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World Neurosurg 2019 Apr 16. Epub 2019 Apr 16.

International Neuroscience Institute Rudolf-Pichlmayr-Straße 4 D - 30625 Hannover Germany.

Background: Skull base chordoma could be a challenging surgical entity due to its invasive nature.

Objective: In this study, we are evaluating the role of intraoperative magnetic resonance imaging to optimize the resection of skull base chordomas METHODS: We performed a retrospective analysis of the operated patients with skull base chordomas in the setting of iMRI. The clinical records, operative notes, radiological images, tumor volumetry, location of the residual tumor, and surgical outcome have been evaluated. Read More

World Neurosurg 2019 Apr 14. Epub 2019 Apr 14.

Mayo Clinic Neuro-Informatics Laboratory, Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA; Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA. Electronic address:

Introduction: Chordomas are slow-growing but locally invasive tumors. Standard of care consists of surgical resection and radiotherapy when complete resection is not possible. Current literature has reached equivocal results with regards to the effect of adding radiotherapy on patient survival. Read More

World Neurosurg 2019 Apr 14. Epub 2019 Apr 14.

Department of Neurosurgery, the First Affiliated Hospital of Fujian Medical University, Fuzhou, People's Republic of China.

Background: Chondrosarcoma is a malignant tumor that originates from mesenchymal cells that have differentiated into chondrocytes, often growing laterally, rarely seen in the cranium, and seldom seen in the saddle area. We believe that only a few cases have been reported in the literature. We report a case of pituitary fossa chondrosarcoma, which was completely resected by an extended endoscopic endonasal approach, and a literature review. Read More

Global Spine J 2019 Apr 30;9(2):191-201. Epub 2018 May 30.

The Johns Hopkins University, Baltimore, MD, USA.

Study Design: Systematic review.

Objectives: Sacral chordomas are rare, primary tumors of the spine, best treated with en bloc resection. The purpose of this study was to assess the literature for resected sacral chordoma and to quantify the prevalence of, risk factors for, and treatment outcomes of local and distant recurrence therein. Read More

Oper Neurosurg (Hagerstown) 2019 May;16(5):557-570

Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

Background: The endoscopic endonasal approach (EEA) was recently added to the neurosurgical armamentarium as an alternative approach to the petrous apex (PA) region. However, the maximal extension, anatomical landmarks, and indications of this procedure remain to be established.

Objective: To investigate the limitations and suggest a classification of PA lesions for endoscopic petrosectomy. Read More

Eur J Pharmacol 2019 Apr 10. Epub 2019 Apr 10.

Department of Orthopedics, China-Japan Union Hospital, Jilin University, Changchun, 130033, PR China.

Previous researches suggested microRNA-140-3p (miR-140-3p) and miR-155-5p as cancer promotor in chordoma. We aimed to investigate the mechanisms of these two miRNAs in chordoma cells. Patient-derived chordoma cell lines were established in vitro. Read More

J Neurosurg Pediatr 2019 Apr 12:1-4. Epub 2019 Apr 12.

1Section of Pediatric Neurosurgery, Riley Hospital for Children, Department of Neurological Surgery, Indiana University School of Medicine, Goodman Campbell Brain and Spine, Indianapolis, Indiana; and.

Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Read More

Neuro Oncol 2019 Apr 12. Epub 2019 Apr 12.

Department of Neurosurgery, MD Anderson Cancer Center, The University of Texas, Houston, Texas.

Background: Spinal chordomas, a sub-type of primary spinal column malignancies (PSCM), are rare tumors with poor prognosis and limited understanding of the molecular drivers of neoplasia.

Methods: Study design was a retrospective review of prospectively collected data with cross-sectional survival. Archived paraffin embedded pathologic specimens were collected for 133 patients from 6 centers within Europe and North America between 1987 and 2012. Read More

J Med Chem 2019 Apr 19. Epub 2019 Apr 19.

UNC Catalyst for Rare Diseases , University of North Carolina at Chapel Hill , Chapel Hill , North Carolina 27599 , United States.

