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Extraskeletal myxoid chondrosarcomas do not show a chondrocytic phenotype
Thomas Aigner1, André M Oliveira, Antonio G Nascimento
1Department of Pathology, University of Erlangen-Nürnberg, Erlangen, Germany.
Summary
Extraskeletal myxoid chondrosarcoma (EMC) may not be a chondrocytic tumor. Analysis of matrix proteins and cellular phenotype suggests EMC arises from primitive mesenchymal cells with varied differentiation.
Area of Science:
- Oncology
- Pathology
- Biochemistry
Background:
- Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft-tissue malignancy.
- Traditionally classified as chondroblastic, this is questioned due to lack of cartilage formation and inconsistent S-100 protein expression.
Purpose of the Study:
- To investigate the matrix composition and cellular phenotype of EMC.
- To clarify the histogenetic origin of extraskeletal myxoid chondrosarcoma.
Main Methods:
- Biochemical analysis of matrix proteins in 14 EMC specimens.
- Immunohistochemical staining for collagen types I, III, VI, II, X, and S-100 protein.
- Assessment of proteoglycan aggrecan expression.
Main Results:
- Most EMC lacked overt cartilage formation and showed minimal aggrecan.
- Collagen types I, III, and VI were consistently present; collagen type II and X were rare.
- S-100 protein was largely absent in the studied specimens.
Conclusions:
- The cellular phenotype of EMC is not chondrocytic or prechondrocytic.
- EMC is likely a tumor of primitive mesenchymal cells with focal, multidirectional differentiation.
- Chondrocytic differentiation is an uncommon feature in EMC.