Chordoma Research Information

Our goal is to be able to identify new molecular targets, assess new therapeutics, and better characterize the prognosis of chordomas.

Chordoma is a rare, slow-growing malignant tumor believed to arise from cellular remnants of the notochord. The treatment of chordomas is difficult. Wide surgical excision is desirable, but it is rarely feasible based on the anatomic location of the tumor at the base of the skull or the base of the spine.

Currently there are no effective drugs for chordoma, and there are no drugs currently approved to treat chordoma. Unlike with other types of sarcoma, there is no specific known tumor-suppressor gene or oncogene associated with chordoma.

There is a need, however, for understanding the molecular biology of chordoma. We have obtained a number of chordoma cell lines. Most importantly, the Massachusetts General Hospital Sarcoma Group has archived more than 150 cases of chordoma tissues and paraffin blocks in our tissue bank. DNA, RNA and protein samples from chordoma tumor tissues will be analyzed by CGH or SNP array, gene and protein expression profile with the hope of identification of chordoma susceptibility genes or pathways.

The new discoveries from these studies will identify potential tumor-suppressor genes and oncogenes, then proteins for testing in chordoma tissues. We plan to determine the efficacy of targeting mTOR, Stat3 expression in chordoma cells by small molecular compounds, siRNA and to evaluate its effect on chordoma cell growth, proliferation and apoptosis.

Our goal is to be able to identify new molecular targets, assess new therapeutics, and better characterize the prognosis of chordomas.

Recent Chordoma Studies

The following articles are a selection of publications from Harris Center researchers from 2012 to 2016, arranged in chronological order with the most recent at the top. Click on a title for the PubMed summary of the article.

How Does the Level of Nerve Root Resection in En Bloc Sacrectomy Influence Patient-Reported Outcomes? van Wulfften Palthe OD1, Houdek MT, Rose PS, Yaszemski MJ, Sim FH, Boland PJ, Healey JH , Hornicek FJ, Schwab JH, Clin Orthop Relat Res. 2016 Mar 18

Characteristics and Patterns of Metastatic Disease from Chordoma Young VA, Curtis KM, Temple HT, Eismont FJ, DeLaney TF, Hornicek FJ, Sarcoma. 2015;2015:517657.

CSPG4 as a prognostic biomarker in chordoma Schoenfeld AJ, Wang X, Wang Y, Hornicek FJ, Nielsen GP, Duan Z, Ferrone S, Schwab JH. Spine J. 2015 Dec 9. pii: S1529-9430(15)

Chordoma: an update on the pathophysiology and molecular mechanisms Sun X, Hornicek F, Schwab JH. Curr Rev Musculoskelet Med. 2015 Dec;8(4):344-52.

High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors. Rotondo RL , Folkert W, Liebsch NJ, Chen YL , Pedlow FX , Schwab JH , Rosenberg AE , Nielsen GP , Szymonifka J, Ferreira AE, Hornicek FJ , DeLaney TF . (2015) J Neurosurg Spine. 2015 Dec;23(6):788-97.

[18F]-Fluoromisonidazole Positron Emission Tomography/Computed Tomography Visualization of Tumor Hypoxia in Patients With Chordoma of the Mobile and Sacrococcygeal Spine. Cheney MD, Chen YL, Lim R, Winrich BK, Grosu AL, Trofimov AV, Depauw N, Shih HA, Schwab JH, Hornicek FJ, DeLaney TF. Int J Radiat Oncol Biol Phys. 2014 Dec 1;90(5):1030-6.

Long-term results of Phase II study of high dose photon/proton radiotherapy in the management of spine chordomas, chondrosarcomas, and other sarcomas. DeLaney TF, Liebsch NJ, Pedlow FX, Adams J, Weyman EA, Yeap BY, Depauw N, Nielsen GP, Harmon DC, Yoon SS, Chen YL, Schwab JH, Hornicek FJ. J Surg Oncol. 2014 Aug;110(2):115-22.

Genotyping Cancer-Associated Genes in Chordoma Identifies Mutations in Oncogenes and Areas of Chromosomal Loss Involving CDKN2A, PTEN, and SMARCB1. Choy E, MacConaill LE, Cote GM, Le LP, Shen JK, Nielsen GP, Iafrate AJ, Garraway LA, Hornicek FJ, Duan Z. PLoS One. 2014 Jul 1;9(7)

Prognostic significance of miRNA-1 (miR-1) expression in patients with chordoma. Duan Z, Shen J, Yang X, Yang P, Osaka E, Choy E, Cote G, Harmon D, Zhang Y, Nielsen GP, Spentzos D, Mankin H, Hornicek F.J Orthop Res. 2014 May;32(5):695-701

Tissue microarray immunohistochemical detection of brachyury is not a prognostic indicator in chordoma. Zhang L, Guo S, Schwab JH, Nielsen GP, Choy E, Ye S, Zhang Z, Mankin H, Hornicek FJ, Duan Z. PLoS One. 2013 Sep 23;8(9)

Definitive High Dose Photon/Proton Radiotherapy for Unresected Mobile Spine and Sacral Chordomas. Chen YL, Liebsch N, Kobayashi W, Goldberg S, Kirsch D, Calkins G, Childs S, Schwab J, Hornicek F, Delaney T. Spine (Phila Pa 1976). 2013 Apr 19

Establishment and characterization of a novel chordoma cell line: CH22. Liu X, Nielsen GP, Rosenberg AE, Waterman PR, Yang W, Choy E, Sassi S, Yang S, Harmon DC, Yang C, Schwab JH, Kobayashi E, Mankin HJ, Xavier R, Weissleder R, Duan Z, Hornicek FJ. J Orthop Res. 2012 Apr 13. doi: 10.1002/jor.22113