We describe the design of a set of inhibitors to investigate the relationship between cyclin G associated kinase (GAK) and epidermal growth factor receptor (EGFR) in chordoma bone cancers. These compounds were characterized both in vitro and using in cell target engagement assays. The most potent chordoma inhibitors were further characterized in a kinome-wide screen demonstrating narrow spectrum profiles. Read More

Indian J Pathol Microbiol 2019 Apr-Jun;62(2):287-289

Department of Pathology, The First Affiliated Hospital of Zhengzhou University; Department of Pathology, Basic Medicine College of Zhengzhou University, Zhengzhou, China.

A unique case of eyelid metastasis from nasopharyngeal chondroid chordoma in a 63-year-old woman was reported. Chordomas are rare tumors of the bone deriving from remnants of the embryonic notochord. Histologically, the tumor showed lobulated structure and concludes two types of cells: liquid drop cell and small round/cubic cell. Read More

Head Neck 2019 Apr 10. Epub 2019 Apr 10.

Department of Otorhinolaryngology-Head and Neck Surgery, University of Pennsylvania Health System, Philadelphia, Pennsylvania.

Background: In this paper, we present three cases where a novel transoral robotic surgery (TORS) approach was used to resect clival chordomas extending into the nasopharynx to demonstrate progress in the evolution of robotic surgery of the head and neck.

Methods: The first patient had a history of metastatic chordoma resection and presented with trismus and dysphagia; the second and third patients had first presentation with nasal congestion and facial numbness, respectively. All patients underwent a novel transpalatal-TORS resection. Read More

Mol Clin Oncol 2019 May 5;10(5):487-491. Epub 2019 Mar 5.

Musculoskeletal Oncology Service, Osaka International Cancer Institute, Osaka, Osaka 541-8567, Japan.

Chordoma is a rare tumor that originates from the notochord. Half of chordomas involve the sacral region. Surgery is considered to be the standard treatment for sacral chordoma. Read More

Nat Commun 2019 04 9;10(1):1635. Epub 2019 Apr 9.

German Cancer Consortium (DKTK), 69120, Heidelberg, Germany.

Chordomas are rare bone tumors with few therapeutic options. Here we show, using whole-exome and genome sequencing within a precision oncology program, that advanced chordomas (n = 11) may be characterized by genomic patterns indicative of defective homologous recombination (HR) DNA repair and alterations affecting HR-related genes, including, for example, deletions and pathogenic germline variants of BRCA2, NBN, and CHEK2. A mutational signature associated with HR deficiency was significantly enriched in 72. Read More

J Oncol Pharm Pract 2019 Apr 8:1078155219841108. Epub 2019 Apr 8.

2 Division of Hematology, Oncology, and Blood and Marrow Transplantation, University of Iowa Holden Comprehensive Cancer Center, Iowa City, IA, USA.

Cancer treatment has changed dramatically with the development of oral targeted therapies. Pazopanib, an oral VEGF tyrosine kinase inhibitor, is currently approved for advanced renal cell carcinoma, advanced soft tissue sarcoma, and is being studied for various tumor types. Due to the potential of increased exposure to pazopanib when crushed, pazopanib should be given as an intact whole tablet. Read More

Int Forum Allergy Rhinol 2019 Apr 8. Epub 2019 Apr 8.

Department of Otolaryngology, School of Medicine, University of North Carolina,, Chapel Hill, NC.

Background: Endoscopic skull-base surgery (ESBS) is a rapidly growing discipline that often combines the expertise of otolaryngology-head and neck surgeons and neurosurgeons to provide care for these challenging conditions. As the field has grown, so too has the literature evaluating this surgical approach and the challenges and complications associated with these interventions. An international, multidisciplinary effort to critically evaluate and grade the current literature on ESBS has resulted in the International Consensus Statement on Endoscopic Skull Base Surgery (ICAR:SB). Read More

Int J Colorectal Dis 2019 Apr 5. Epub 2019 Apr 5.

Division of Colorectal Surgery, University Surgical Cluster, National University Health System, 1E Kent Ridge Road, Singapore, 119228, Singapore.

