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Chordomas

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https://www.readbyqxmd.com/read/28797506/notochordal-tumors-an-update-on-molecular-pathology-with-therapeutic-implications
#1
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
#2
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
#3
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 2, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28777153/a-diagnostic-pitfall-atypical-teratoid-rhabdoid-tumor-versus-dedifferentiated-poorly-differentiated-chordoma-analysis-of-a-mono-institutional-series
#4
Anna Maria Buccoliero, Chiara Caporalini, Mirko Scagnet, Gianna Baroni, Selene Moscardi, Federico Mussa, Flavio Giordano, Iacopo Sardi, Lorenzo Genitori
Atypical teratoid/rhabdoid tumor (AT/RT) and dedifferentiated/poorly differentiated chordoma are pediatric tumors with some overlapping morphologic, immunohistochemical, and molecular features. Both these tumors have alterations in the tumor suppressor gene SMARCB1 resulting in loss of expression of the INI-1 protein. On the contrary, dedifferentiated/poorly differentiated chordoma expresses the transcription factor brachyury, whereas AT/RT does not. In this article we have reviewed the clinicopathologic features of a pediatric series of tumors (17 samples from 14 patients) located in the brain or within the axial spine and the base of the skull diagnosed as AT/RTs or as dedifferentiated/poorly differentiated chordomas...
August 2, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28776089/chordoma-arising-from-benign-multifocal-notochordal-tumors
#5
Abdulrehman Arain, Francis John Hornicek, Joseph H Schwab, Ivan Chebib, Timothy A Damron
This case reports a 25-year-old woman initially diagnosed with adjacent benign notochordal cell tumors (BNCTs) of L3 and L4 based on needle biopsy of L3 and stable imaging over a 3-year period who was ultimately found to have a chordoma arising from a BNCT at L3. It illustrates the potential relationship between benign and malignant notochordal tumors and the difficulty in distinguishing them by clinical, radiological, and even histopathological means.
August 3, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28766007/multicentric-chordoma-an-uncommon-and-incompletely-understood-presentation
#6
Jason R Jones, Anita Huttner, Ajay Malhotra
No abstract text is available yet for this article.
August 1, 2017: Clinical Neuroradiology
https://www.readbyqxmd.com/read/28765485/large-chordoma-of-the-sacrum
#7
Owen Godkin, Hussam Elkhwad, John McCabe
No abstract text is available yet for this article.
July 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28764925/profile-of-european-proton-and-carbon-ion-therapy-centers-assessed-by-the-eortc-facility-questionnaire
#8
Damien C Weber, André Abrunhosa-Branquinho, Alessandra Bolsi, Andrzej Kacperek, Rémi Dendale, Dirk Geismar, Barbara Bachtiary, Annika Hall, Jens Heufelder, Klaus Herfarth, Jürgen Debus, Maurizio Amichetti, Mechthild Krause, Roberto Orecchia, Vladimir Vondracek, Juliette Thariat, Tomasz Kajdrowicz, Kristina Nilsson, Cai Grau
BACKGROUND: We performed a survey using the modified EORTC Facility questionnaire (pFQ) to evaluate the human, technical and organizational resources of particle centers in Europe. MATERIAL AND METHODS: The modified pFQ consisted of 235 questions distributed in 11 sections accessible on line on an EORTC server. Fifteen centers from 8 countries completed the pFQ between May 2015 and December 2015. RESULTS: The average number of patients treated per year and per particle center was 221 (range, 40-557)...
July 29, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28761544/removal-of-a-low-clival-chordoma-in-a-teenager-by-dorsolateral-suboccipital-transcondylar-approach
#9
Deepal Attanayake, Buddhika Dias
Chordomas are rare midline tumors of the central nervous system which arise from the remnants of the primitive notochord and have unique diagnostic and management challenges. Although recommended treatment for chordoma is radical resection, this may require extended skull base approaches. We report a case of low clival chordoma in a teenage patient which was successfully treated by dorsolateral suboccipital transcondylar approach.
