Objectives Characteristics from a large systematic review.
Design The first systematic review of benign notochordal cell tumours.
Subjects All studies, in any language, published at any time, identified using specified search terms.
Methods Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).
Results So-called benign notochordal cell tumours exhibited the following features: pain was the sole presenting symptom in 37/81 (45.7%) and neurological deficit in 36/81 (44.4%). Incidental discovery upon imaging was reported in 8/81 (9.9%). Radiologically, lesions were small, (≤30 millimetres at their maximum diameter) in 57/65 (87.7%), hypointense on T1 sequences and hyperintense on T2 sequences on magnetic resonance imaging (106/130; 81.5%), did not enhance following contrast administration (62/74; 83.8%) and were non-lytic (80/160; 50.0%). Histologically, these tumours lacked myxoid matrix (66/84; 78.6%) and nuclear atypia (100/112; 89.3%). In those managed conservatively, follow up was between 8.5 and 120 months. Most were stable at follow up (22/25; 88.0%), one lesion grew (4.0%), one patient died of an unrelated cause (4.0%) and outcome was not stated in one case (4.0%).
Conclusions BNCTs exhibit a spectrum of features, none of which are pathognomonic and diagnosis is subjective. The majority of reports describe small, non-progressive, non-lytic lesions that lack contrast enhancement, intercellular myxoid matrix and nuclear atypia. They are nevertheless difficult to distinguish from small chordomas.
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