Objective: Our study seeks to describe our surgical technique of the use of a tissue expander and a pelvic sling in order to perform high-dose pelvic irradiation without incurring radiation toxicity to the small bowel. High-dose radiation therapy for pelvic tumours comes at a risk of radiation toxicity to the small bowel. Our study discusses our novel surgical technique of compartmentalising the abdomen and the pelvis through the use of a tissue expander and pelvic sling to avoid small bowel radiation toxicity. Read More

Exp Cell Res 2019 Apr 2. Epub 2019 Apr 2.

Department of Medical Genetics, Yeditepe University Medical School, 34755, Istanbul, Turkey. Electronic address:

Chordoma is a rare, slow-growing tumor thought to arise from remnants of embryonic notochord associated with an aggressive outcome. Cancer stem-like cells (CSCs) are related to tumorigenesis, recurrence, and resistance in cancers. Therefore, chordoma CSCs are possible targets for chordoma treatment. Read More

J Korean Med Sci 2019 Apr 8;34(13):e107. Epub 2019 Apr 8.

Department of Pathology, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, Korea.

Background: Chordomas are aggressive bone tumors that have a predilection for the axial skeleton including the skull base and spinal/sacral bones. However, the histopathological and clinical differences between skull base chordoma (SBC) and sacral/spinal chordoma (SC) are unclear as previous studies have been focused on patient prognosis and treatment outcome. This study aimed to evaluate the clinicopathologic features and prognosis of chordoma according to its location. Read More

Br J Radiol 2019 Apr 3:20180883. Epub 2019 Apr 3.

1 Center for Proton Therapy, Paul Scherrer Institute, ETH Domain, Villigen PSI , Switzerland.

Objectives: : Large inoperable sacral chordomas show unsatisfactory local control rates even when treated with high dose proton therapy (PT). The aim of this study is assessing feasibility and reporting early results of patients treated with PT and concomitant hyperthermia (HT).

Methods: : Patients had histologically proven unresectable sacral chordomas and received 70Gy(RBE) in 2. Read More

J Belg Soc Radiol 2018 Sep 6;102(1):56. Epub 2018 Sep 6.

Institut Kassab of Orthopaedics, TN.

Primary malignant tumors of the spine are rare and mainly include chordoma, chondrosarcoma, Ewing sarcoma or primitive neuroectodermal tumor, and osteosarcoma. The final diagnosis is based on the combination of patient age, topographic and histologic features of the tumor, and lesion pattern on computed tomography (CT) and magnetic resonance (MR) imaging. Imaging evaluation includes radiography, CT, bone scintigraphy, and MR imaging. Read More

Auris Nasus Larynx 2019 Mar 20. Epub 2019 Mar 20.

Departments of Otolaryngology and Head and Neck Surgery, Graduate School of Biomedical & Health Sciences, and Applied Life Sciences, Japan.

Background: Extra-axial chordomas are exceedingly rare malignant tumours. Herein, we present the first case of an extra-axial chordoma in the gingivae.

Methods And Results: A 21-year-old man presented with a suspected chordoma in the upper right gingiva. Read More

J Clin Neurosci 2019 Mar 19. Epub 2019 Mar 19.

Neurosurgery Department, DFV Neuro, São Paulo, Brazil; Hospital Sírio Libanês, São Paulo, Brazil; Hospital Alemão Oswaldo Cruz, São Paulo, Brazil. Electronic address:

Introduction: Chordoma is a malignant tumor that usually involves the axial skeleton. Intradural chordomas are even rarer and 37 cases have been reported to the best of our knowledge. We present a case of a patient with an atypical metastatic diffuse intradural spinal involvement. Read More

Zh Vopr Neirokhir Im N N Burdenko 2019;83(1):67-74

Burdenko Neurosurgical Institute, Moscow, Russia.

Sacral tumors are a group of neoplasms heterogeneous in the histological type, malignancy, and growth pattern, but with common localization. Surgical treatment of these tumors is associated with the risk of major and minor complications, both during and after surgery. Usually, any surgery aimed at removing tumors in the sacrum or sacral region is associated with the need for reconstruction of bones and/or soft tissues to provide conditions for normal wound healing and the possibility of activating the patient. Read More

Eur Spine J 2019 Mar 21. Epub 2019 Mar 21.

