This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Gottschalk, D. Articles by Aigner, T. Search for Related Content PubMed PubMed Citation Articles by Gottschalk, D. Articles by Aigner, T.

(American Journal of Pathology. 2001;158:1571-1578.)
© 2001 American Society for Investigative Pathology

Short Communications

Matrix Gene Expression Analysis and Cellular Phenotyping in Chordoma Reveals Focal Differentiation Pattern of Neoplastic Cells Mimicking Nucleus Pulposus Development

Detlev Gottschalk^*, Marita Fehn, Stephan Patt, Wolfgang Saeger, Thomas Kirchner^* and Thomas Aigner^*

From the Department of Pathology,^*
University of Erlangen-Nürnberg, Erlangen, Germany; the Department of Orthopaedic Surgery,
Technical University, Aachen, Germany; the Department of Pathology,
Marienkrankenhaus, Hamburg, Germany; and the Department of Pathology (Neuropathology),
University of Jena, Jena, Germany

Chordoma is the fourth most common malignant primary neoplasm of the skeleton and almost the only one showing a real epithelial phenotype. Besides classic chordoma, so-called chondroid chordoma was described as a specific entity showing cartilage-like tissue within chordomatoid structures. However, since its first description, strongly conflicting results have been reported about the existence of chondroid chordoma and several studies suggested chondroid chordomas being in fact low-grade conventional chondrosarcomas. In the present study, we used cytoprotein expression profiling and molecular in situ localization techniques of marker gene products indicative of developmental phenotypes of chondrocytes to elucidate origin and biology of chondroid chordoma. We were able to demonstrate the chondrogenic potential of chordomas irrespectively of the appearance of overt cartilage formation by identifying the multifocal expression of type II collagen, the main marker of chondrocytic differentiation. Additionally, the cartilage-typical large aggregating proteoglycan aggrecan was present throughout all chordomas and, thus, a very characteristic gene product and marker of these neoplasms. Biochemical matrix composition and cell differentiation pattern analysis showed a high resemblance of classic chordomas and in chordoid areas of chondroid chordomas to the fetal chorda dorsalis, whereas chondroid areas of chondroid chordomas showed features similar to adult nucleus pulposus. This demonstrates on the cell function level the chondrocytic differentiation potential of neoplastic chordoid cells as a characteristic facet of chordomas, mimicking fetal vertebral development, ie, the transition of the chorda dorsalis to the nucleus pulposus. Our study firmly establishes a focal real chondrocytic phenotype of neoplastic cells in chordomas. Chondroid chordoma is neither a low-grade chondrosarcoma nor a misnomer as discussed previously.

This article has been cited by other articles:

				A. Gruber, S. Kneissl, B. Vidoni, and A. Url Cervical Spinal Chordoma with Chondromatous Component in a Dog Veterinary Pathology, September 1, 2008; 45(5): 650 - 653. [Abstract] [Full Text] [PDF]

				M. Zamecnik and T. Aigner Chondroma of Epithelial Cell Origin Am. J. Pathol., July 1, 2003; 163(1): 367 - 368. [Full Text] [PDF]