Study design: Case report.
Objective: To present a rare case of a notochordal cell tumor.
Summary of background data: We report on a 27-year-old female patient with pain at the lower back and muscle cramps in the area of the right hip. Image studies demonstrated a cystic lesion of the coccyx.
Methods: As clinical symptoms became chronic and were resistant to conservative treatment, a resection of the coccyx was performed.
Results: Histology revealed an intraosseous benign notochordal cell tumor. This tumor represents a recently described notochordal cell proliferation biologically distinct from chordomas.
Conclusions: Overdiagnosis of these notochordal cell proliferations as chordomas may occur if clinicians and pathologists are unfamiliar with the spectrum of notochordal proliferations.