Unit of Oncologic and Degenerative Spine Surgery, Rizzoli Institute, Bologna, Italy.

Purpose: Primary vascular bone tumors of the spine represent a challenge for oncology surgeons, due to the need of planning a surgical strategy appropriate to the tumor behavior. But these tumors represent a challenge also for pathologists, as immunohistochemical and molecular analyses have recently refined the terminology.

Methods: A cohort of 81 cases was retrospectively reviewed, targeting the evolution of diagnoses and the treatment-related outcome. Read More

Neurosurgery 2019 Mar 20. Epub 2019 Mar 20.

Department of Pathology, The Second Xiangya Hospital, Central South University, Changsha, China.

Background: Currently, clinical characteristics and prognostic factors of extra-axial chordoma (EAC) remain poorly understood.

Objective: To characterize clinicopathological characteristics in a large EAC cohort and investigate their correlation with survival. We also attempted to compare these outcomes with axial chordoma (AC). Read More

Plast Reconstr Surg Glob Open 2018 Nov 5;6(11):e2002. Epub 2018 Nov 5.

USC Division of Plastic and Reconstructive Surgery, Los Angeles, Calif.

Background: Sacral pathology requiring partial or total sacrectomy is rare, and reconstructing the ensuing defects requires careful decision-making to minimize morbidity. The purpose of this study was to review the experience of a single institution with reconstructing large sacral defects, to identify risk factors for suboptimal outcomes.

Methods: A retrospective chart review was conducted of all patients who underwent sacrectomy over a 10-year period. Read More

J Neurosurg 2019 Mar 15:1-3. Epub 2019 Mar 15.

J Neurosurg Spine 2019 Mar 1:1-6. Epub 2019 Mar 1.

1University of Pennsylvania Perelman School of Medicine, Department of Neurological Surgery.

Chordomas are rare, locally aggressive neoplasms that develop from remnants of the notochord. The typical approach to chordomas of the clivus and axial cervical spine often limits successful en bloc resection. In this case report, authors describe the first-documented transoral approach using both transoral robotic surgery (TORS) for exposure and the Sonopet bone scalpel under navigational guidance to achieve en bloc resection of a cervical chordoma. Read More

Zhonghua Bing Li Xue Za Zhi 2019 Mar;48(3):199-203

Department of Pathology, Henan Provincial People's Hospital, Zhengzhou 450003, China.

To investigate the histological type and clinicopathological characteristics of the craniocerebral slope tumors with chondromucinous features. Retrospective analysis was conducted to analyze chondromucinous tumors in the slope area diagnosed at Henan Provincial People's Hospital from October 2011 to June 2018. Relevant clinical and pathological data were reviewed, and immunohistochemistry was used to investigate the immunophenotype of the tumors. Read More

Nat Rev Drug Discov 2019 Mar;18(3):174

Neurol Res 2019 Mar 1:1-8. Epub 2019 Mar 1.

a Department of Neurosurgery , West China Hospital, Sichuan University , Chengdu , Sichuan , China.

Objective: Chordoma is a rare tumor with a certain rate of distant metastasis. Skull base and sacrum are the two most common origin sites. This study tends to identify key differentially expressed genes (DEGs) between classical clival and sacral chordomas, provide new targets for future treatment options of chordomas. Read More

Cureus 2018 Dec 24;10(12):e3772. Epub 2018 Dec 24.

Neurosurgery, Seattle Science Foundation, Seattle, USA.

Chordomas are primary low-grade bone tumors derived from the embryonic notochord that make up less than 5% of all osseous malignancies and commonly affect the spine at its vertebral body and at its two ends i.e., skull base and the sacrum. Read More

Turk J Biol 2018 9;42(4):279-285. Epub 2018 Aug 9.

Department of Genetics and Bioengineering, Faculty of Engineering, Yeditepe University , İstanbul , Turkey.