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28761532/skull-base-bony-lesions-management-nuances-a-retrospective-analysis-from-a-tertiary-care-centre
#10
Amit Kumar Singh, Arun Kumar Srivastava, Jayesh Sardhara, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Rabi Narayan Sahu, Awadhesh Kumar Jaiswal, Sanjay Behari
BACKGROUND: Skull base lesions are not uncommon, but their management has been challenging for surgeons. There is large no of bony tumors at the skull base which has not been studied in detail as a group. These tumors are difficult not only because of their location but also due to their variability in the involvement of important local structure. Through this retrospective analysis from a Tertiary Care Centre, we are summarizing the details of skull base bony lesions and its management nuances...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28761513/outcomes-following-surgical-management-of-cervical-chordoma-a-review-of-published-case-reports-and-case-series
#11
My Pham, Mohammed Awad
AIM: This review aimed to summarize the clinical outcomes in relation to tumor resection margins of cervical chordomas. METHODS: Studies that described any surgical intervention for cervical chordoma were identified. Cervical chordomas with cranial or spinal extension, purely retropharyngeal chordomas or where resection type was not reported, were excluded from the study. RESULTS: Seventy-six articles were obtained and these reported a total of 195 patients...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28758185/cystic-chordoma-of-cavernous-sinus
#12
Prashant Sathe, Amit Mahore, Aadil Chagla
Chordomas are the tumors commonly involving base of skull which are predominantly solid in consistency. A cystic chordoma of cavernous sinus is an extremely rare entity and has never been reported. We report a 26- year-old male presenting with gradually progressive right sided sixth nerve palsy and headache. Computerized tomography (CT) scan and Magnetic resonant imaging (MRI ) scan of brain showed a cystic lesion in right cavernous sinus.Patient was successfully treated using transnasal endoscopic trans-sphenoidal approach ...
June 14, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28758183/neuronavigation-guided-endoscopic-endonasal-excision-of-schwannoma-like-chordoma-of-the-meckel-s-cave-a-case-report
#13
Ali Akay, Sercan Göde, M Sedat Çağli
Chordomas are locally aggressive malignant tumors due to their recurrence potential, originating from the embryonic notochord remnants. Chordomas can originate anywhere on the axial skeleton. They are extradural and are spread by bone destruction. Chordomas are locally aggressive tumors which invade the dura mater, and may also present with secondary intradural growth. The Meckel's cave location of chordomas has been very rarely reported in the literature. Chordomas located in the Meckel's cave can be radiologically confused with trigeminal schwannomas...
June 14, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28757915/diagnosis-of-occipital-neuralgia-due-to-upper-cervical-chordoma
#14
EDITORIAL
Young Bok Lee
No abstract text is available yet for this article.
July 2017: Korean Journal of Pain
https://www.readbyqxmd.com/read/28753115/brachyury-gene-copy-number-gain-and-activation-of-the-pi3k-akt-pathway-association-with-upregulation-of-oncogenic-brachyury-expression-in-skull-base-chordoma
#15
Ryohei Otani, Akitake Mukasa, Masahiro Shin, Mayu Omata, Shunsaku Takayanagi, Shota Tanaka, Keisuke Ueki, Nobuhito Saito
OBJECTIVE Chordoma is a slow-growing but clinically malignant tumor, and the prognosis remains poor in many cases. There is a strong impetus to develop more effective targeted molecular therapies. On this basis, the authors investigated the potential of Brachyury, a transcription factor involved in notochord development, as a candidate molecular target for the treatment of chordoma. METHODS Brachyury gene copy number and expression levels were evaluated by quantitative polymerase chain reaction in 27 chordoma samples, and the transcriptomes of Brachyury high-expression tumors (n = 4) and Brachyury low-expression tumors (n = 4) were analyzed...
