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Parachordoma exists--but what is it?
1Royal Marsden Hospital, London, United Kingdom.
Advances in Anatomic Pathology
|May 16, 2000
Summary
Parachordoma is a rare soft tissue tumor with epithelial differentiation, distinct from chordoma. This enigmatic neoplasm typically grows slowly and rarely recurs or metastasizes.
Area of Science:
- Oncology
- Pathology
- Soft Tissue Neoplasms
Background:
- Parachordoma is a rare peripheral soft tissue tumor.
- Its lineage is currently unknown, though it shares similarities with chordoma.
- It presents as a circumscribed, firm tumor in deep soft tissues.
Purpose of the Study:
- To discuss the terminology, origin, and nature of parachordoma.
- To differentiate parachordoma from similar soft tissue tumors.
- To review the histological and immunophenotypical characteristics of parachordoma.
Main Methods:
- Histological examination of parachordoma.
- Ultrastructural analysis.
- Immunophenotyping, including S-100 protein and cytokeratin expression.
Main Results:
- Parachordoma exhibits diverse histologic patterns and cytologic features, including vacuolated cells.
- Ultrastructure and immunophenotype suggest epithelial differentiation; S-100 protein is positive.
- It is distinct from extraskeletal myxoid chondrosarcoma and soft tissue myoepithelioma.
- Parachordoma differs from chordoma in cytokeratin immunophenotype and clinical behavior.
Conclusions:
- Parachordoma is a distinct entity with epithelial differentiation.
- It is histologically and clinically different from chordoma.
- Parachordoma is a slow-growing tumor with a low metastatic potential.