Ae. Rosenberg et al., CHONDROID CHORDOMA - A VARIANT OF CHORDOMA - A MORPHOLOGIC AND IMMUNOHISTOCHEMICAL STUDY, American journal of clinical pathology, 101(1), 1994, pp. 36-41
In 1973, Heffelfinger and coworkers(6) described a variant of chordoma
that contained cartilaginous areas indistinguishable from hyaline typ
e chondrosarcoma. They designated these tumors chondroid chordomas and
found that they had a better prognosis than classic (nonchondroid) ch
ordomas. Since that time, there has been an ongoing debate over whethe
r chondroid chordoma is best considered a distinct clinicopathologic e
ntity separable from chondrosarcoma or a misdiagnosed chondrosarcoma w
hose concept developed from the erroneous interpretation of morphology
. In an attempt to clarify the issue, the authors used light microscop
y and immunohistochemistry to study 12 chondroid chordomas, 38 classic
chordomas, and 28 chondrosarcomas that arose in the base of the skull
or spine. As a reference, they also analyzed the immunohistochemical
profile of fetal notochord, ecchordosis physaliphora, and fetal hyalin
e cartilage. They found that all chondroid and nonchondroid chordomas
were positive for cytokeratin, and the majority werealso positive for
epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA).
In contrast, none of the chondrosarcomas stained for cytokeratin, EMA
or CEA. Vimentin and S-100 were positive in more than 95% of both clas
sic and chondroid chordomas and chondrosarcomas. The immunohistochemic
al profile of these tumors was similar to the pattern of immunoreactiv
ity of their nonneoplastic counterparts. The authors conclude that cho
ndroid chordomas is a variant of chordoma and should not be confused w
ith chondrosarcoma. Because chondroid chordomas have been reported to
have a better prognosis, they felt that this nosologic term should be
preserved and that chondroid chordoma should continue to be a focus of
clinical and pathologic study.