July 28, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28753113/a-potential-therapy-for-chordoma-via-antibody-dependent-cell-mediated-cytotoxicity-employing-nk-or-high-affinity-nk-cells-in-combination-with-cetuximab
#16
Rika Fujii, Jeffrey Schlom, James W Hodge
OBJECTIVE Chordoma is a rare bone tumor derived from the notochord and is resistant to conventional therapies such as chemotherapy, radiotherapy, and targeting therapeutics. Expression of epidermal growth factor receptor (EGFR) in a large proportion of chordoma specimens indicates a potential target for therapeutic intervention. In this study the authors investigated the potential role of the anti-EGFR antibody cetuximab in immunotherapy for chordoma. METHODS Since cetuximab is a monoclonal antibody of the IgG1 isotype, it has the potential to mediate antibody-dependent cell-mediated cytotoxicity (ADCC) employing natural killer (NK) cells as effectors...
July 28, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28750274/medical-treatment-of-advanced-chordomas
#17
REVIEW
Vittoria Colia, Silvia Stacchiotti
Chordoma is a very rare bone sarcoma that can arise from any site along the spine and from the skull base. Enbloc resection is the gold standard for treatment while radiation therapy has been shown to provide both curative and palliative benefit. Unfortunately, local recurrences are common, even after a complete surgical resection, and up to 40% of patients suffer from distant metastases, while salvage treatments are challenging. Patients carrying an advanced disease need a systemic treatment. Unluckily, conventional chordoma are insensitive to cytotoxic chemotherapy that is considered the standard treatment option in patients with metastatic sarcoma...
July 24, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28735379/multidisciplinary-management-of-clival-chordomas-long-term-clinical-outcome-in-a-single-institution-consecutive-series
#18
Petter Förander, Jiri Bartek, Michael Fagerlund, Hamza Benmaklouf, Ernest Dodoo, Alia Shamikh, Pär Stjärne, Tiit Mathiesen
OBJECTIVE: Chordomas of the skull base have high recurrence rates even after radical resection and adjuvant radiotherapy. We evaluate the long-term clinical outcome using multidisciplinary management in the treatment of clival chordomas. METHODS: Between 1984 and 2015, 22 patients diagnosed with an intracranial chordoma were treated at the Karolinska University Hospital, Stockholm, Sweden. Sixteen of 22 were treated with Gamma Knife radiosurgery (GKRS) for tumour residual or progression during the disease course...
July 22, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28730223/histopathological-diagnosis-and-its-correlations-with-anatomoclinical-features-surgical-approach-and-postoperative-prognosis-in-sacral-tumors
#19
Oana Ilona David, Corina Veronica Lupaşcu-Ursulescu, Cristian Dumitru Lupaşcu, Aurelia Mihaela Sandu, Victor Dan Eugen Strâmbu, Daniel Alin Cristian, Ştefan Iulian Bedereag, Irina Alexandra Calangea, Elena Violeta Radu, IonuŢ Simion Coman, Vlad Andrei Porojan, Valentin Titus Grigorean
INTRODUCTION: Sacral tumors encompass numerous histopathological types. They represent an uncommon pathology and, when diagnosed, they are often in advanced stage of the disease, becoming a therapeutic challenge. The correct treatment of a sacral tumor should be established by a multidisciplinary team that will assess the exact anatomical, imagistic and histopathological characteristic of the tumors thus choosing an optimal surgical approach while taking into consideration the risk of recurrence...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28720745/-a-case-of-intradural-retroclival-chordoma
#20
Hiroki Takahashi, Yasuyuki Kinoshita, Satoshi Usui, Koji Arihiro, Shuji Kimura, Takafumi Mitsuhara, Kaoru Kurisu
A 67-year-old woman was referred to our department with a retroclival lesion including a cyst on MRI. MRI revealed a lesion appearing as an isointense region on a diffusion-weighted image(DWI). Gadolinium(Gd)-DTPA T1-WI showed heterogeneous enhancement of the lesion in the prepontine cistern. Computed tomography(CT)revealed an isodense lesion with no invasion into the clival bone. Based on a preoperative diagnosis of retroclival chordoma, extended trans-sphenoidal surgery(TSS)was performed by a direct endoscopic endonasal approach via the left nostril...
July 2017: No Shinkei Geka. Neurological Surgery
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