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Soft tissue chordomas: a clinicopathologic analysis of 11 cases
Scott R Lauer1, Mark A Edgar, Jerad M Gardner
1Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA 30322, USA.
The American Journal of Surgical Pathology
|April 17, 2013
Summary
Soft tissue chordomas (STCs) are rare tumors that can be challenging to diagnose. This study analyzed 11 STC cases, finding they are histologically similar to bone chordomas but occur more distally, leading to better outcomes.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Soft tissue chordomas (STCs) are rare and often misdiagnosed.
- Systematic studies on STCs are lacking due to their rarity and resemblance to other lesions.
Purpose of the Study:
- To analyze the clinicopathological features and outcomes of soft tissue chordomas.
- To confirm the diagnosis of STCs using brachyury expression.
Main Methods:
- Retrospective review of institutional and consultation files (1989-2011).
- Inclusion criteria: chordoma or parachordoma, arising in soft tissue, no bone origin, no prior axial chordoma.
- Histopathological analysis and immunohistochemical staining for brachyury, cytokeratins, and S100 protein.
Main Results:
- Eleven cases of STC met inclusion criteria.
- Tumors were located in various distal soft tissues (buttock, wrist, leg, etc.).
- Histology showed cords/syncytia of cells with vacuolated cytoplasm; brachyury, cytokeratins, and S100 protein were expressed.
- Follow-up in 10 patients showed 6 were disease-free, 3 had recurrence or metastasis, and 1 died.
Conclusions:
- Soft tissue chordomas are histologically identical to osseous chordomas.
- STCs have a predilection for distal locations, potentially leading to better disease control due to smaller size and resectability.
- The existence of STCs suggests notochordal remnants are not essential for chordoma development.