Chordoma is a slowly growing and invasive bone tumor  with a tendency to metastasize locally in advanced stages.  It is essential to discover new therapeutics that target genes involved in the metastasis of chordoma. Epithelial-mesenchymal transition (EMT) might robustly influence the metastasis of a tumor bulk. Read More

Mol Pharm 2019 Apr 11;16(4):1433-1443. Epub 2019 Mar 11.

Department of Neurosurgery , Mayo Clinic , Jacksonville , Florida 32224 , United States.

Glioblastoma (GBMs) is the most common and aggressive type of primary brain tumor in adults with dismal prognosis despite radical surgical resection coupled with chemo- and radiotherapy. Recent studies have proposed the use of small-molecule inhibitors, including verteporfin (VP), to target oncogenic networks in cancers. Here we report efficient encapsulation of water-insoluble VP in poly(lactic- co-glycolic acid) microparticles (PLGA MP) of ∼1. Read More

J Neurosurg Pediatr 2019 Feb 22:1-6. Epub 2019 Feb 22.

Departments of1Neurological Surgery.

An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Read More

World Neurosurg 2019 Feb 18. Epub 2019 Feb 18.

Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island, USA. Electronic address:

Introduction: The primary treatment for patients with sacral chordoma is en bloc surgical resection with negative margins, which has been shown to reduce local recurrence and tumor-related morbidity. Here we describe the use of intraoperative neuronavigation using preoperative spine magnetic resonance imaging fused to intraoperative computed tomography (CT) to create 3-dimensional tumor reconstructions in the operating room for intraoperative identification of bone and soft-tissue margins for maximal safe tumor resection.

Methods: A single-institution retrospective chart review was completed to encompass our experience of 6 consecutive patients who had sacral chordoma resections using our described navigation protocol. Read More

Front Oncol 2019 1;9:30. Epub 2019 Feb 1.

Division of Spine, Department of Orthopedics, Tongji Hospital Affiliated to Tongji University School of Medicine, Shanghai, China.

Chordoma is a rare bone malignancy that affects the spine and skull base. Treatment dilemma leads to a high rate of local relapse and distant metastases. Molecular targeted therapy (MTT) is an option for advanced chordoma, but its therapeutic efficacy and safety have not been investigated systematically. Read More

J Bone Oncol 2019 Apr 29;15:100221. Epub 2019 Jan 29.

Department of Orthopedic Surgery, Sarcoma Biology Laboratory, David Geffen School of Medicine, University of California, 615 Charles E. Young. Dr. South, Los Angeles, CA 90095, USA.

Bone sarcomas are a collection of sporadic malignancies of mesenchymal origin. The most common subtypes include osteosarcoma, Ewing sarcoma, chondrosarcoma, and chordoma. Despite the use of aggressive treatment protocols consisting of extensive surgical resection, chemotherapy, and radiotherapy, outcomes have not significantly improved over the past few decades for osteosarcoma or Ewing sarcoma patients. Read More

Radiother Oncol 2019 Feb 31;131:112-119. Epub 2018 Dec 31.

Massachusetts General Hospital, Boston, USA. Electronic address:

Introduction: Radiation-induced optic neuropathy (RION) is a complication of radiation therapy (RT) that causes blindness. We aimed to define the tolerance of the anterior optic pathway to fractionated RT and identify risk factors for RION.

Materials/methods: Patients with chordoma or chondrosarcoma of the skull base treated with proton and photon therapy between 1983 and 2013, who received a minimum of 30 Gy (relative biologic effectiveness [RBE]) to the anterior optic pathway were assessed. Read More

J Clin Med 2019 Feb 15;8(2). Epub 2019 Feb 15.

Department of Neurosurgery, University Medical Center Göttingen, Georg-August-University Göttingen, 37075 Göttingen, Germany.

Chordomas is rare malignant bone tumors thought to arise from remnants of embryonic notochord along the spine, frequently at the skull base and sacrum. Although chordoma is slow growing tumors, while are extremely recurrent, and aggressive, as well as the rate of prognosis remains poorly. Radical surgery and high-dose radiation are the most used treatments. Read More

Br J Neurosurg 2019 Feb 11:1-6. Epub 2019 Feb 11.

a Department of Neurosurgery , Birmingham University Hospital , Birmingham , England.

