Publications

Resulting publications and posters

The following research publications and posters have resulted from our work:

Year	Title	Publication	Our role
2024	Organelle resolved proteomics uncovers PLA2R1 as a novel cell surface marker required for chordoma growth	Acta Neuropathologica Communications	models
2024	Multimodal profiling of chordoma immunity reveals distinct immune contextures	Journal for ImmunoTherapy of Cancer	funding
2023	Pilot study of high-dose pemetrexed in patients with progressive chordoma	Clinical Cancer Research	funding, study participant outreach
2023	The NIH pediatric/young adult chordoma clinic and natural history study: Making advances in a very rare tumor	Pediatric Blood & Cancer	authorship, study participant outreach
2023	Virus-like particle-based delivery of Cas9/guide RNA ribonucleoprotein efficiently edits the brachyury gene and inhibits chordoma growth in vivo	Discover Oncology	funding
2023	Activity of pemetrexed in pre‑clinical chordoma models and humans	Scientific Reports	authorship, drug screening
2023	Assessing replication stress as an actionable therapeutic opportunity in chordoma	AACR Annual Meeting	authorship, drug screening
2023	Mechanisms of EGFR inhibitor sensitivity and resistance in chordoma	AACR Annual Meeting	authorship, drug screening
2023	Mapping the landscape of genetic dependencies in chordoma	Nature Communications	funding, in vivo drug testing, models, authorship
2023	Quality of life in chordoma co-survivors: Results from the Chordoma Foundation Survivorship Survey	World Neurosurgery	authorship, data
2023	Transcriptional profiling supports the notochordal origin of chordoma and its dependence on a TGFB1-TBXT network	American Journal of Pathology	funding
2022	SMARCB1 loss in poorly-differentiated chordomas drives tumor progression	American Journal of Pathology	models, biobank samples, technical assistance
2022	Radiotherapy after gross total resection of skull base chordoma: A SEER database analysis of survival outcomes	World Neurosurgery	authorship
2022	Multi-spectral immunofluorescence evaluation of the myeloid, T cell, and natural killer cell tumor immune microenvironment in chordoma may guide immunotherapeutic strategies	Frontiers in Oncology	funding, biobank samples
2022	Emerging target discovery and drug repurposing opportunities in chordoma	Frontiers in Oncology	authorship
2022	Selinexor inhibits growth of patient derived chordomas in vivo as a single agent and in combination with abemaciclib through diverse mechanisms	Frontiers in Oncology	in vivo drug testing, authorship
2021	Combinatorial natural killer cell–based immunotherapy approaches selectively target chordoma cancer stem cells	Cancer Research Communications	funding, models
2021	Molecular features and vulnerabilities of recurrent chordomas	Journal of Experimental & Clinical Cancer Research	data, prize
2021	Higher cMET dependence of sacral compared to clival chordoma cells: contributing to a better understanding of cMET in chordoma	Scientific Reports	models
2021	Phase 1 open-label trial of intravenous administration of MVA-BN-brachyuryTRICOM vaccine in patients with advanced cancer	Journal for ImmunoTherapy of Cancer	trial design, trial participant outreach
2021	Randomized, double-blind, placebo-controlled phase II study of yeast-brachyury vaccine (GI-6301) in combination with standard-of-care radiotherapy in locally advanced, unresectable chordoma	The Oncologist	trial design, trial participant outreach
2021	Aldehyde dehydrogenase, a therapeutic target in chordoma: Analysis in 3D cellular models	Cells	models
2021	DNA Methylation based prognostic subtypes of chordoma tumors in tissue and plasma	Neuro-Oncology	funding, models
2021	AZD8055 enhances in vivo efficacy of afatinib in chordomas	Journal of Pathology	funding, models
2021	Targeted brachyury degradation disrupts a highly specific autoregulatory program controlling chordoma cell identity	Cell Reports Medicine	funding, models

