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.
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.
Authors: M Dominici; K Le Blanc; I Mueller; I Slaper-Cortenbach; Fc Marini; Ds Krause; Rj Deans; A Keating; Dj Prockop; Em Horwitz Journal: Cytotherapy Date: 2006 Impact factor: 5.414
Authors: Paul O'donnell; Roberto Tirabosco; Sonja Vujovic; William Bartlett; Timothy W R Briggs; Stephen Henderson; Chris Boshoff; Adrienne M Flanagan Journal: Skeletal Radiol Date: 2006-06-30 Impact factor: 2.199
Authors: Seung Yong Park; So Ri Kim; Yeong Hun Choe; Ka Young Lee; Seoung Ju Park; Heung Bum Lee; Gong Yong Jin; Kyu Yun Jang; Yong Chul Lee Journal: Respiration Date: 2008-05-22 Impact factor: 3.580
Authors: Jan Neumann; Alexandra S Gersing; Thomas F Barth; Melanie Boxberg; Klaus Woertler Journal: Skeletal Radiol Date: 2019-05-18 Impact factor: 2.199
Authors: Wesley Hsu; Ahmed Mohyeldin; Sagar R Shah; Colette M ap Rhys; Lakesha F Johnson; Neda I Sedora-Roman; Thomas A Kosztowski; Ola A Awad; Edward F McCarthy; David M Loeb; Jean-Paul Wolinsky; Ziya L Gokaslan; Alfredo Quiñones-Hinojosa Journal: J Neurosurg Date: 2011-06-24 Impact factor: 5.115
Authors: Jason M Davies; Aaron E Robinson; Cynthia Cowdrey; Praveen V Mummaneni; Gregory S Ducker; Kevan M Shokat; Andrew Bollen; Byron Hann; Joanna J Phillips Journal: J Neurosurg Date: 2013-11-29 Impact factor: 5.115