Purpose: Clival Chordomas are locally aggressive tumours which pose a significant treatment challenge. Endoscopic endonasal approach for clival chordomas is correlated with higher resection rates and lower morbidity rates in comparison to open approaches. We present our initial single institution experience and short-term patient outcomes following endoscopic endonasal approach for resection of clival chordomas. Read More

J Surg Oncol 2019 Feb 7. Epub 2019 Feb 7.

Division of Orthopaedic Surgery, Department of Surgery, University Musculoskeletal Oncology Unit Mount Sinai Hospital, University of Toronto, Toronto, Canada.

Background: We reviewed the disease control and complications of the treatment of sacrococcygeal chordoma from four tertiary cancer centers with emphasis on the effects of radiotherapy in surgically treated patients.

Methods: A total of 193 patients with primary sacrococcygeal chordoma from 1990 to 2015 were reviewed. There were 124 males, with a mean age of 59 ± 15 years and a mean follow-up of 7 ± 4 years. Read More

J Neurosurg 2019 Feb;130(2):337-346

2Otorhinolaryngology, NewYork-Presbyterian Hospital/Weill Cornell Medicine, New York; and.

OBJECTIVEEndoscopic skull base surgery (ESBS) is a relatively recent addition to the neurosurgical armamentarium. As with many new approaches, there has been significant controversy regarding its value compared with more traditional approaches to ventral skull base pathology. Although early enthusiasm for new approaches that appear less invasive is usually high, these new techniques require rigorous study to ensure that widespread implementation is in the best interest of patients. Read More

Clin Neurol Neurosurg 2019 Mar 25;178:51-55. Epub 2019 Jan 25.

Department of Neurosurgery and Gamma Knife Center, International Medical Center (IMC), 42km. Ismailia Desert Road, Cairo, Egypt. Electronic address:

Objective: This study primarily aims to evaluate the efficacy of gamma knife surgery in controlling growth progression rate of residual clival chordoma through retrospective outcome analysis of 12 consecutive patients.

Patients And Methods: Data for 12 consecutive patients underwent GKS for post-operative residual histologically verified clival chordoma at our institution (IMC - Cairo- Egypt) from 2006 through end of 2017 were retrospectively reviewed and analyzed with mean follow-up period of 45 months (range12-120 months).

Results: In the last follow up MR, tumor growth control was achieved in 33. Read More

Radiat Oncol 2019 Feb 1;14(1):24. Epub 2019 Feb 1.

Service de Cancérologie-Radiothérapie, Hôpital A.Michallon, CHU de Grenoble, Grenoble, France.

Background: Carbon ion radiotherapy (CIRT) has been delivered to more than 20,000 patients worldwide. International trials have been recommended in order to emphasize the actual benefits. The ULICE program (Union of Light Ion Centers in Europe) addressed the need for harmonization of CIRT practices. Read More

Eur Spine J 2019 Jan 25. Epub 2019 Jan 25.

Department of Orthopedic Oncology, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China.

Purpose: Plasma D-dimer levels, a marker of hypercoagulation, have not only been used as an indicator for cascaded reaction in the coagulation process but are also reported to be an underlying biomarker in several types of cancers. This retrospective cohort study was designed to evaluate the prognostic value of preoperative plasma D-dimer level in patients with spinal chordoma.

Methods: We enrolled 224 patients who underwent surgery for spinal chordoma between 2002 and 2015 at Changzheng Orthopedic Oncology Center. Read More

Nat Med 2019 Feb 21;25(2):292-300. Epub 2019 Jan 21.

Broad Institute of Harvard and MIT, Cambridge, MA, USA.

Chordoma is a primary bone cancer with no approved therapy. The identification of therapeutic targets in this disease has been challenging due to the infrequent occurrence of clinically actionable somatic mutations in chordoma tumors. Here we describe the discovery of therapeutically targetable chordoma dependencies via genome-scale CRISPR-Cas9 screening and focused small-molecule sensitivity profiling. Read More