Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors’ knowledge, have not been previously documented at this anatomical site.
An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.
A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.
These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.
ABBREVIATIONSBNCT = benign notochordal cell tumor; EP = ecchordosis physaliphora.
CarterJM, WengerDE, RosePS, InwardsCY: Atypical notochordal cell tumors: a series of notochordal-derived tumors that defy current classification schemes. 41:39–48, 201710.1097/PAS.000000000000076627819871)| false
ChoudhriOFerozeAHwangPVogelHAjlanAHarshG IV: Endoscopic resection of a giant intradural retroclival ecchordosis physaliphora: surgical technique and literature review. World Neurosurg82:912.e21–912.e262014
CiarpagliniRPasquiniEMazzatentaDAmbrosini-SpaltroASciarrettaVFrankG: Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report. Neurosurgery64:E387–E3882009
YamaguchiTSuzukiSIshiiwaHShimizuKUedaY: Benign notochordal cell tumors: a comparative histological study of benign notochordal cell tumors, classic chordomas, and notochordal vestiges of fetal intervertebral discs. Am J Surg Pathol28:756–7612004
YamaguchiTYamatoMSaotomeK: First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions. Skeletal Radiol31:413–4182002
YamaguchiT, YamatoM, SaotomeK: First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions. 31:413–418, 200210.1007/s00256-002-0514-z)| false