IS Case 416: Chordoma
Imaging Sciences URMC 2010
Publication Date: 2010-08-27
CT and MR images demonstrated a mass arising from the sacrococcyx
Chordomas are slow growing malignant neoplasms arising from remnants of the embryonic notochord. The notochord is the normal embryonic precursor to the nucleus pulposus of intervertebral discs. Thus chordomas tend to occur in the midline. They are most common at the sacrococcyx (50%), followed by the clivus (35%). Vertebral chordomas are much less common and usually cervical. Chordomas most commonly occur in the 5-6th decades of life and affect men twice as often as women.
Chordomas appear as lobulated, heterogeneous septated masses with characteristic T2 hyperintensity greater than vertebral discs and even CSF. They may have amorphous internal calcifications as well. Enhancement is variable. They are locally aggressive, often infiltrating along intervertebral discs and destroying adjacent bone, potentially leading to spinal cord compression.
Pathologically, identification of vacuolated physaliphorous cells is pathognomonic.
The differential diagnosis is based on location and patient age and includes: chondrosarcoma, sacrococcygeal teratoma (pediatric patients; fat content), lymphoma, metastases, plasmacytoma, and giant cell tumor.
- Baratti D, Gronchi A, Pennacchioli E, et al. Chordoma: natural history and results in 28 patients treated at a single institution. Ann Surg Oncol. 2003 Apr;10(3):291-6. PMID: 12679315
- Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF. Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 2001 Feb 25;93(1):40-51. PMID: 11241265 [PubMed]