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Derivation and Characterization of an Extra-Axial Chordoma Cell Line (EACH-1) from a Scapular Tumor
Amalia M. DeComas, MD1; Patrice Penfornis, MSc2; Michael R. Harris, BSc2; Mark S. Meyer, MD3; Radhika R. Pochampally, PhD2
1 Department of Orthopaedics, University of Texas M.D. Anderson Cancer Center, 1400 Pressler Boulevard, Suite FCT 10.5067, Houston, TX 77030
2 Tulane Center for Gene Therapy, Tulane University Health Sciences Center, JBJ Building, 1324 Tulane Avenue, SL-99, New Orleans, LA 70112. E-mail address for R.R. Pochampally: rpocham@tulane.edu
3 Department of Orthopaedics, Ochsner Medical Center, 1514 Jefferson Highway, New Orleans, LA 70121
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Disclosure: In support of their research for or preparation of this work, one or more of the authors received, in any one year, outside funding or grants in excess of $10,000 from the Louisiana Cancer Research Consortium; HCA—The Healthcare Company; and the Louisiana Gene Therapy Research Consortium. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity.

Investigation performed at Ochsner Medical Center, New Orleans, and Tulane University Health Sciences Center, New Orleans, Louisiana

Copyright ©2010 American Society for Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2010 May 01;92(5):1231-1240. doi: 10.2106/JBJS.I.00594
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Abstract

Background: 

Extra-axial chordomas are rare low-grade malignant tumors thought to arise from notochordal remnants in the extra-axial skeleton. Few studies have been done on this neoplasm because of its rarity. In addition, there is a lack of a good in vitro model on which to perform more characterization.

Methods: 

We describe a twenty-eight-year-old man with a mass in the right scapula. Cytomorphology and immunohistochemistry, including brachyury staining, were used to formulate the final diagnosis. A fragment of the tumor was placed in culture, and cells obtained were injected subcutaneously in an immunocompromised mouse. From the tumor developed in mice, a cell line has been derived and characterized by fluorescence-activated cell-sorting analysis, karyotyping, clonogenicity, and cell and tumor growth curves.

Results: 

Cytomorphology on the tumor showed nests of round cells with vacuoles and also physaliferous-like cells with uniform nuclei. Immunochemistry revealed a tumor positive for vimentin, moderately positive for S-100 and cytokeratin AE1/AE3, weakly positive for epithelial membrane antigen, and negative for p63 and cytokeratin (CK)-7. Further analysis revealed the tumor was diffusely and strongly positive for brachyury. The cell line derived from the tumor showed rapid doubling-time, a strong expression of mesenchymal cell surface markers, a karyotype of diploid or hypotetraploid clones with numerous chromosomal aberrations, and the ability to form colonies without attachment and to form tumors in immunocompromised mice.

Conclusions: 

The diagnosis of the extra-axial chordoma is difficult but can be resolved by the detection of a strong brachyury expression. In addition, the derivation of a human extra-axial chordoma cell line could be a useful tool for the basic research of this rare neoplasm.

Clinical Relevance: 

We describe the importance of brachyury staining in the diagnosis of this tumor. The extra-axial chordoma cell line (EACH-1) may also provide a new research tool to establish new ways of diagnosing and treating this rare malignancy.

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    Accreditation Statement
    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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