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Benjamin L Hoch, Gunnlaugur P Nielsen, Norbert J Liebsch, Andrew E Rosenberg
Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58%) were conventional chordomas, some of which had unusual histopathologic features...
July 2006: American Journal of Surgical Pathology
Gwi Hyun Choi, Moon-Sool Yang, Do Heum Yoon, Hyun Chyul Shin, Keung Nyun Kim, Seong Yi, Dong Yeop Lee, Poong Gi Ahn, Yoon Ha
INTRODUCTION: The majority of chordomas occur between the fifth and seventh decades of life and are thus extremely rare in children, which account for less than 5% of all spinal chordoma cases. CASE REPORT: We report on the development of this rare condition in two boys aged 7 and 10 years. One patient presented with a palpable neck mass and dysphagia, while the other presented with posterior neck pain. Radiological studies revealed an extensive mass in the cervical vertebrae and paravertebral soft tissue of both patients...
June 2010: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Maria Koutourousiou, Paul A Gardner, Matthew J Tormenti, Stephanie L Henry, Susan T Stefko, Amin B Kassam, Juan C Fernandez-Miranda, Carl H Snyderman
BACKGROUND: Gross total resection (GTR) of cranial base chordomas represents a surgical challenge because of the location, invasiveness, and tumor extension. In the past decade, the endoscopic endonasal approach (EEA) has been used with notable outcomes. OBJECTIVE: To present the endoscopic endonasal experience in the treatment of cranial base chordomas at our institution. METHODS: From April 2003 to March 2011, 60 patients underwent an EEA for primary (n = 35) or previously treated (n = 25) cranial base chordomas...
September 2012: Neurosurgery
K W Yeom, R M Lober, B C Mobley, G Harsh, H Vogel, R Allagio, M Pearson, M S B Edwards, N J Fischbein
BACKGROUND AND PURPOSE: Chordoma and chondrosarcoma of the skull base are rare tumors with overlapping presentations and anatomic imaging features but different prognoses. We hypothesized that these tumors might be distinguished by using diffusion-weighted MR imaging. MATERIALS AND METHODS: We retrospectively reviewed 19 patients with pathologically confirmed chordoma or chondrosarcoma who underwent both conventional and diffusion-weighted MR imaging. Differences in distributions of ADC were assessed by the Kruskal-Wallis test...
May 2013: AJNR. American Journal of Neuroradiology
Rajni Yadav, Mehar Chand Sharma, Prit Benny Malgulwar, Pankaj Pathak, Elanthenral Sigamani, Vaishali Suri, Chitra Sarkar, Amandeep Kumar, Manmohan Singh, Bhawani Shankar Sharma, Ajay Garg, Sameer Bakhshi, Mohammed Faruq
BACKGROUND: Chordomas are slow-growing tumors and most commonly involve the sacrum and clivus. Multiple recurrences are frequent. Childhood chordomas are rare and often show exceptionally aggressive behavior, resulting in short survival and a high incidence of metastatic spread. OBJECTIVE: This study examined the histologic features and immunohistochemical profile of pediatric chordomas and compared them with their adult counterparts. METHODS: Nine pediatric and 13 adult cases were included in the study...
March 2014: Neuro-oncology
Arman Jahangiri, Aaron T Chin, Jeffrey R Wagner, Sandeep Kunwar, Christopher Ames, Dean Chou, Igor Barani, Andrew T Parsa, Michael W McDermott, Arnau Benet, Ivan H El-Sayed, Manish K Aghi
BACKGROUND: Clival chordomas frequently recur because of their location and invasiveness. OBJECTIVE: To investigate clinical, operative, and anatomic factors associated with clival chordoma recurrence. METHODS: Retrospective review of clival chordomas treated at our center from 1993 to 2013. RESULTS: Fifty patients (56% male) with median age of 59 years (range, 8-76) were newly diagnosed with clival chordoma of mean diameter 3...
February 2015: Neurosurgery
Liang Wang, Kaibing Tian, Ke Wang, Junpeng Ma, Xiaojuan Ru, Jiang Du, Guijun Jia, Liwei Zhang, Zhen Wu, Junting Zhang
Skull base chordoma is a rare and fatal disease, recurrence of which is inevitable, albeit variable. We aimed to investigate the clinicopathologic features of disease progression, identify prognostic factors, and construct a nomogram for predicting progression in individual patients. Data of 229 patients with skull base chordoma treated by one institution between 2005 and 2014 were retrieved and grouped as primary and recurrent. Kaplan-Meier survival of progression was estimated, taking competing risks into account...
September 2016: Cancer Medicine
Songbai Gui, Xuyi Zong, Xinsheng Wang, Chuzhong Li, Peng Zhao, Lei Cao, Yazhuo Zhang
The aim of this study is to retrospectively analyze 161 cases of surgically treated skull base chordoma, so as to summarize the clinical classification of this tumor and the surgical approaches for its treatment via transnasal endoscopic surgery. Between August 2007 and October 2013, a total of 161 patients (92 males and 69 females) undergoing surgical treatment of skull base chordoma were evaluated with regard to the clinical classification, surgical approach, and surgical efficacy. The tumor was located in the midline region of the skull base in 134 cases, and in the midline and paramedian regions in 27 cases (extensive type)...
April 2016: Neurosurgical Review
Nicola Boari, Filippo Gagliardi, Andrea Cavalli, Marco Gemma, Luca Ferrari, Paola Riva, Pietro Mortini
OBJECTIVE Skull base chordomas (SBCs) are rare dysembryogenetic invasive tumors with a variable tendency for recurrence. According to previous studies, the recurrence rate seems to be affected by both clinical variables and tumor biological features. The authors present the results of treatment of SBCs in a large series of patients and investigate the role of 1p36 chromosomal region loss of heterozygosity (LOH) as a prognostic factor. METHODS Between 1990 and 2011, 45 patients were treated for SBCs. The mean follow-up was 76 months (range 1-240 months)...
August 2016: Journal of Neurosurgery
Andrew J Schoenfeld, Xinhui Wang, Yangyang Wang, Francis J Hornicek, G Petur Nielsen, Zhenfeng Duan, Soldano Ferrone, Joseph H Schwab
BACKGROUND: There are currently no generally accepted biomarkers used in the clinical treatment of chordoma tumors. CSPG4 has been associated with disease severity in other tumors. PURPOSE: This study aimed to characterize the frequency of CSPG4 expression in chordoma tumors and to correlate it with disease severity and clinical outcome. STUDY DESIGN: A retrospective review of clinical outcomes and immunohistochemical staining using tissue micro-array was carried out...
June 2016: Spine Journal: Official Journal of the North American Spine Society
Raewyn G Campbell, Daniel M Prevedello, Leo Ditzel Filho, Brad A Otto, Ricardo L Carrau
PURPOSE OF REVIEW: Clival chordomas are rare malignant tumors associated with a poor prognosis. In this article, we review the current literature to identify a variety of strategies that provide guidelines toward the optimal management for this aggressive tumor. RECENT FINDINGS: Molecular disease, particularly, the development of characterized chordoma cell lines, has become one of the new cornerstones for the histological diagnosis of chordomas and for the development of effective chemotherapeutic agents against this tumor...
April 2015: Current Opinion in Otolaryngology & Head and Neck Surgery
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