Chordomas are rare, low to intermediate grade malignant bone
tumors of the axial skeleton. Current treatment options are limited to
surgical procedures, as
chordomas are largely resistant to conventional radiation and
chemotherapy. Cell lines are valuable tools for exploring molecular mechan-isms involved in
tumorigenesis and they have a fundamental impact on the development of new
anticancer agents. To date, only two
chordoma cell lines exist world-wide. In the present study we report a third
chordoma cell line, MUG-Chor1, as well as corresponding cultured fibroblasts established from a recur-rent morphologically 'classic' sacrococcygeal
chordoma of a 58-year-old Caucasian female. The cells are
brachyury-positive and have the characteristics of
chordoma. The genetic profile of the primary
chordoma and the established
chordoma cell line was investigated during the culturing period (early and late passage). MUG-Chor1 is karyotypically, <2n>43-47,XX,del(3)(q1?)[11], +7,del(9)(p1?),der(9;15)(q10;q10),-10,+der(12)t(9;12)(p2?;q1?),der (12)t(12;19)(p;p)t(17;19)(q;q),-15,der(17;21)(q10;q10),der(20)t(10;20) (q25?26?;q11?12?),-21,-22[20]/idemx2[5] and displays known,
chordoma-typical genetic changes, such as chromosomal gains at T/
brachyury locus (6q27), losses at 9p24.3-p13.1 (includes the CDKN2a/CDKN2b locus), 10p15.3-q23.32 (includes the PTEN locus) and losses of 10q25.2 (includes the PDCD4 locus). MUG-Chor1 bears a marked resemblance to
chordomas in vivo and is, therefore, an optimal in vitro
chordoma model.