Year	Title	Publication	Our role
2020	Defective HLA Class I expression and patterns of lymphocyte infiltration in chordoma tumors	Clinical Orthopaedics and Related Research	funding
2020	The mutational landscape of spinal chordomas and their sensitive detection using circulating tumor DNA	Neuro-Oncology Advances	funding
2020	Inhibition of histone H3K27 demethylases inactivates brachyury (TBXT) and promotes chordoma cell death	Cancer Research	funding, models
2020	The sacral chordoma margin	European Journal of Surgical Oncology	authorship
2020	Synergistic drug combinations and machine learning for drug repurposing in chordoma	Scientific Reports	models
2020	Frequent alterations in p16/CDKN2A identified by immunohistochemistry and FISH in chordoma	Journal of Pathology	funding, models
2020	Rationale for the advancement of PI3K pathway inhibitors for personalized chordoma therapy	Journal of Neuro-Oncology	models, in vivo drug testing
2019	Distinctive role of dysregulated miRNAs in chordoma cancer stem-like cell maintenance	Experimental Cell Research	models
2019	Combination of PARP inhibitor and temozolomide to suppress chordoma progression	Journal of Molecular Medicine	models
2019	Design of a cyclin G associated kinase (GAK)/epidermal growth factor receptor (EGFR) inhibitor set to interrogate the relationship of EGFR and GAK in chordoma	Journal of Medicinal Chemistry	models
2019	Small-molecule targeting of brachyury transcription factor addiction in chordoma	Nature Medicine	funding, models, authorship
2018	CDK4 expression in chordoma: A potential therapeutic target.	Journal of Orthopaedic Research	models
2018	Immunotherapy utilizing the combination of natural killer– and antibody dependent cellular cytotoxicity (ADCC)–mediating agents with poly (ADP-ribose) polymerase (PARP) inhibition	Journal for ImmunoTherapy of Cancer	models
2018	Brachyury gene copy number gain and activation of the PI3K/Akt pathway: association with upregulation of oncogenic Brachyury expression in skull base chordoma.	Journal of Neurosurgery	models
2018	Protein phosphatase 2A inhibition enhances radiation sensitivity and reduces tumor growth in chordoma	Neuro-oncology	models
2018	Characterization of the endolysosomal system in human chordoma cell lines: is there a role of lysosomes in chemoresistance of this rare bone tumor?	Histochemistry and Cell Biology	models
2018	A potential therapy for chordoma via antibody-dependent cell-mediated cytotoxicity employing NK or high-affinity NK cells in combination with cetuximab	Journal of Neurosurgery	models
2018	U-CH17P, -M and -S, a new cell culture system for tumor diversity and progression in chordoma	International Journal of Cancer	prize
2018	Afatinib is a new therapeutic approach in chordoma with a unique ability to target EGFR and Brachyury	Molecular Cancer Therapeutics	models, in vivo drug testing, authorship

