The American journal of surgical pathology
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Am. J. Surg. Pathol. · Jan 2017
Case ReportsAtypical Notochordal Cell Tumors: A Series of Notochordal-derived Tumors That Defy Current Classification Schemes.
By the current WHO classification, benign notochordal cell tumor (BNCT) and chordoma comprise the entire spectrum of notochordal-derived tumors. They have defined radiologic and histologic criteria, and differ considerably in management and clinical outcome. Chordomas are malignant tumors; they show progressive, destructive growth and have the capacity for metastasis. ⋯ We would argue that designating these atypical notochordal tumors as chordoma precipitates potentially overly aggressive surgical management. Patients with ANCT may be better managed by close observation and serial imaging. Additional studies with more cases and longer clinical follow-up should clarify the relationship of ANCT to BNCT and chordoma.