Benign notochordal cell tumor of the clivus with chordoma component: report of 2 cases

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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.

ABBREVIATIONS BNCT = benign notochordal cell tumor; EP = ecchordosis physaliphora.

Article Information

Correspondence Jamie J. Van Gompel: Mayo Clinic, Rochester, MN. vangompel.jamie@mayo.edu.

INCLUDE WHEN CITING Published online September 13, 2019; DOI: 10.3171/2019.6.JNS19529.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Preoperative sagittal precontrast (A) and postcontrast (B) T1-weighted MR images, axial T2-weighted MR images (C and D), and axial CT scans (E and F) of the skull base. Arrowhead (<) in panel F denotes pits in the mostly eroded clivus where these encompassed rests of chordoma/BNCT arise. Postoperative postcontrast sagittal T1-weighted (G) and T2-weighted (H) MR images demonstrating gross-total resection of the mass with postoperative reconstruction.

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    Case 1. BNCT component: notochordal cells with abundant clear cytoplasm fill marrow spaces between sclerotic trabeculae of bone (A), and tumor cells lack cytological atypia (B) and show strong expression of keratin AE1/AE3 (C), a feature that aids in distinguishing it from adipose tissue. Chordoma component: a solid extraosseous mass of notochordal tissue with abundant extracellular myxoid matrix (D) containing tumor cells with mild to moderate cytological atypia arranged in cords (E). H & E (A, B, D, and E), original magnification ×100 (A and D), ×200 (B, C, and E). Figure is available in color online only.

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    Case 2. Preoperative sagittal precontrast (A) and postcontrast (B) T1-weighted MR images, axial T2-weighted MR images (C and D) showing a hyperintense mass, and axial CT scans (E and F) of the skull base.

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    Case 2. BNCT component: a sheet of notochordal cells rimmed by trabeculae of bone demarcate the cells from hematopoietic marrow (A), tumor cells are composed of small nuclei surrounded by clear to flocculent eosinophilic cytoplasm (B), and a keratin AE1/AE3 stain shows strong positivity by the tumor cells (C). Chordoma component: tumor is composed of cells with moderate cytological atypia and vacuolated cytoplasm forming small clusters and cords within myxoid matrix (D and E). H & E (A, B, D, and E), original magnification ×40 (A and C), ×100 (D), ×200 (B and E). Figure is available in color online only.

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