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Diagnosis and Treatment of Benign Notochordal Cell Tumors of the Spine: Report of 3 Cases and Literature Review

Terzi, Silvia MD*; Mobarec, Sebastian MD; Bandiera, Stefano MD*; Gasbarrini, Alessandro MD*; Barbanti-Bròdano, Giovanni MD*; Alberghini, Marco MD; Boriani, Stefano MD*

doi: 10.1097/BRS.0b013e318266e7e6
Case Report

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.

Three patients with a benign notochordal cell tumor are presented. Clinical, radiological, and histological characteristics are reported, and decision-making process and medium-term follow-up are discussed. A review of the literature on this rare tumor is presented.

*Department of Oncologic and Degenerative Spine Surgery, Rizzoli Institute, Bologna, Italy

Department of Orthopaedic Surgery, Facultad de Medicina, Pontificia Universidad Catolica de Chile, Santiago, Chile

Department of Pathology, Rizzoli Institute, Bologna, Italy.

Address correspondence and reprint requests to Sebastian Mobarec, MD, Department of Orthopaedic Surgery, Facultad de Medicina, Pontificia Universidad Catolica de Chile, Marcoleta 352, Santiago 8330033, Chile; E-mail: smobarec@med.puc.cl

Acknowledgment date: March 13, 2012. Revision date: June 12, 2012. Acceptance date: June 26, 2012.

The manuscript submitted does not contain information about medical device(s)/drug(s).

No funds were received in support of this work.

No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this manuscript.

© 2012 Lippincott Williams & Wilkins, Inc.