Presentation
Headache
Patient Data
A hypodense retroclival mass with associated bony destruction is noted.
The retroclival mass is of very high T2 signal and extends into the clivus. It demonstrates minimal if any enhancement. Incidental left cerebellar hemisphere developmental venous anomaly (DVA).
Conclusion: The most likely diagnosis is of a large ecchordosis physaliphora or a small chordoma. A chondrosarcoma and an extra-dural epidermoid cyst are less likely differentials.
Case Discussion
The patient went on to have a resection.
Histology
Sections of decalcified bone show an intraosseous proliferation of vacuolated cells diffusely extending between bony trabeculae, with the adjacent normal bone marrow containing viable hematopoietic elements. There is associated benign paucicellular intraosseous chondroid tissue undergoing endochondral ossification. The vacuolated cells contain abundant pale-to-clear cytoplasm with mucin droplets. The cells contain small round, bland nuclei with inconspicuous nucleoli. No necrosis, atypia or mitoses are seen.
Immunohistochemically vacuolated cells stain positive for CKAE1/3 and S100+. The Ki67 proliferation index is approximately 1-2%.
FINAL DIAGNOSIS: ecchordosis physaliphora.
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