Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 30, 2015
Benign Notochordal Cell Tumor
- Synonyms: Notochordal hamartoma, ecchordosis physaliphora vertebralis
- Background:
- Benign notochordal cell tumor, or giant vertebral notochordal rest (GVNR), is a new and evolving term used to describe benign notochordal tissue that may produce a macroscopic vertebral lesion, with only a few cases reported in the literature. The distinction between GVNR and cordoma relies mostly on imaging findings.
- Notochordal cells may persist in the nucleus pulposus through the 2nd decade. These cells herniate into the vertebral body through minor discontinuities in the end plate, and proliferate in the bone marrow.
- Clinical Features: Asymptomatic, corresponds to an incidental finding
- Key Diagnostic Features:
- CT: Normal or revealing sclerosis ranging from mild to severe. The lesions are in the vertebral body and can extend to the cortex or occupy the whole vertebral body, resulting in an ivory vertebra. Trabecular and cortical destruction will not be seen.
- MRI: Well defined intraosseous lesion with low T1-weighted and high T2-weighted signal intensity. No enhancement or diffusion restriction is seen.
- There will be no bone destruction or soft tissue extension. Lack of bone destruction, soft tissue extension, and long-term stability are important imaging criteria for radiological diagnosis of GVNR.
- DDx:
- Chordoma: They are large, destructive lytic lesions with soft tissue extension. They can involve more than one vertebral body. They will enhance on MRI.
- Metastases/plasmocytoma: Usually enhance. Will show restricted diffusion due to increased cellularity.
- Atypical hemangioma: CT will show various degrees of internal thickened bone trabeculae.
- Treatment: Long-term imaging follow-up in order to detect possible transformation into a chordoma
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