Background: Much discussion about benign notochordal cell tissue in vertebrae has centered on the nature of its relationship, if any, to chordoma. Often referred to as benign notochordal cell tumors (BNCTs), these lesions have unique morphological features, however, differentiating between notochordal cells in discs, BNCT, and chordoma can be difficult. They are described as radiologically distinct from chordoma, with lysis, contrast enhancement, and a soft tissue mass indicating chordoma.
Methods: All chordomas diagnosed at our institution, the Istituto Ortopedico Rizzoli (Bologna, Italy), prior to 2008 were reviewed, yielding 174 cases. Five were limited to bone; one was a recurrent chordoma without original data available. The remaining four were re-evaluated in detail.
Results: There were three women and one man, aged 33-57 years (mean, 48 years). Two were BNCTs and two were mixed lesions containing BNCT and chordoma. On computed tomography, all were radiopaque with areas of lysis. One BNCT was heterogeneous on magnetic resonance imaging, enhancing after contrast. Microscopically, one BNCT had a well-defined cystic area with a sclerotic border. The other had a minute atypical area; it recurred as chordoma. The mixed lesions had areas of definitive BNCT, definitive chordoma, and atypical areas that did not meet the criteria for either. The atypical areas in all three cases 'blended' with areas of chordoma or BNCT.
Conclusion: These cases illustrate the ongoing challenges in differentiating between BNCT and chordoma. All had unique imaging features; three had atypical microscopic areas blending with BNCT or chordoma, strengthening the argument for a relationship between the two entities and supporting the idea that some BNCTs may progress to chordoma. Our study dispels the notion that any single radiologic criterion used to distinguish between chordoma and BNCT is reliable, opening the discussion as to whether or how to monitor BNCTs.
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| http://dx.doi.org/10.1186/1470-7330-14-4 | DOI Listing |
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Notochordal cell derived lesions: a 55-year casuistic analysis of 50 cases with radiologic-pathologic correlation in a tertiary referral hospital, and literature review.
Eur Spine J
September 2024
Pathology Department, La Paz University Hospital, Paseo de la Castellana, 261, Madrid, 28046, Spain.
Article Synopsis
- This study examines 50 cases of distinct lesions derived from notochordal cells (NCDL), including various types of chordomas and benign tumors over the past 55 years in a tertiary hospital setting.
- Conventional chordomas were found to be lobulated masses with specific imaging characteristics and a variety of growth patterns, while benign notochordal cell tumors (BNCT) were less aggressive, presenting as well-defined lesions without infiltration.
- Follow-up strategies for BNCT involved radiological monitoring, showing no local recurrence or metastasis, while conventional chordoma cases faced higher risks with significant rates of local recurrence and metastasis, necessitating more aggressive treatments.
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