Diagnosis and treatment of benign notochordal cell tumors of the spine: Report of 3 cases and literature review

STUDY DESIGN. Clinical case series. OBJECTIVE. To describe the clinical, radiological, and histological presentation of a series of patients presenting with benign notochordal tumors, and review the existing literature on the topic. SUMMARY OF BACKGROUND DATA.: During the past decade, several authors have reported a spine tumor with benign clinical characteristics and histological resemblance to notochordal tissue. The prognosis and appropriate management remain controversial. METHODS. Description of clinical, radiological, and histological findings in 3 patients with benign notochordal tumors of the spine. RESULTS. All cases presented with subacute mild pain, without neurological deficit. Tumors developed at L3, S1, and S4, without canal involvement or apparent instability. The first 2 patients presented with classic imagenological findings were treated conservatively, showing no progression on follow-up. The last patient presented an atypical lytic pattern and contrast enhancement on magnetic resonance imaging, and underwent en bloc resection, with significant associated morbidity. Histopathology of the specimen revealed coexistent foci of incipient chordoma. CONCLUSION. Benign notochordal cell tumors represent a clinical entity derived from notochordal tissue, with characteristics distinct but closely related to the classic chordoma. Some radiological features may suggest the presence of chordoma precursors. Because its true potential for aggressiveness is still undetermined, a careful decision making must weigh the morbidity of en bloc procedures in the spine with uncertain natural history.