Year	Title	Publication	Our role
2017	Emerging opportunities for target discovery in rare cancers	Cell Chemical Biology	data
2017	The driver landscape of sporadic chordoma	Nature Communications	funding, models, authorship
2017	Establishment and genomic characterization of the new chordoma cell line Chor-IN-1	Scientific Reports	models, prize
2017	Validation of reference genes in human chordoma	Surgical Neurology International	funding, models
2017	UM-Chor1: establishment and characterization of first validated clival chordoma cell line	Journal of Neurosurgery	models, prize
2017	Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group	Annals of Oncology	funding, authorship
2017	Notochord isolation using laser capture microdissection	Journal of Chemical Neuroanatomy	funding
2017	Integrated and quantitative proteomics of human tumors	Methods of Enzymology	funding
2017	Symptom burden and life challenges reported by adult chordoma patients and their caregivers	Quality of Life	data, authorship
2016	STAT3 inhibition as a therapeutic strategy for chordoma	Journal of Neurological Surgery	models
2016	Establishment of clival chordoma cell line-MUG-CC1 and lymphoblastoid cells as a model for potential new treatment strategies	Scientific Reports	prize
2016	IKK/NF-kB signaling contributes to glioblastoma stem cell maintenance	Oncotarget	models, authorship
2016	EGFR inhibitors identified as potential treatment for chordoma in a focused compound screen	The Journal of Pathology	models, in vivo drug testing
2015	Building a global consensus approach to chordoma: a position paper from the medical and patient community	The Lancet Oncology	funding, authorship
2015	Genome-wide DNA methylation profiling of recurrent and non-recurrent chordomas	Epigenetics	models
2015	Expression of programmed cell death ligand 1 (PD-L1) and prevalence of tumor-infiltrating lymphocytes (TILs) in chordoma	Oncotarget	models
2015	MicroRNA-155 expression is independently predictive of outcome in chordoma	Oncotarget	funding, models
2015	Phase 1 trial of yeast-based therapeutic cancer vaccine (GI-6301) targeting the transcription factor brachyury	Cancer Immunology Research	clinical trial support
2015	Preclinical characterization of novel chordoma cell systems and their targeting by pharmacological inhibitors of the CDK4/6 cell-cycle pathway	Cancer Research	prize
2015	Combined PDGFR and HDAC inhibition overcomes PTEN disruption in chordoma	PLoS ONE	models
2014	PD-1, PD-L1, PD-L2 expression in the chordoma microenvironment	Journal of Neuro-Oncology	funding, models
2014	The FGFR/MEK/ERK/brachyury pathway is critical for chordoma cell growth and survival	Carcinogenesis	models
2014	Investigating microenvironmental regulation of human chordoma cell behavior	PLoS ONE	funding, models
2014	Prognostic significance of miRNA-1 (miR-1) expression in patients with chordoma	Journal of Orthopaedic Research	funding, models
2014	FAS/FASL are dysregulated in chordoma and their loss-of-function impairs zebrafish notochord formation	Oncotarget	models
2014	Molecular characterization of chordoma xenografts generated from a novel primary chordoma cell source and two chordoma cell lines	Journal of Neurosurgery: Spine	models
2014	Characterization of T gene sequence variants and germline duplications in familial and sporadic chordoma	Human Genetics	funding

Year	Title	Publication	Our role
2013	Development of transplantable human chordoma xenograft for preclinical assessment of novel therapeutic strategies	Neuro-Oncology	funding
2013	Generation of a patient-derived chordoma xenograft and characterization of the phosphoproteome in a recurrent chordoma	Journal of Neurosurgery	prize
2013	Erlotinib inhibits growth of a patient-derived chordoma xenograft	PLoS ONE	funding
2013	A novel chordoma xenograft allows in vitro drug testing and reveals the importance of NF-kB signaling in chordoma biology	PLoS ONE	funding
2013	MicroRNA expression profiling reveals the potential function of microRNA-31 in chordomas	Journal of Neuro-Oncology	models
2013	Identification of repurposed small molecule drugs for chordoma therapy	Cancer Biology & Therapy	models, authorship
2012	An integrated functional genomics approach identifies the regulatory network directed by brachyury (T) in chordoma	Journal of Pathology	models
2012	A common single-nucleotide variant in T is strongly associated with chordoma	Nature Genetics	funding
2012	Characterization of cancer stem-like cells in chordoma	Journal of Neurosurgery	models
2012	Controlled release of doxorubicin from PH-responsive microgels	Acta Biomaterialia	models
2011	The effects of chemotherapeutic agents on differentiated chordoma cells	Journal of Neurosurgery: Spine	models
2011	In vitro characterization of cells derived from chordoma cell line U-CH1 following treatment with X-rays, heavy ions, and chemotherapeutic drugs	Radiation Oncology	models
2011	Massive genomic rearrangement acquired in a single catastrophic event during cancer development	Cell	funding
2011	Role of transcription factor T (brachyury) in the pathogenesis of sporadic chordoma: a genetic and functional based study	Journal of Pathology	funding, models
2010	Molecular characterization of putative chordoma cell lines	Sarcoma	funding, authorship
2010	Methylthioadenosine phosphorylase and activated insulin-like growth factor-1 receptor/insulin receptor: potential therapeutic targets in chordoma	Journal of Pathology	authorship
2009	T (brachyury) gene duplication confers major susceptibility to familial chordoma	Nature Genetics	funding
2009	Aberrant hyperactivation of akt and Mammalian target of rapamycin complex 1 signaling in sporadic chordomas	Clinical Cancer Research	models