvani, Silvia Stacchiotti, Elena Tamborini, Antonella Isacchi
Chordomas are rare, slowly growing tumors with high medical need, arising in the axial skeleton from notochord remnants. The transcription factor "brachyury" represents a distinctive molecular marker and a key oncogenic driver of chordomas. Tyrosine kinase receptors are also expressed, but so far kinase inhibitors have not shown clear clinical efficacy in chordoma patients. The need for effective therapies is extremely high, but the paucity of established chordoma cell lines has limited preclinical research...
August 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28822420/-a-case-report-of-chordoma-in-the-jugular-foramen-area
#16
X W Wang, S H Jiang
No abstract text is available yet for this article.
August 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28812319/poorly-differentiated-chordoma-with-loss-of-smarcb1-ini1-expression-in-pediatric-patients-a-report-of-two-cases-and-review-of-the-literature
#17
Yoon Jin Cha, Chang-Ki Hong, Dong-Seok Kim, Seung-Koo Lee, Hyeon Jin Park, Se Hoon Kim
Identification of loss of SMARCB1/INI1 expression in poorly differentiated (PD) chordoma in pediatric patients suggests that PD chordoma is an entity molecularly distinct from conventional chordoma or atypical teratoid/rhabdoid tumor, which is also characterized by loss of SMARCB1/INI1 expression by inactivating mutation of the SMARCB1/INI gene. So far, around 20 cases of pediatric PD chordoma with loss of SMARCB1/INI1 expression have been reported. Here, we report two cases of pediatric PD chordoma with loss of SMARCB1/INI1 expression, which is very rare among the pediatric chordoma types...
August 15, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28797506/notochordal-tumors-an-update-on-molecular-pathology-with-therapeutic-implications
#18
REVIEW
Takehiko Yamaguchi, Hiroki Imada, Shun Iida, Karoly Szuhai
Recent molecular investigations of chordoma show common expression of various receptor tyrosine kinases and activation of downstream signaling pathways contributing to tumor growth and progression. The transcription factor brachyury (also known as T) is important in notochord differentiation, and germline duplication of the gene is often found in familial chordomas. Nuclear expression of brachyury is consistent in chordoma and in benign notochordal cell tumor. Based on the molecular evidence, targeting of several kinds of molecular agents has been attempted for the treatment of uncontrolled chordomas and achieved partial response or stable condition in many cases...
September 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28791543/chordoma
#19
EDITORIAL
Ossama Al-Mefty
No abstract text is available yet for this article.
August 8, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28780352/expression-of-far-upstream-element-binding-protein-1-correlates-with-c-myc-expression-in-sacral-chordomas-and-is-associated-with-tumor-progression-and-poor-prognosis
#20
Hai Wen, Hong Ma, Pengzhi Li, Jiaoyun Zheng, Yipin Yu, Guohua Lv
The far upstream element (FUSE)-binding protein 1 (FUBP1), a well-known transcriptional regulator of the proto-oncogene c-Myc, has been demonstrated by previous work to be aberrantly expressed in a variety of tumors and plays a critical role in tumor progression; however, its expression and function in relatively rare and aggressive chordomas remains unclear. In this retrospective study, we reviewed clinicopathologic characteristics of 40 patients diagnosed with sacral chordoma, and analyzed 40 tumor and 20 distant normal tissues obtained from patients during the primary surgical tumor excision...
August 3, 2017: Biochemical and Biophysical Research Communications
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